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e3cbf4be0b5a-0 | 592 Neurocognitive Disorders
Neurocognitive Domains
The criteria for the various NCDs are all base d on defined cognitive domains. Table 1 pro-
vides for each of the key domains a working definition, examples of symptoms or obser-
vations regarding impairments in everyday activities, and examples of assessments. The
domains thus defined, along with guidelines for clinical thresholds, form the basis on
which the NCDs, their levels, and their subtypes may be diagnosed. | dsm5.pdf |
d3cc162b5ffc-0 | Neurocognitive Disorders 593TABLE 1 Neurocognitive domains
Cognitive domain Examples of symptoms or observations Examples of assessments
Complex attention
(sustained attention,
divided attention,
selective attention,
processing speed)Major: Has increased difficulty in environments with multiple
stimuli (TV, radio, conversation); is easily distracted by compet-
ing events in the environment. Is unable to attend unless input is
restricted and simplified. Has difficulty holding new information
in mind, such as recalling phone numbers or addresses just given,
or reporting what was just said. Is unable to perform mental cal-
culations. All thinking takes longer than usual, and components
to be processed must be simplified to one or a few.
Mild: Normal tasks take longer than previously. Begins to find
errors in routine task s; finds work needs more double-checking
than previously. Thinking is ea sier when not competing with
other things (radio, TV, other conversations, cell phone, driving).Sustained attention: Maintenance of attention over time (e.g., pressing
a button every time a tone is heard, and over a period of time).
Selective attention: Maintenance of attention despite competing stim-
uli and/or distractors: hearing nu mbers and letters read and asked
to count only letters.
Divided attention: Attending to two tasks within the same time
period: rapidly tapping while lear ning a story being read. Process-
ing speed can be quantified on any task by timing it (e.g., time to
put together a design of blocks; time to match symbols with num-
bers; speed in responding, such as counting speed or serial 3
speed).
Executive function
(planning, decision
making, working | dsm5.pdf |
d3cc162b5ffc-1 | speed).
Executive function
(planning, decision
making, working
memory, respond-
ing to feedback/
error correction,
overriding habits/
inhibition, mental
flexibility)Major: Abandons complex projects. Needs to focus on one task
at a time. Needs to rely on othe rs to plan instrumental activi-
ties of daily living or make decisions.
Mild: Increased effort required to complete multistage projects.
Has increased difficulty multitasking or difficulty resuming a
task interrupted by a visitor or phone call. May complain of
increased fatigue from the extra effort required to organize,
plan, and make decisions. May repo rt that large social gather-
ings are more taxing or less enjoyable because of increased
effort required to follow shifting conversations.Planning: Ability to find the exit to a maze; interpret a sequential pic-
ture or object arrangement.
Decision making: Performance of tasks that assess process of deciding
in the face of competing alternat ives (e.g., simulated gambling).
Working memory: Ability to hold information for a brief period and to
manipulate it (e.g., adding up a lis t of numbers or repeating a series
of numbers or words backward).
Feedback/error utilization: Ability to benefit from feedback to infer the
rules for solving a problem.
Overriding habits/inhibition: Ability to choose a more complex and
effortful solution to be correct (e.g., looking away from the direc-
tion indicated by an arrow; naming the color of a word’s font rather
than naming the word).
Mental/cognitive flexibility: Ability to shift between two concepts,
tasks, or response rules (e.g., from number to letter, from verbal to | dsm5.pdf |
d3cc162b5ffc-2 | tasks, or response rules (e.g., from number to letter, from verbal to
key-press response, from adding numbers to ordering numbers,
from ordering objects by si ze to ordering by color). | dsm5.pdf |
8228e232adda-0 | 594 Neurocognitive DisordersLearning and mem-
ory (immediate
memory, recent
memory [including
free recall, cued
recall, and recogni-
tion memory],
very-long-term
memory [semantic;
autobiographical],
implicit learning) Major: Repeats self in conversation, often within the same con-
versation. Cannot keep track of short list of items when shop-
ping or of plans for the day. Requires frequent reminders to
orient to task at hand.
Mild: Has difficulty recalling recent events, and relies increas-
ingly on list making or calendar. Needs occasional reminders
or re-reading to keep track of characters in a movie or novel.
Occasionally may repeat self over a few weeks to the same per-
son. Loses track of whether bills have already been paid.
Note: Except in severe forms of major neurocognitive disorder,
semantic, autobiographical, an d implicit memory are rela-
tively preserved, compar ed with recent memory. Immediate memory span: Ability to repeat a list of words or digits.
Note: Immediate memory sometimes subsumed under “working
memory” (see “Executive Function”).
Recent memory: Assesses the process of encoding new information
(e.g., word lists, a short story, or diagrams). The aspects of recent
memory that can be tested include 1) free recall (the person is asked
to recall as many words, diagrams, or elements of a story as possi-
ble); 2) cued recall (examiner aids recall by providing semantic cues
such as “List all the food items on the list” or “Name all of the chil-
dren from the story”); and 3) re cognition memory (examiner asks | dsm5.pdf |
8228e232adda-1 | dren from the story”); and 3) re cognition memory (examiner asks
about specific items—e.g., “Was ’apple’ on the list?” or “Did you
see this diagram or figure?”). Othe r aspects of memory that can be
assessed include semantic memory (memory for facts), autobio-
graphical memory (memory for pers onal events or people), and
implicit (procedural) learning (u nconscious learning of skills).
Language (expres-
sive language
[including nam-
ing, word finding,
fluency, and gram-
mar, and syntax]
and receptive
language)Major: Has significant difficulties with expressive or receptive
language. Often uses general-use phrases such as “that thing”
and “you know what I mean,” and prefers general pronouns
rather than names. With seve re impairment, may not even
recall names of closer friends and family. Idiosyncratic word
usage, grammatical errors, and spontaneity of output and
economy of utterances occur. Stereotypy of speech occurs;
echolalia and automatic speec h typically precede mutism.
Mild: Has noticeable word-finding difficulty. May substitute
general for specific terms. May avoid use of specific names of
acquaintances. Grammatical errors involve subtle omission or
incorrect use of articles, prepos itions, auxiliary verbs, etc. Expressive language: Confrontational naming (identification of objects
or pictures); fluency (e.g., name as many items as possible in a
semantic [e.g., animals] or phonemic [e.g., words starting with “f”]
category in 1 minute). | dsm5.pdf |
8228e232adda-2 | category in 1 minute).
Grammar and syntax (e.g., omission or incorrect use of articles, prep-
ositions, auxiliary verbs): Errors observed during naming and flu-
ency tests are compared with norm s to assess frequency of errors
and compare with normal slips of the tongue.
Receptive language: Comprehension (word definition and object-
pointing tasks involving animate and inanimate stimuli): perfor-
mance of actions/activities according to verbal command.TABLE 1 Neurocognitive domains (continued)
Cognitive domain Examples of symptoms or observations Examples of assessments | dsm5.pdf |
3310d5afb3c5-0 | Neurocognitive Disorders 595Perceptual-motor
(includes abilities
subsumed under
the terms visual
perception, visuo-
constructional,
perceptual-motor,
praxis, and gnosis ) Major: Has significant difficulties with previously familiar activ-
ities (using tools, driving motor vehicle), navigating in familiar
environments; is often more confused at dusk, when shadows
and lowering levels of light change perceptions.
Mild: May need to rely more on maps or others for directions.
Uses notes and follows others to get to a new place. May find
self lost or turned around when not concentrating on task. Is
less precise in parking. Needs to expend greater effort for spa-
tial tasks such as carpentry, assembly, sewing, or knitting.Visual perception: Line bisection tasks can be used to detect basic
visual defect or attentional neglect. Motor-free perceptual tasks
(including facial recognition) require the identification and/or
matching of figures—best when task s cannot be verbally mediated
(e.g., figures are not objects); some require the decision of whether
a figure can be “real” or no t based on dimensionality.
Visuoconstructional: Assembly of items requiring hand-eye coordina-
tion, such as drawing, copying, and block assembly.
Perceptual-motor: Integrating perception with purposeful movement
(e.g., inserting blocks into a form board without visual cues; rap-
idly inserting pegs into a slotted board).
Praxis: Integrity of learned movements, such as ability to imitate
gestures (wave goodbye) or pant omime use of objects to command
(“Show me how you would use a hammer”). | dsm5.pdf |
3310d5afb3c5-1 | (“Show me how you would use a hammer”).
Gnosis: Perceptual integrity of awaren ess and recognition, such as
recognition of faces and colors.
Social cognition
(recognition of
emotions, theory
of mind)Major: Behavior clearly out of acceptable social range; shows
insensitivity to social standards of modesty in dress or of polit-
ical, religious, or sexual topics of conversation. Focuses exces-
sively on a topic despite group’s disinterest or direct feedback.
Behavioral intention without regard to family or friends.
Makes decisions without regard to safety (e.g., inappropriate
clothing for weather or social setting). Typically, has little
insight into these changes.
Mild: Has subtle changes in behavior or attitude, often described
as a change in personality, such as less ability to recognize
social cues or read facial expressions, decreased empathy,
increased extraversion or introversion, decreased inhibition,
or subtle or episodic apathy or restlessness.Recognition of emotions: Identification of emot ion in images of faces
representing a variety of both positive and negative emotions.
Theory of mind: Ability to consider another person’s mental state
(thoughts, desires, intentions) or experience—story cards with
questions to elicit information about the mental stat e of the individ-
uals portrayed, such as “Where will the girl look for the lost bag?”
or “Why is the boy sad?” TABLE 1 Neurocognitive domains (continued)
Cognitive domain Examples of symptoms or observations Examples of assessments | dsm5.pdf |
93b6bf655355-0 | 596 Neurocognitive Disorders
Delirium
Diagnostic Criteria
A. A disturbance in attention (i.e., reduced ability to direct, focus, sustain, and shift atten-
tion) and awareness (reduced orientation to the environment).
B. The disturbance develops over a short period of time (usually hours to a few days), rep-
resents a change from baseline attention and awareness, and tends to fluctuate in se-
verity during the course of a day.
C. An additional disturbance in cognition (e.g., memory deficit, disorientation, language,
visuospatial ability, or perception).
D. The disturbances in Criteria A and C are not better explained by another preexisting,
established, or evolving neurocognitive disorder and do not occur in the context of a
severely reduced level of arousal, such as coma.
E. There is evidence from the history, physical examination, or laboratory findings that the
disturbance is a direct physiological consequence of another medical condition, sub-
stance intoxication or withdrawal (i.e., due to a drug of abuse or to a medication), or
exposure to a toxin, or is due to multiple etiologies.
Specify whether:
Substance intoxication delirium: This diagnosis should be made instead of sub-
stance intoxication when the symptoms in Criteria A and C predominate in the clinical
picture and when they are sufficiently severe to warrant clinical attention.
Coding note: The ICD-9-CM and ICD-10-CM codes for the [specific substance] in-
toxication delirium are indicated in the table below. Note that the ICD-10-CM code
depends on whether or not there is a comorbid substance use disorder present for
the same class of substance. If a mild substance use disorder is comorbid with the | dsm5.pdf |
93b6bf655355-1 | the same class of substance. If a mild substance use disorder is comorbid with the
substance intoxication delirium, the 4th position character is “1,” and the clinician
should record “mild [substance] use disorder” before the substance intoxication de-
lirium (e.g., “mild cocaine use disorder with cocaine intoxication delirium”). If a mod-
erate or severe substance use disorder is comorbid with the substance intoxication
delirium, the 4th position character is “2,” and the clinician should record “moderate
[substance] use disorder” or “severe [substance] use disorder,” depending on the
severity of the comorbid substance use disorder. If there is no comorbid substance
use disorder (e.g., after a one-time heavy use of the substance), then the 4th posi-
tion character is “9,” and the clinician should record only the substance intoxication
delirium.
ICD-10-CM
ICD-9-CMWith use
disorder,
mildWith use
disorder,
moderate or
severeWithout use
disorder
Alcohol 291.0 F10.121 F10.221 F10.921
Cannabis 292.81 F12.121 F12.221 F12.921
Phencyclidine 292.81 F16.121 F16.221 F16.921
Other hallucinogen 292.81 F16.121 F16.221 F16.921
Inhalant 292.81 F18.121 F18.221 F18.921
Opioid 292.81 F11.121 F11.221 F11.921 | dsm5.pdf |
a0f332bb7258-0 | Delirium 597
Substance withdrawal delirium: This diagnosis should be made instead of sub-
stance withdrawal when the symptoms in Criteria A and C predominate in the clinical
picture and when they are sufficiently severe to warrant clinical attention.
Code [specific substance] withdrawal delirium: 291.0 (F10.231) alcohol; 292.0
(F11.23) opioid; 292.0 (F13.231) sedative, hypnotic, or anxiolytic; 292.0 (F19.231)
other (or unknown) substance/medication.
Medication-induced delirium: This diagnosis applies when the symptoms in Criteria
A and C arise as a side effect of a medication taken as prescribed.
Coding note: The ICD-9-CM code for [specific medication]-induced delirium is
292.81. The ICD-10-CM code depends on the type of medication. If the medication
is an opioid taken as prescribed, the code is F11.921. If the medication is a seda-
tive, hypnotic, or anxiolytic taken as prescribed, the code is F13.921. If the medica-
tion is an amphetamine-type or other stimulant taken as prescribed, the code is
F15.921. For medications that do not fit into any of the classes (e.g., dexametha-
sone) and in cases in which a substance is judged to be an etiological factor but the
specific class of substance is unknown, the code is F19.921.
293.0 (F05) Delirium due to another medical condition: There is evidence from the
history, physical examination, or laboratory findings that the disturbance is attributable
to the physiological consequences of another medical condition. | dsm5.pdf |
a0f332bb7258-1 | to the physiological consequences of another medical condition.
Coding note: Include the name of the other medical condition in the name of the
delirium (e.g., 293.0 [F05] delirium due to hepatic encephalopathy). The other med-
ical condition should also be coded and listed separately immediately before the
delirium due to another medical condition (e.g., 572.2 [K72.90] hepatic encepha-
lopathy; 293.0 [F05] delirium due to hepatic encephalopathy).
293.0 (F05) Delirium due to multiple etiologies: There is evidence from the history,
physical examination, or laboratory findings that the delirium has more than one etiol-
ogy (e.g., more than one etiological medical condition; another medical condition plus
substance intoxication or medication side effect).
Coding note: Use multiple separate codes reflecting specific delirium etiologies
(e.g., 572.2 [K72.90] hepatic encephalopathy, 293.0 [F05] delirium due to hepatic
failure; 291.0 [F10.231] alcohol withdrawal de lirium). Note that the etiological med-
ical condition both appears as a separate code that precedes the delirium code and
is substituted into the delirium due to another medical condition rubric.
Specify if:
Acute: Lasting a few hours or days.
Persistent: Lasting weeks or months.Sedative, hypnotic, or anxioly tic 292.81 F13.121 F13.221 F13.921
Amphetamine (or other
stimulant)292.81 F15.121 F15.221 F15.921 | dsm5.pdf |
a0f332bb7258-2 | stimulant)292.81 F15.121 F15.221 F15.921
Cocaine 292.81 F14.121 F14.221 F14.921
Other (or unknown) substance 292.81 F19.121 F19.221 F19.921ICD-10-CM
ICD-9-CMWith use
disorder,
mildWith use
disorder,
moderate or
severeWithout use
disorder | dsm5.pdf |
82dff363697a-0 | 598 Neurocognitive Disorders
Specify if:
Hyperactive: The individual has a hyperactive level of psychomotor activity that may be
accompanied by mood lability, agitation, and/or refusal to cooperate with medical care.
Hypoactive: The individual has a hypoactive level of psychomotor activity that may be
accompanied by sluggishness and lethargy that approaches stupor.
Mixed level of activity: The individual has a normal level of psychomotor activity even
though attention and awareness are disturbed. Also includes individuals whose activity
level rapidly fluctuates.
Recording Procedures
Substance intoxication delirium
ICD-9-CM. The name of the substance/medication intoxication delirium begins with
the specific substance (e.g., co caine, dexamethasone) that is presumed to be causing the
delirium. The diagnostic code is selected from the table includ ed in the criteria set, which
is based on the drug class. For substances that do not fit into any of the classes (e.g., dexa-
methasone), the code for “other substance” should be used; and in cases in which a sub-
stance is judged to be an etiological factor but the specific class of substance is unknown,
the category “unknown substance” should be used.
The name of the disorder is followed by the course (i.e., acute, persistent), followed by
the specifier indicating level of psychomotor activity (i.e., hyperactive, hypoactive, mixed
level of activity). Unlike the recording proc edures for ICD-10-CM, which combine the sub-
stance/medication intoxication delirium and subs tance use disorder into a single code, for
ICD-9-CM a separate diagnostic code is give n for the substance use disorder. For example, | dsm5.pdf |
82dff363697a-1 | in the case of acute hyperactive intoxication delirium occurring in a man with a severe co-
caine use disorder, the diagnosis is 292.81 cocaine intoxication delirium, acute, hyperac-
tive. An additional diagnosis of 304.20 severe cocaine use disorder is also given. If the
intoxication delirium occurs without a comorbid substance use disorder (e.g., after a one-
time heavy use of the substance), no accompan ying substance use disorder is noted (e.g.,
292.81 phencyclidine intoxication delirium, acute, hypoactive).
ICD-10-CM. The name of the substance/ medication intoxication de lirium begins with the
specific substance (e.g., cocaine, dexamethasone) that is presumed to be causing the delirium.
The diagnostic code is selected from the table in cluded in the criteria set, which is based on the
drug class and presence or absence of a comorbid substance use disorder. For substances that
do not fit into any of the classes (e.g., dexamethasone), the code for “other substance” should
be used; and in cases in which a substance is judg ed to be an etiological factor but the specific
class of substance is unknown, the category “unknown substance” should be used.
When recording the name of the disorder, the comorbid substance use disorder (if any) is
listed first, followed by the word “with,” foll owed by the name of th e substance intoxication
delirium, followed by the course (i.e., acute, persistent), follo wed by the specifier indicating
level of psychomotor activity (i.e., hyperactive, hypoactive, mixed level of activity). For exam- | dsm5.pdf |
82dff363697a-2 | ple, in the case of acute hyperactive intoxication delirium occurring in a man with a severe co-
caine use disorder, the diagnosis is F14.221 severe cocaine use disorder with cocaine
intoxication delirium, acute, hy peractive. A separate diagnosis of the comorbid severe cocaine
use disorder is not given. If the intoxication delirium occurs without a comorbid substance use
disorder (e.g., after a one-time heavy use of the substance), no accompanying substance use
disorder is noted (e.g., F16.921 phencyclidine intoxication delirium, acute, hypoactive).
Substance withdrawal delirium
ICD-9-CM. The name of the substance/medication withdrawal delirium begins with the
specific substance (e.g., alcohol) that is presumed to be causing the withdrawal delirium. The | dsm5.pdf |
0af9659e3abd-0 | ICD-9-CM. The name of the substance/medication withdrawal delirium begins with the
specific substance (e.g., alcohol) that is presumed to be causing the withdrawal delirium. The
diagnostic code is selected from substance-spec ific codes included in the coding note included | dsm5.pdf |
e48c68f8ea3a-0 | Delirium 599
in the criteria set. The name of the disorder is fo llowed by the course (i.e., acute, persistent), fol-
lowed by the specifier indicating level of psycho motor activity (i.e., hy peractive, hypoactive,
mixed level of activity). Unlike the recording procedures for ICD-10-CM, which combine the
substance/medication withdrawal delirium and su bstance use disorder into a single code, for
ICD-9-CM a separate diagnostic code is given for the substance use disorder. For example, in
the case of acute hyperactive withdrawal delirium occurring in a man with a severe alcohol use
disorder, the diagnosis is 291.0 alcohol withdrawal delirium, acute, hyperactive. An additional
diagnosis of 303.90 severe alcohol use disorder is also given.
ICD-10-CM. The name of the substa nce/medication withdrawal delirium begins with
the specific substance (e.g., al cohol) that is presumed to be causing the withdrawal delir-
ium. The diagnostic code is selected from su bstance-specific codes included in the coding
note included in the criteria set. When re cording the name of the disorder, the comorbid
moderate or severe substance use disorder (if any) is listed first, followed by the word
“with,” followed by the substance withdrawal delirium, followed by the course (i.e., acute,
persistent), followed by the specifier indicating level of psychomotor activity (i.e., hyper-
active, hypoactive, mixed level of activity). Fo r example, in the case of acute hyperactive
withdrawal delirium occurring in a man with a severe alcohol use disorder, the diagnosis
is F10.231 severe alcohol use disorder with alcohol withdrawal delirium, acute, hyperac- | dsm5.pdf |
e48c68f8ea3a-1 | tive. A separate diagnosis of the comorbid severe alcohol use disorder is not given.
Medication-induced delirium. The name of the medication-induced delirium begins
with the specific substance (e.g., dexamethasone) that is presumed to be causing the de-
lirium. The name of the disorder is followed by the course (i.e., acute, persistent), followed
by the specifier indicating le vel of psychomotor activity (i.e., hyperactive, hypoactive,
mixed level of activity). For example, in th e case of acute hyperactive medication-induced
delirium occurring in a man using dexamethasone as prescribed, the diagnosis is 292.81
(F19.921) dexamethasone-induced delirium, acute, hyperactive.
Specifiers
Regarding course, in hospital settings, delirium usually lasts about 1 week, but some
symptoms often persist even after indivi duals are discharged from the hospital.
Individuals with delirium may rapidly switch between hyperactive and hypoactive
states. The hyperactive state may be more common or more frequently recognized and
often is associated with medication side effe cts and drug withdrawal . The hypoactive state
may be more frequent in older adults.
Diagnostic Features
The essential feature of deliriu m is a disturbance of attention or awareness that is accom-
panied by a change in baseline cognition that cannot be better explained by a preexisting
or evolving neurocognitive disorder (NCD). The disturbance in attention (Criterion A) is
manifested by reduced ability to direct, focus, sustain, and shift attention. Questions must
be repeated because the individual’s attentio n wanders, or the individual may perseverate
with an answer to a previous question rather than appropriately shift attention. The indi- | dsm5.pdf |
e48c68f8ea3a-2 | with an answer to a previous question rather than appropriately shift attention. The indi-
vidual is easily distracted by irrelevant st imuli. The disturbance in awareness is mani-
fested by a reduced orientation to the environment or at times even to oneself.
The disturbance develops over a short period of time, usually hours to a few days, and
tends to fluctuate during the course of the day, often with worsen ing in the evening and
night when external orienting stimuli decrease (Criterion B). There is evidence from the
history, physical examination, or laboratory findings that the disturbance is a physiologi-
cal consequence of an underlying medical condition, substance intoxication or with- | dsm5.pdf |
b921808313a4-0 | history, physical examination, or laboratory findings that the disturbance is a physiologi-
cal consequence of an underlying medical condition, substance intoxication or with-
drawal, use of a medication, or a toxin ex posure, or a combinat ion of these factors
(Criterion E). The etiology should be coded a ccording to the etiologically appropriate sub-
type (i.e., substance or medication intoxica tion, substance withdr awal, another medical | dsm5.pdf |
ae76c0e0f97a-0 | 600 Neurocognitive Disorders
condition, or multiple etiologi es). Delirium often occurs in the context of an underlying
NCD. The impaired brain function of individuals with mild and major NCD renders them
more vulnerable to delirium.
There is an accompanying change in at le ast one other area that may include memory
and learning (particularly recent memory), diso rientation (particularly to time and place),
alteration in language, or perceptual distorti on or a perceptual-motor disturbance (Crite-
rion C). The perceptual dist urbances accompanying delirium include misinterpretations,
illusions, or hallucinations; these disturbances are typically visual, but may occur in other
modalities as well, and range from simple an d uniform to highly complex. Normal atten-
tion/arousal, delirium, and coma lie on a cont inuum, with coma defined as the lack of any
response to verbal stimuli. The ability to evaluate cognition to diagnose delirium depends
on there being a level of arousal sufficient for response to verbal stim ulation; hence, delir-
ium should not be diagnosed in the context of coma (Criterion D). Many noncomatose pa-
tients have a reduced level of arousal. Those patients who show only minimal responses to
verbal stimulation are incapable of engaging with attempts at standardized testing or even
interview. This inability to engage should be classified as severe inattention. Low-arousal
states (of acute onset) should be recognized as indicating severe inattention and cognitive
change, and hence delirium. They are clinically indistinguishable from delirium diag-
nosed on the basis of inattentio n or cognitive change elicited through cognitive testing and
interview.
Associated Features Supporting Diagnosis | dsm5.pdf |
ae76c0e0f97a-1 | interview.
Associated Features Supporting Diagnosis
Delirium is often associated with a disturbanc e in the sleep-wake cycl e. This disturbance
can include daytime sleepiness, nighttime ag itation, difficulty falling asleep, excessive
sleepiness throughout the day, or wakefulnes s throughout the night. In some cases, com-
plete reversal of the night-day sleep-wake cy cle can occur. Sleep-wake cycle disturbances
are very common in delirium and have been proposed as a core criterion for the diagnosis.
The individual with deliri um may exhibit emotional di sturbances, such as anxiety,
fear, depression, irritability, anger, euphori a, and apathy. There may be rapid and unpre-
dictable shifts from one emot ional state to another. The di sturbed emotional state may also
be evident in calling out, screaming, cursin g, muttering, moaning, or making other
sounds. These behaviors are especially prevalent at nigh t and under conditions in which
stimulation and environmen tal cues are lacking.
Prevalence
The prevalence of delirium is highest amon g hospitalized older individuals and varies
depending on the individuals’ characteristics, setting of care, and se nsitivity of the detec-
tion method. The prevalence of delirium in the community overall is low (1%–2%) but in-
creases with age, rising to 14% among individuals older than 85 years. The prevalence is
10%–30% in older individuals presenting to emergency departments, where the delirium
often indicates a medical illness.
The prevalence of delirium when individuals are admitted to the hospital ranges from
14% to 24%, and estimates of the incidence of delirium arising du ring hospitalization | dsm5.pdf |
ae76c0e0f97a-2 | range from 6% to 56% in general hospital populations. Delirium occurs in 15%–53% of
older individuals postoperatively and in 70% –87% of those in intensive care. Delirium oc-
curs in up to 60% of individuals in nursing ho mes or post–acute care settings and in up to
83% of all individuals at the end of life.
Development and Course
While the majority of individuals with deli rium have a full recovery with or without
treatment, early recognition and intervention usually shortens the duration of the delir- | dsm5.pdf |
4c6b808f7df3-0 | Delirium 601
ium. Delirium may progress to stupor, coma, seizures, or death, particularly if the under-
lying cause remains untreated. Mortality amon g hospitalized individuals with delirium is
high, and as many as 40% of individuals with delirium, particularly those with malignan-
cies and other significant underlying medical illness, die within a year after diagnosis.
Risk and Prognostic Factors
Environmental. Delirium may be increased in the cont ext of functional impairment, im-
mobility, a history of falls, low levels of activity, and use of drugs and medications with
psychoactive properties (particularly alcohol and anticholinergics).
Genetic and physiological. Both major and mild NCDs can increase the risk for delir-
ium and complicate the course. Older individu als are especially susceptible to delirium
compared with younger adults. Susceptibilit y to delirium in infancy and through child-
hood may be greater than in early and mi ddle adulthood. In ch ildhood, delirium may be
related to febrile illnesses and certain medications (e.g., anticholinergics).
Diagnostic Markers
In addition to laboratory find ings characteristic of underlying medical conditions (or in-
toxication or withdrawal states), there is often generalized slowing on electroencephalog-
raphy, and fast activity is oc casionally found (e.g., in some cases of alcohol withdrawal
delirium). However, electroence phalography is insufficiently sensitive and specific for di-
agnostic use.
Functional Consequences of Delirium
Delirium itself is associated with increased functional decline and risk of institutional
placement. Hospitalized individuals 65 years or older with delirium have three times the | dsm5.pdf |
4c6b808f7df3-1 | placement. Hospitalized individuals 65 years or older with delirium have three times the
risk of nursing home placemen t and about three times the func tional decline as hospital-
ized patients without delirium at both discharge and 3 months postdischarge.
Differential Diagnosis
Psychotic disorders and bipolar and depre ssive disorders with psychotic features.
Delirium that is characterized by vivid hallucinations, delusions, language disturbances,
and agitation must be distinguished from br ief psychotic disorder, schizophrenia, schizo-
phreniform disorder, and other psychotic disord ers, as well as from bipolar and depres-
sive disorders with psychotic features.
Acute stress disorder. Delirium associated with fear, an xiety, and dissociative symptoms,
such as depersonalization, must be distingu ished from acute stress disorder, which is pre-
cipitated by exposure to a severely traumatic event.
Malingering and factitious disorder. Delirium can be distinguished from these disor-
ders on the basis of the often atypical presen tation in malingering and factitious disorder
and the absence of another medical condition or substance that is etiologically related to
the apparent cognitive disturbance.
Other neurocognitive disorders. The most common differential diagnostic issue when
evaluating confusion in older adults is dise ntangling symptoms of delirium and dementia.
The clinician must determine whether the in dividual has delirium; a delirium superim-
posed on a preexisting NCD, such as that due to Alzheimer’s disease; or an NCD without
delirium. The traditional distinction between delirium and dementia according to acute-
ness of onset and temporal course is particularly difficult in those elderly individuals who
had a prior NCD that may not have been reco gnized, or who develop persistent cognitive | dsm5.pdf |
4c6b808f7df3-2 | had a prior NCD that may not have been reco gnized, or who develop persistent cognitive
impairment following an episode of delirium. | dsm5.pdf |
132f4dcfd83a-0 | 602 Neurocognitive Disorders
Other Specified Delirium
780.09 (R41.0)
This category applies to presentations in which symptoms characteristic of delirium that
cause clinically significant distress or impairm ent in social, occupational, or other impor-
tant areas of functioning predominate but do not meet the full criteria for delirium or any of
the disorders in the neurocognitive disorders diagnostic class. The other specified delirium
category is used in situations in which the clinician chooses to communicate the specific
reason that the presentation does not meet the criteria for delirium or any specific neuro-
cognitive disorder. This is done by recording “other specified delirium” followed by the spe-
cific reason (e.g., “attenuated delirium syndrome”).
An example of a presentation that can be specified using the “other specified” desig-
nation is the following:
Attenuated delirium syndrome: This syndrome applies in cases of delirium in which
the severity of cognitive impairment falls short of that required for the diagnosis, or in
which some, but not all, diagnostic criteria for delirium are met.
Unspecified Delirium
780.09 (R41.0)
This category applies to presentations in which symptoms characteristic of delirium that
cause clinically significant distress or impairm ent in social, occupational, or other impor-
tant areas of functioning predominate but do not meet the full criteria for delirium or any of
the disorders in the neurocognitive disorder s diagnostic class. The unspecified delirium
category is used in situations in which the clinician chooses not to specify the reason that
the criteria are not met for delirium, and includes presentations for which there is insuffi-
cient information to make a more specific diagnosis (e.g., in emergency room settings). | dsm5.pdf |
132f4dcfd83a-1 | cient information to make a more specific diagnosis (e.g., in emergency room settings).
Major and Mild Neurocognitive Disorders
Major Neurocognitive Disorder
Diagnostic Criteria
A. Evidence of significant cognitive decline from a previous level of performance in one
or more cognitive domains (complex attent ion, executive function, learning and mem-
ory, language, perceptual-motor, or social cognition) based on:
1. Concern of the individual, a knowledgeable informant, or the clinician that there has
been a significant decline in cognitive function; and
2. A substantial impairment in cognitive performance, preferably documented by stan-
dardized neuropsychological testing or, in its absence, another quantified clinical
assessment.
B. The cognitive deficits interfere with independence in everyday activities (i.e., at a min-
imum, requiring assistance with complex instrumental activities of daily living such as
paying bills or managing medications).
C. The cognitive deficits do not occur e xclusively in the context of a delirium. | dsm5.pdf |
3e37ba963d43-0 | Major and Mild Neurocognitive Disorders 603
D. The cognitive deficits are not better explai ned by another mental disorder (e.g., major
depressive disorder, schizophrenia).
Specify whether due to:
Alzheimer’s disease (pp. 611–614)
Frontotemporal lobar degeneration (pp. 614–618)
Lewy body disease (pp. 618–621)
Vascular disease (pp. 621–624)
Traumatic brain injury (pp. 624–627)
Substance/medication use (pp. 627–632)
HIV infection (pp. 632–634)
Prion disease (pp. 634–636)
Parkinson’s disease (pp. 636–638)
Huntington’s disease (pp. 638–641)
Another medical condition (pp. 641–642)
Multiple etiologies (pp. 642–643)
Unspecified (p. 643)
Coding note: Code based on medical or substance etiology. In some cases, there is need
for an additional code for the etiological medi cal condition, which must immediately pre-
cede the diagnostic code for major neurocognitive disorder, as follows:
Etiological subtypeAssociated etiological
medical code for major
neurocognitive disorderaMajor neurocogni-
tive disorder codebMild neurocogni-
tive disorder codec
Alzheimer’s
diseaseProbable: 331.0 (G30.9)
Possible: no additional
medical codeProbable: 294.1x
(F02.8x)
Possible: 331.9
(G31.9)c331.83 (G31.84)
(Do not use addi- | dsm5.pdf |
3e37ba963d43-1 | (Do not use addi-
tional code for
Alzheimer’s
disease.)
Frontotemporal
lobar degeneration Probable: 331.19
(G31.09)
Possible: no additional
medical codeProbable: 294.1x
(F02.8x)
Possible: 331.9
(G31.9)c331.83 (G31.84)
(Do not use addi-
tional code for
frontotemporal
disease.)
Lewy body disease Probable: 331.82
(G31.83)
Possible: no additional
medical codeProbable: 294.1x
(F02.8x)
Possible: 331.9
(G31.9)c331.83 (G31.84)
(Do not use addi-
tional code for
Lewy body disease.)
Vascular disease No additional medical
codeProbable: 290.40
(F01.5x)
Possible: 331.9
(G31.9)c331.83 (G31.84)
(Do not use addi-
tional code for the
vascular disease.)
Traumatic brain
injury907.0 (S06.2X9S) 294.1x (F02.8x) 331.83 (G31.84)
(Do not use additional
code for the trau-
matic brain injury.)
Substance/
medication-
inducedNo additional medical
codeCode based on the
type of substance
causing the major
neurocognitive
disorderc, dCode based on the
type of substance | dsm5.pdf |
3e37ba963d43-2 | disorderc, dCode based on the
type of substance
causing the mild
neurocognitive
disorderd | dsm5.pdf |
5af21aea5c37-0 | 604 Neurocognitive Disorders
HIV infection 042 (B20) 294.1x (F02.8x) 331.83 (G31.84)
(Do not use addi-
tional code for HIV
infection.)
Prion disease 046.79 (A81.9) 294.1x (F02.8x) 331.83 (G31.84)
(Do not use addi-
tional code for
prion disease.)
Parkinson’s
diseaseProbable: 332.0 (G20)
Possible: No additional
medical codeProbable: 294.1x
(F02.8x)
Possible: 331.9
(G31.9)c331.83 (G31.84)
(Do not use addi-
tional code for
Parkinson’s
disease.)
Huntington’s
disease333.4 (G10) 294.1x (F02.8x) 331.83 (G31.84)
(Do not use addi-
tional code for
Huntington’s
disease.)
Due to another
medical conditionCode the other medical
condition first
(e.g., 340 [G35]
multiple sclerosis)294.1x (F02.8x) 331.83 (G31.84)
(Do not use addi-
tional codes for the
presumed etiologi-
cal medical condi-
tions.)
Due to multiple
etiologiesCode all of the etiological
medical conditions first
(with the exception of
vascular disease) 294.1x (F02.8x)
(Plus the code for | dsm5.pdf |
5af21aea5c37-1 | (Plus the code for
the relevant sub-
stance/medication-
induced major neu-
rocognitive disor-
ders if substances
or medications
play a role in the
etiology.)331.83 (G31.84)
(Plus the code for
the relevant sub-
stance/medication-
induced mild neuro-
cognitive disor-
ders if substances
or medications play
a role in the etiol-
ogy. Do not use ad-
ditional codes for
the presumed
etiological medical
conditions.)
Unspecified neuro-
cognitive disorderNo additional medical
code799.59 (R41.9) 799.59 (R41.9)
aCode first, before code for major neurocognitive disorder.
bCode fifth character based on symptom specifier: .x0 without behavioral disturbance; .x1 with be-
havioral disturbance (e.g., psychotic symptoms, mo od disturbance, agitatio n, apathy, or other be-
havioral symptoms).
cNote: Behavioral disturbance specifier cannot be coded but should still be indicated in writing.
dSee “Substance/Medication-Induced Majo r or Mild Neurocognitive Disorder.”Etiological subtypeAssociated etiological
medical code for major
neurocognitive disorderaMajor neurocogni-
tive disorder codebMild neurocogni-
tive disorder codec | dsm5.pdf |
c4ec325f6ceb-0 | Major and Mild Neurocognitive Disorders 605
Specify:
Without behavioral disturbance: If the cognitive disturbance is not accompanied by
any clinically significant behavioral disturbance.
With behavioral disturbance (specify disturbance): If the cognitive disturbance is ac-
companied by a clinically significant behavioral disturbance (e.g., psychotic symptoms,
mood disturbance, agitation, apathy, or other behavioral symptoms).
Specify current severity:
Mild: Difficulties with instrumental activities of daily living (e.g., housework, managing
money).
Moderate: Difficulties with basic activities of daily living (e.g., feeding, dressing).
Severe: Fully dependent.
Mild Neurocognitive Disorder
Diagnostic Criteria
A. Evidence of modest cognitive decline from a previous level of performance in one or
more cognitive domains (complex attention, executive function, learning and memory,
language, perceptual-motor, or social cognition) based on:
1. Concern of the individual, a knowledgeable informant, or the clinician that there has
been a mild decline in cognitive function; and
2. A modest impairment in cognitive per formance, preferably documented by stan-
dardized neuropsychological testing or, in its absence, another quantified clinical
assessment.
B. The cognitive deficits do not interfere with capacity for independence in everyday
activities (i.e., complex instrumental activities of daily living such as paying bills or
managing medications are preserved, but gr eater effort, compensatory strategies, or
accommodation may be required).
C. The cognitive deficits do not occur e xclusively in the context of a delirium.
D. The cognitive deficits are not better explai ned by another mental disorder (e.g., major
depressive disorder, schizophrenia).
Specify whether due to:
Alzheimer’s disease (pp. 611–614) | dsm5.pdf |
c4ec325f6ceb-1 | Specify whether due to:
Alzheimer’s disease (pp. 611–614)
Frontotemporal lobar degeneration (pp. 614–618)
Lewy body disease (pp. 618–621)
Vascular disease (pp. 621–624)
Traumatic brain injury (pp. 624–627)
Substance/medication use (pp. 627–632)
HIV infection (pp. 632–634)
Prion disease (pp. 634–636)
Parkinson’s disease (pp. 636–638)
Huntington’s disease (pp. 638–641)
Another medical condition (pp. 641–642)
Multiple etiologies (pp. 642–643)
Unspecified (p. 643)
Coding note: For mild neurocognitive disorder due to any of the medical etiologies listed
above, code 331.83 (G31.84). Do not use additional codes for the presumed etiological
medical conditions. For substance/medicati on-induced mild neurocognitive disorder, code
based on type of substance; see “Substance/ Medication-Induced Major or Mild Neurocog-
nitive Disorder.” For unspecified mild neurocognitive disorder, code 799.59 (R41.9). | dsm5.pdf |
ddde3cde8f35-0 | 606 Neurocognitive Disorders
Specify:
Without behavioral disturbance: If the cognitive disturbance is not accompanied by
any clinically significant behavioral disturbance.
With behavioral disturbance (specify disturbance): If the cognitive disturbance is ac-
companied by a clinically significant behavioral disturbance (e.g., psychotic symptoms,
mood disturbance, agitation, apathy, or other behavioral symptoms).
Subtypes
Major and mild neurocognitive disorders (NCD s) are primarily subtyped according to the
known or presumed etiological/pa thological entity or entities underlying the cognitive de-
cline. These subtypes are distin guished on the basis of a combin ation of time course, charac-
teristic domains affected, and associated sy mptoms. For certain etio logical subtypes, the
diagnosis depends substantially on the presence of a potentially causative entity, such as Par-
kinson’s or Huntington’s disease, or a traumati c brain injury or stroke in the appropriate time
period. For other etiological subt ypes (generally the neurodegenerative diseases like Alzhei-
mer’s disease, frontotemporal lobar degenerati on, and Lewy body disease), the diagnosis is
based primarily on the cognitive, behavioral, an d functional symptoms. Typically, the differ-
entiation among these syndromes that lack an independently recognized etiological entity is
clearer at the level of major NCD than at the level of mild NCD, but sometimes characteristic
symptoms and associated features are present at the mild level as well.
NCDs are frequently managed by clinicians in multiple disciplines. For many sub-
types, multidisciplinary international expert groups have developed specialized consen-
sus criteria based on clinicopathological corr elation with underlying brain pathology. The
subtype criteria here have been harm onized with those expert criteria.
Specifiers | dsm5.pdf |
ddde3cde8f35-1 | subtype criteria here have been harm onized with those expert criteria.
Specifiers
Evidence for distinct be havioral features in NCDs has been recognized, particularly in the
areas of psychotic symptoms and depression . Psychotic features are common in many
NCDs, particularly at the mild-to-moderate stage of major NCDs due to Alzheimer’s dis-
ease, Lewy body disease, an d frontotemporal lobar degene ration. Paranoia and other
delusions are common features, and often a persecutory theme may be a prominent aspect
of delusional ideation. In contrast to psychotic disorders with onset in earlier life (e.g.,
schizophrenia), disorganized speech and diso rganized behavior are not characteristic of
psychosis in NCDs. Hallucinations may occur in any modality, although visual hallucina-
tions are more common in NCDs than in de pressive, bipolar, or psychotic disorders.
Mood disturbances, including depression, anxiety, and elat ion, may occur. Depression
is common early in the course (including at the mild NCD level) of NCD due to Alzhei-
mer’s disease and Parkinson’s disease, while elation may occur more commonly in fron-
totemporal lobar degeneration. When a full a ffective syndrome meetin g diagnostic criteria
for a depressive or bipolar disorder is pres ent, that diagnosis should be coded as well.
Mood symptoms are increasingly recognized to be a significant feature in the earliest stages
of mild NCDs such that clinical recogn ition and intervention may be important.
Agitation is common in a wide variety of NCDs, particularly in major NCD of moder-
ate to severe severity, and often occurs in th e setting of confusion or frustration. It may | dsm5.pdf |
ddde3cde8f35-2 | ate to severe severity, and often occurs in th e setting of confusion or frustration. It may
arise as combative behaviors, particularly in the context of resisting caregiving duties such
as bathing and dressing. Agitation is characte rized as disruptive motor or vocal activity
and tends to occur with advanced stages of cognitive impairment across all of the NCDs.
Individuals with NCD can present with a wi de variety of behavioral symptoms that
are the focus of treatment. Sleep disturbance is a common symptom that can create a need
for clinical attention and may include symptoms of insomnia, hypersomnia, and circadian | dsm5.pdf |
47d105e1fe68-0 | are the focus of treatment. Sleep disturbance is a common symptom that can create a need
for clinical attention and may include symptoms of insomnia, hypersomnia, and circadian
rhythm disturbances. | dsm5.pdf |
eec291eed380-0 | Major and Mild Neurocognitive Disorders 607
Apathy is common in mild and mild major NCD. It is observed particularly in NCD due
to Alzheimer’s disease and ma y be a prominent feature of NCD due to frontotemporal
lobar degeneration. Apathy is typically char acterized by diminished motivation and re-
duced goal-directed behavior accompanied by decreased emotional responsiveness.
Symptoms of apathy may manifest early in the course of NCDs when a loss of motivation
to pursue daily activities or hobbies may be observed.
Other important behavioral symptoms include wandering, disinhibition, hyperpha-
gia, and hoarding. Some of these symptoms are characteristic of specific disorders, as dis-
cussed in the relevant sections. When more th an one behavioral disturbance is observed,
each type should be noted in writing with the specifier “with behavioral symptoms.”
Diagnostic Features
Major and mild NCDs exist on a spectrum of cognitive and functional impairment. Major
NCD corresponds to the conditio n referred to in DSM-IV as dementia, retained as an alter-
native in this volume. The co re feature of NCDs is acquir ed cognitive decline in one or
more cognitive domains (Criterion A) based on both 1) a concern about cognition on the
part of the individual, a knowledgeable informant, or the clinician, and 2) performance on
an objective assessment that falls below the expected level or that has been observed to de-
cline over time. Both a concer n and objective evidence are re quired because they are com-
plementary. When there is an exclusive focus on objectiv e testing, a disorder may go
undiagnosed in high-functioning individuals whose currently “normal” performance ac-
tually represents a substantial decline in abilities, or an illness may be incorrectly diag- | dsm5.pdf |
eec291eed380-1 | tually represents a substantial decline in abilities, or an illness may be incorrectly diag-
nosed in individuals whose currently “low” performance does not represent a change
from their own baseline or is a result of extraneous factors li ke test conditions or a passing
illness. Alternatively, excessive focus on subj ective symptoms may fail to diagnose illness
in individuals with poor insigh t, or whose informants deny or fail to notice their symptoms,
or it may be overly sensitiv e in the so-called worried well.
A cognitive concern differs from a complaint in that it may or may not be voiced spon-
taneously. Rather, it may need to be elicited by careful questioning about specific symp-
toms that commonly occur in individuals wi th cognitive deficits (see Table 1 in the
introduction to this chapter). For example, memory concerns include difficulty remember-
ing a short grocery list or keeping track of the plot of a television program; executive con-
cerns include difficulty resuming a task wh en interrupted, organizing tax records, or
planning a holiday meal. At the mild NCD level, the individual is likely to describe these
tasks as being more difficult or as requiring ex tra time or effort or compensatory strategies.
At the major NCD level, such tasks may on ly be completed with assistance or may be
abandoned altogether. At the mild NCD level, individuals and their families may not no-
tice such symptoms or may view them as norm al, particularly in the elderly; thus, careful
history taking is of paramount importance. The difficulties must represent changes rather
than lifelong patterns: the individual or info rmant may clarify this i ssue, or the clinician
can infer change from prior experience with the patient or from occupational or other
clues. It is also critical to determine that the difficulties are related to cognitive loss rather | dsm5.pdf |
eec291eed380-2 | clues. It is also critical to determine that the difficulties are related to cognitive loss rather
than to motor or sensory limitations.
Neuropsychological testing, with performance compared with norms appropriate to
the patient’s age, educational attainment, and cultural background, is part of the standard
evaluation of NCDs and is part icularly critical in the evaluation of mild NCD. For major
NCD, performance is typically 2 or more standard deviations below appropriate norms | dsm5.pdf |
2b5b71fd39ac-0 | evaluation of NCDs and is part icularly critical in the evaluation of mild NCD. For major
NCD, performance is typically 2 or more standard deviations below appropriate norms
(3rd percentile or below). For mild NCD, performance typically lies in the 1–2 standard de-
viation range (between the 3rd and 16th perc entiles). However, neur opsychological test-
ing is not available in all settings, and neuropsychological thresholds are sensitive to the
specific test(s) and norms employed, as well as to test conditions, sensory limitations, and
intercurrent illness. A variety of brief office-based or “bedside” asse ssments, as described | dsm5.pdf |
0184da59cf14-0 | 608 Neurocognitive Disorders
in Table 1, can also supply objective data in settings where such testing is unavailable or
infeasible. In any case, as with cognitive co ncerns, objective perfor mance must be inter-
preted in light of the individual’s prior pe rformance. Optimally, this information would
be available from a prior administration of the same test, but often it must be inferred
based on appropriate norms, along with the individual’s educational history, occupation,
and other factors. Norms are more challenging to interpret in individuals with very high
or very low levels of education and in indivi duals being tested outside their own language
or cultural background.
Criterion B relates to the individual’s leve l of independence in everyday functioning.
Individuals with major NCD will have impairment of sufficient severity so as to interfere
with independence, such that others will have to take over tasks that the individuals were
previously able to complete on their own. Individuals with mild NCD will have preserved
independence, although there may be subtle in terference with function or a report that
tasks require more effort or take more time than previously.
The distinction between major and mild NCD is inherently arbitrary, and the disorders
exist along a continuum. Precis e thresholds are therefore difficult to determine. Careful
history taking, observation, and integration wi th other findings are required, and the im-
plications of diagnosis should be considered when an individual’s clinical manifestations
lie at a boundary.
Associated Features Supporting Diagnosis
Typically the associated features that suppo rt a diagnosis of major or mild NCD will be
specific to the etiological subtype (e.g., neur oleptic sensitivity and visual hallucinations in
NCD due to Lewy body disease). Diagnostic features specific to each of the subtypes are
found in the relevant sections.
Prevalence | dsm5.pdf |
0184da59cf14-1 | found in the relevant sections.
Prevalence
The prevalence of NCD varies widely by ag e and by etiological su btype. Overall preva-
lence estimates are generally only availabl e for older population s. Among individuals
older than 60 years, prevalence increases steep ly with age, so prev alence estimates are
more accurate for narrow age bands than for br oad categories such as “over 65” (where the
mean age can vary greatly with the life expect ancy of the given population). For those eti-
ological subtypes occurring across the lifespa n, prevalence estimates for NCD are likely to
be available, if at all, only as the fraction of individuals who develop NCD among those
with the relevant condition (e.g., tr aumatic brain injury, HIV infection).
Overall prevalence estimates for dementia (which is largely congruent with major
NCD) are approximately 1%–2% at age 65 years and as high as 30% by age 85 years. The
prevalence of mild NCD is very sensitive to th e definition of the di sorder, particularly in
community settings, where evaluations are less de tailed. In addition, in contrast with clin-
ical settings, where cognitive concern must be high to seek and locate care, there may be a
less clear decline from baseline functioning. Es timates of the prevalence of mild cognitive
impairment (which is substantially congruent with mild NCD) among older individuals
are fairly variable, ranging from 2% to 10% at age 65 and 5% to 25% by age 85.
Development and Course
The course of NCD varies acro ss etiological subtypes, and this variation can be useful in
differential diagnosis. Some subtypes (e.g., those related to traumatic brain injury or | dsm5.pdf |
0184da59cf14-2 | differential diagnosis. Some subtypes (e.g., those related to traumatic brain injury or
stroke) typically begin at a specific time and (at least after initial symptoms related to in-
flammation or swelling subside) remain stat ic. Others may fluctuate over time (although
if this occurs, the possibility of delirium superimposed on NCD should be considered).
NCDs due to neurodegenerative diseases like Alzheimer’s disease or frontotemporal
lobar degeneration typically are marked by insidious onset and gradual progression, and | dsm5.pdf |
9e7af252cf07-0 | Major and Mild Neurocognitive Disorders 609
the pattern of onset of cognitive deficits and associated features helps to distinguish among
them.
NCDs with onset in childhood and adolescence may have broad repercussions for so-
cial and intellectual development, and in th is setting intellectual disability (intellectual
developmental disorder) and/or other neurod evelopmental disorders may also be diag-
nosed to capture the full diagnostic picture and ensure the provision of a broad range of
services. In older individuals, NCDs often occu r in the setting of medical illnesses, frailty,
and sensory loss, which complicate the clin ical picture for diagnosis and treatment.
When cognitive loss occurs in youth to midlife, individuals and families are likely to
seek care. NCDs are typically easiest to identify at younger ages, although in some settings
malingering or other factitious disorders may be a concern. Very late in life, cognitive
symptoms may not cause concern or may go unnoticed. In late life, mild NCD must also be
distinguished from the more mo dest deficits associated with “normal aging,” although a
substantial fraction of what has been ascribe d to normal aging likely represents prodromal
phases of various NCDs. In addition, it beco mes harder to recognize mild NCD with age
because of the increasing prevalence of me dical illness and sensory deficits. It becomes
harder to differentiate among subtypes with ag e because there are multiple potential sources
of neurocognitive decline.
Risk and Prognostic Factors
Risk factors vary not only by etiological subtype but also by age at onset within etiological
subtypes. Some subtypes are distributed thro ughout the lifespan, whereas others occur
exclusively or primarily in late life. Even within the NCDs of aging, the relative prevalence | dsm5.pdf |
9e7af252cf07-1 | varies with age: Alzheimer’s disease is unco mmon before age 60 years, and the prevalence
increases steeply thereafter, while the overa ll less common frontotemporal lobar degener-
ation has earlier onset and represents a progre ssively smaller fraction of NCDs with age.
Genetic and physiological. The strongest risk factor for major and mild NCDs is age,
primarily because age increases the risk of neurodegenerative and cerebrovascular dis-
ease. Female gender is associat ed with higher prevalence of dementia overall, and especially
Alzheimer’s disease, but this difference is larg ely, if not wholly, attr ibutable to greater lon-
gevity in females.
Culture-Related Diagnostic Issues
Individuals’ and families’ level of awareness and concern about neurocognitive symp-
toms may vary across ethnic and occupational groups. Neurocognitive symptoms are
more likely to be noticed, particularly at the mild level, in individuals who engage in com-
plex occupational, domestic, or recreational activities. In addition , norms for neuropsy-
chological testing tend to be available only for broad populations, and thus they may not
be easily applicable to individuals with less than high school ed ucation or those being
evaluated outside their primary language or culture.
Gender-Related Diagnostic Issues
Like age, culture, and occupa tion, gender issues may affect the level of concern and aware-
ness of cognitive symptoms. In addition, for la te-life NCDs, females are likely to be older,
to have more medical comorbidity, and to liv e alone, which can complicate evaluation and
treatment. In addition, there are gender differ ences in the frequency of some of the etio-
logical subtypes.
Diagnostic Markers | dsm5.pdf |
9e7af252cf07-2 | logical subtypes.
Diagnostic Markers
In addition to a careful history, neuropsych ological assessments are the key measures for
diagnosis of NCDs, particularly at the mild level, where functional changes are minimal | dsm5.pdf |
c95d788e40f4-0 | 610 Neurocognitive Disorders
and symptoms more subtle. Ideally, individu als will be referred for formal neuropsycho-
logical testing, which will provide a quantitative assessment of all relevant domains and
thus help with diagnosis; provide guidance to the family on areas where the individual
may require more support; and serve as a be nchmark for further decline or response to
therapies. When such testing is unavailable or not feasible, the brief assessments in Table 1
can provide insight into each domain. More glob al brief mental status tests may be helpful
but may be insensitive, particul arly to modest changes in a single domain or in those with
high premorbid abilities, and may be overly sensitive in those with low premorbid abilities.
In distinguishing among etiological subtypes, additional diagnostic markers may
come into play, particularly neuroimaging studies such as magnetic resonance imaging
scans and positron emission tomography scans. In addition, specific markers may be in-
volved in the assessment of specific subtypes and may become more important as addi-
tional research findings accumulate over time, as discussed in the relevant sections.
Functional Consequences of
Major and Mild Neurocognitive Disorders
By definition, major and mild NCDs affect func tioning, given the central role of cognition in
human life. Thus, the criteria for the disorder s, and the threshold for differentiating mild
from major NCD, are based in part on functi onal assessment. Within major NCD there is a
broad range of functional impairment, as implem ented in the severity specifiers. In addition,
the specific functions that are compromised can help identify the cognitive domains affected,
particularly when neuropsychological testing is not available or is difficult to interpret.
Differential Diagnosis
Normal cognition. The differential diagnosis between normal cognition and mild NCD, | dsm5.pdf |
c95d788e40f4-1 | Differential Diagnosis
Normal cognition. The differential diagnosis between normal cognition and mild NCD,
as between mild and major NCD, is challenging because the boundaries are inherently ar-
bitrary. Careful history taking and objective as sessment are critical to these distinctions. A
longitudinal evaluation using quantified a ssessments may be key in detecting mild NCD.
Delirium. Both mild and major NCD may be difficult to distinguish from a persistent de-
lirium, which can co-occur. Careful assessment of attention and arou sal will help to make
the distinction.
Major depressive disorder. The distinction between mild NCD and major depressive
disorder, which may co-occur with NCD, can also be challenging. Specific patterns of cog-
nitive deficits may be helpfu l. For example, consistent memory and executive function
deficits are typical of Alzheimer’s disease, whereas nonspecific or more variable perfor-
mance is seen in major depression. Alternat ively, treatment of the depressive disorder
with repeated observation over time may be required to make the diagnosis.
Specific learning disorder and other neurodevelopmental disorders. A careful clari-
fication of the individual’s baseline status will help distinguish an NCD from a specific
learning disorder or other neurodevelopmental disorders. Additional issues may enter the
differential for specific etiological subtypes, as described in the relevant sections.
Comorbidity
NCDs are common in older individuals and thus often co-occur with a wide variety of age-
related diseases that may co mplicate diagnosis or treatme nt. Most notable of these is
delirium, for which NCD increases the risk. In older individuals, a delirium during hos- | dsm5.pdf |
c95d788e40f4-2 | pitalization is, in many cases, the first time that an NCD is noticed, although a careful his-
tory will often reveal evidence of earlier decline. Mixed NC Ds are also common in older
individuals, as many etiological entities incre ase in prevalence with age. In younger indi-
viduals, NCD often co-occurs with neurodevelop mental disorders; for example, a head in- | dsm5.pdf |
f51e91283e7c-0 | Major or Mild Neurocognitive Disorder Due to Alzheimer’s Disease 611
jury in a preschool child may also lead to significant developmental and learning issues.
Additional comorbidity of NCD is often relate d to the etiological subtype, as discussed in
the relevant sections.
Major or Mild Neurocognitive Disorder
Due to Alzheimer’s Disease
Diagnostic Criteria
A. The criteria are met for major or mild neurocognitive disorder.
B. There is insidious onset and gradual progression of impairment in one or more cognitive
domains (for major neurocognitive disorder, at least two domains must be impaired).
C. Criteria are met for either probable or possible Alzheimer’s disease as follows:
For major neurocognitive disorder:
Probable Alzheimer’s disease is diagnosed if either of the following is present; oth-
erwise, possible Alzheimer’s disease should be diagnosed.
1. Evidence of a causative Alzheimer’s disease genetic mutation from family history
or genetic testing.
2. All three of the following are present:
a. Clear evidence of decline in memory and learning and at least one other cogni-
tive domain (based on detailed history or serial neuropsychological testing).
b. Steadily progressive, gradual decline in cognition, without extended plateaus.
c. No evidence of mixed etiology (i.e., absence of other neurodegenerative or
cerebrovascular disease, or another neurological, mental, or systemic disease
or condition likely contributing to cognitive decline).
For mild neurocognitive disorder:
Probable Alzheimer’s disease is diagnosed if there is evidence of a causative Alz-
heimer’s disease genetic mutation from either genetic testing or family history.
Possible Alzheimer’s disease is diagnosed if there is no evidence of a causative Alz-
heimer’s disease genetic mutation from either genetic testing or family history, and all | dsm5.pdf |
f51e91283e7c-1 | heimer’s disease genetic mutation from either genetic testing or family history, and all
three of the following are present:
1. Clear evidence of decline in memory and learning.
2. Steadily progressive, gradual decline in cognition, without extended plateaus.
3. No evidence of mixed etiology (i.e., absence of other neurodegenerative or cere-
brovascular disease, or another neurologica l or systemic disease or condition likely
contributing to cognitive decline).
D. The disturbance is not better explained by cerebrovascular disease, another neurode-
generative disease, the effects of a substance, or another mental, neurological, or sys-
temic disorder.
Coding note: For probable major neurocognitive disorder due to Alzheimer’s disease,
with behavioral disturbance, code first 331.0 (G30.9) Alzheimer’s disease, followed by
294.11 (F02.81) major neurocognitive disorder due to Alzheimer’s disease. For probable
neurocognitive disorder due to Alzheimer’s disease, without behavioral disturbance, code
first 331.0 (G30.9) Alzheimer’s disease, followed by 294.10 (F02.80) major neurocognitive
disorder due to Alzheimer’s disease, without behavioral disturbance.
For possible major neurocognitive disor der due to Alzheimer’s disease, code 331.9
(G31.9) possible major neurocognitive disorder due to Alzheimer’s disease. ( Note: Do not
use the additional code for Alzheimer’s disease. Behavioral disturbance cannot be coded
but should still be indicated in writing.) | dsm5.pdf |
b55b7c071645-0 | 612 Neurocognitive Disorders
For mild neurocognitive disorder due to Alzheimer’s disease, code 331.83 (G31.84).
(Note: Do not use the additional code for Alzheimer’s disease. Behavioral disturbance
cannot be coded but should still be indicated in writing.)
Diagnostic Features
Beyond the neurocognitive disorder (NCD) syndro me (Criterion A), the core features of ma-
jor or mild NCD due to Alzheimer’s disease include an insidious onset and gradual pro-
gression of cognitive and beha vioral symptoms (Criterion B) . The typical presentation is
amnestic (i.e., with impairment in memory and learning). Unusual nonamnestic presen-
tations, particularly visuospa tial and logopenic aphasic vari ants, also exist. At the mild
NCD phase, Alzheimer’s disease manifests typically with impairment in memory and learn-
ing, sometimes accompanied by deficits in executive function. At the major NCD phase,
visuoconstructional/perceptual -motor ability and language w ill also be impaired, partic-
ularly when the NCD is moderate to severe. Social cognition tends to be preserved until
late in the course of the disease.
A level of diagnostic certainty must be sp ecified denoting Alzheimer’s disease as the
“probable” or “possible” etiology (Criterion C). Probable Alzheimer’s disease is diagnosed in
both major and mild NCD if there is evidence of a causative Alzheimer’s disease gene, ei-
ther from genetic testing or from an autosomal dominant family history coupled with au-
topsy confirmation or a genetic test in an affected family member. For major NCD, a
typical clinical picture, without extended plat eaus or evidence of mixed etiology, can also | dsm5.pdf |
b55b7c071645-1 | typical clinical picture, without extended plat eaus or evidence of mixed etiology, can also
be diagnosed as due to probab le Alzheimer’s disease. For mild NCD, given the lesser de-
gree of certainty that the deficits will prog ress, these features are only sufficient for a
possible Alzheimer’s etiology. If the etiology appe ars mixed, mild NCD due to multiple eti-
ologies should be diagnosed. In any case, for both mild and major NCD due to Alzhei-
mer’s disease, the clinical features must no t suggest another primary etiology for the NCD
(Criterion D).
Associated Features Supporting Diagnosis
In specialty clinical settings, approximately 80% of individuals with major NCD due to
Alzheimer’s disease have behavioral and psyc hological manifestations; these features are
also frequent at the mild NCD stage of impa irment. These symptoms are as or more dis-
tressing than cognitive manifestations and ar e frequently the reason that health care is
sought. At the mild NCD stage or the mildest level of majo r NCD, depression and/or ap-
athy are often seen. With moderately severe major NCD, psychotic fe atures, irritability,
agitation, combativeness, and wandering are common. Late in the illness, gait distur-
bance, dysphagia, incontinence, myoc lonus, and seizures are observed.
Prevalence
The prevalence of overall deme ntia (major NCD) rises steeply with age. In high-income
countries, it ranges from 5% to 10% in the seventh decade to at least 25% thereafter. U.S.
census data estimates suggest that approxim ately 7% of individuals diagnosed with Alz- | dsm5.pdf |
b55b7c071645-2 | heimer’s disease are between ages 65 and 74 years, 53% are between ages 75 and 84 years,
and 40% are 85 years and older. The percentage of dementias attributable to Alzheimer’s
disease ranges from about 60% to over 90%, depending on the setting and diagnostic cri-
teria. Mild NCD due to Alzheimer’s disease is likely to represent a substantial fraction of
mild cognitive impairment (MCI) as well.
Development and Course
Major or mild NCD due to Alzheimer’s disease progresses gradually, sometimes with
brief plateaus, through severe dementia to death. The mean duration of survival after di- | dsm5.pdf |
2eac55f64161-0 | Major or Mild Neurocognitive Disorder Due to Alzheimer’s Disease 613
agnosis is approximately 10 years, reflecting th e advanced age of the majority of individ-
uals rather than the course of the disease; some individuals can live with the disease for as
long as 20 years. Late-stage individuals are eventually mute and bedbound. Death most
commonly results from aspiration in those who survive through the full course. In mild
NCD due to Alzheimer’s disease, impairments increase over time, and functional status
gradually declines until symptoms reach the threshold for the diagnosis of major NCD.
The onset of symptoms is usually in the eighth and ninth decades; early-onset forms
seen in the fifth and sixth decades are often related to known causative mutations. Symp-
toms and pathology do not differ markedly at different onset ages. However, younger in-
dividuals are more likely to survive the full course of the disease, while older individuals
are more likely to have numerous medical co morbidities that affect the course and man-
agement of the illness. Diagnostic complexity is higher in older adults because of the in-
creased likelihood of comorbid me dical illness and mixed pathology.
Risk and Prognostic Factors
Environmental. Traumatic brain injury increases risk for major or mild NCD due to Alz-
heimer’s disease.
Genetic and physiological. Age is the strongest risk factor for Alzheimer’s disease. The
genetic susceptibility polymorphism apolipoprotein E4 increases risk and decreases age
at onset, particularly in homozygous individu als. There are also extremely rare causative
Alzheimer’s disease genes. Individuals with Down’s syndrome (trisomy 21) develop Alz-
heimer’s disease if they survive to midlife. Multiple vascular risk factors influence risk for | dsm5.pdf |
2eac55f64161-1 | heimer’s disease if they survive to midlife. Multiple vascular risk factors influence risk for
Alzheimer’s disease and may act by increasing cerebrovascular pathology or also through
direct effects on Alzheimer pathology.
Culture-Related Diagnostic Issues
Detection of an NCD may be more difficult in cultural and socioeconomic settings where
memory loss is considered normal in old age, where older adults face fewer cognitive de-
mands in everyday life, or where very low educational levels pose greater challenges to
objective cognitive assessment.
Diagnostic Markers
Cortical atrophy, amyloid-predominant neuritic plaques, and tau-predominant neurofibril-
lary tangles are hallmarks of the pathological diagnosis of Alzheimer’s disease and may be
confirmed via postmortem histopathological examination. For early-onset cases with auto-
somal dominant inheritance, a mutation in on e of the known causative Alzheimer’s disease
genes—amyloid precursor protein (APP), presen ilin 1 (PSEN1), or presenilin 2 (PSEN2)—
may be involved, and genetic testing for such mutations is commercially available, at least
for PSEN1. Apolipoprotein E4 cannot serve as a diagnostic marker because it is only a risk
factor and neither necessary nor sufficient for disease occurrence.
Since amyloid beta-42 deposition in the br ain occurs early in the pathophysiological
cascade, amyloid-based diagnostic tests such as amyloid imaging on brain positron emis-
sion tomography (PET) scans and reduced leve ls of amyloid beta-42 in the cerebrospinal
fluid (CSF) may have diagnostic value. Signs of neuronal injury, such as hippocampal and
temporoparietal cortical atrophy on a magnetic resonance image scan, temporoparietal | dsm5.pdf |
2eac55f64161-2 | temporoparietal cortical atrophy on a magnetic resonance image scan, temporoparietal
hypometabolism on a fluorodeoxyglucose PET sc an, and evidence for elevated total tau
and phospho-tau levels in CSF, provide evidence of neuronal damage but are less specific
for Alzheimer’s disease. At present, these biomarkers are not fully validated, and many
are available only in tertiary care settings. However, some of them, along with novel bio-
markers, will likely move into wider clinical practice in the coming years. | dsm5.pdf |
e6a3600e33dd-0 | 614 Neurocognitive Disorders
Functional Consequences of Major or Mild
Neurocognitive Disorder Du e to Alzheimer’s Disease
The prominence of memory loss can cause significant difficulties relatively early in the
course. Social cognition (and thus social func tioning) and procedural memory (e.g., danc-
ing, playing musical instruments) may be relatively preserved for extended periods.
Differential Diagnosis
Other neurocognitive disorders. Major and mild NCDs due to other neurodegenera-
tive processes (e.g., Lewy body disease, fron totemporal lobar degene ration) share the in-
sidious onset and gradual decline caused by Alzheimer’s disease but have distinctive core
features of their own. In major or mild vascular NCD, th ere is typically history of stroke
temporally related to the onset of cognitive impairment, and infarcts or white matter hy-
perintensities are judged sufficient to accoun t for the clinical picture. However, particu-
larly when there is no clear hi story of stepwise decline, ma jor or mild vascular NCD can
share many clinical features with Alzheimer’s disease.
Other concurrent, active neurological or systemic illness. Other neurological or sys-
temic illness should be considered if there is an appropriate temporal relationship and
severity to account for the clinical picture. At the mild NCD level, it may be difficult to dis-
tinguish an Alzheimer’s disease etiology from that of another medical condition (e.g., thy-
roid disorders, vitamin B12 deficiency).
Major depressive disorder. Particularly at the mild NCD level, the differential diagnosis
also includes major depression. The presence of depression may be associated with re-
duced daily functioning and poor concentration that may resemble an NCD, but improve-
ment with treatment of depression may be useful in making the distinction.
Comorbidity | dsm5.pdf |
e6a3600e33dd-1 | ment with treatment of depression may be useful in making the distinction.
Comorbidity
Most individuals with Alzheimer’s disease are elderly and have multiple medical conditions
that can complicate diagnosis and influence th e clinical course. Major or mild NCD due to
Alzheimer’s disease commonly co-occurs with cerebrovascular disease, which contributes
to the clinical picture. When a comorbid condition contribute s to the NCD in an individual
with Alzheimer’s disease, then NCD due to multiple etiologies should be diagnosed.
Major or Mild Frontotemporal
Neurocognitive Disorder
Diagnostic Criteria
A. The criteria are met for major or mild neurocognitive disorder.
B. The disturbance has insidious onset and gradual progression.
C. Either (1) or (2):
1. Behavioral variant:
a. Three or more of the following behavioral symptoms:
i. Behavioral disinhibition.
ii. Apathy or inertia.
iii. Loss of sympathy or empathy.
iv. Perseverative, stereotyped or compulsive/ritualistic behavior.
v. Hyperorality and dietary changes.
b. Prominent decline in social cognition and/or executive abilities. | dsm5.pdf |
258fe4e80718-0 | Major or Mild Frontotemporal Neurocognitive Disorder 615
2. Language variant:
a. Prominent decline in language ability, in the form of speech production, word
finding, object naming, grammar, or word comprehension.
D. Relative sparing of learning and memory and perceptual-motor function.
E. The disturbance is not better explained by cerebrovascular disease, another neurode-
generative disease, the effects of a substance, or another mental, neurological, or sys-
temic disorder.
Probable frontotemporal neurocognitive disorder is diagnosed if either of the following
is present; otherwise, possible frontotemporal neurocognitive disorder should be di-
agnosed:
1. Evidence of a causative frontotemporal neur ocognitive disorder genetic mutation, from
either family history or genetic testing.
2. Evidence of disproportionate frontal and/or temporal lobe involvement from neuroim-
aging.
Possible frontotemporal neurocognitive disorder is diagnosed if there is no evidence
of a genetic mutation, and neuroimaging has not been performed.
Coding note: For probable major neurocognitive disorder due to frontotemporal lobar de-
generation, with behavioral disturbance, code first 331.19 (G31.09) frontotemporal dis-
ease, followed by 294.11 (F02.81) probable major neurocognitive disorder due to
frontotemporal lobar degeneration, with behavioral disturbance. For probable major neu-
rocognitive disorder due to frontotemporal lobar degeneration, without behavioral distur-
bance, code first 331.19 (G31.09) frontotemporal disease, followed by 294.10 (F02.80)
probable major neurocognitive disorder due to frontotemporal lobar degeneration, without | dsm5.pdf |
258fe4e80718-1 | probable major neurocognitive disorder due to frontotemporal lobar degeneration, without
behavioral disturbance.
For possible major neurocognitive disorder due to frontotemporal lobar degeneration, code
331.9 (G31.9) possible major neurocognitive disorder due to frontotemporal lobar degen-
eration. ( Note: Do not use the additional code for frontote mporal disease. Behavioral distur-
bance cannot be coded but should still be indicated in writing.)
For mild neurocognitive disorder due to frontotemporal lobar degeneration, code 331.83
(G31.84). (Note: Do not use the additional code for frontotemporal disease. Behavioral
disturbance cannot be coded but should still be indicated in writing.)
Diagnostic Features
Major or mild frontotemporal neurocognitive disorder (NCD) comprises a number of syn-
dromic variants characterized by the progressi ve development of behavioral and personality
change and/or language impairment. The behavioral variant and three language variants (se-
mantic, agrammatic/nonfluent, and logopenic) ex hibit distinct patterns of brain atrophy and
some distinctive neuropathology. The criteria must be met for either the behavioral or the lan-
guage variant to make the diagnosis, but many individuals present with features of both.
Individuals with behavioral-variant major or mild frontotemporal NCD present with
varying degrees of apathy or disinhibition. They may lose interest in socialization, self-
care, and personal responsibilities, or displa y socially inappropriate behaviors. Insight is
usually impaired, and this often delays medical consultation. The first referral is often to a
psychiatrist. Individuals may develop changes in social style, and in religious and political | dsm5.pdf |
258fe4e80718-2 | psychiatrist. Individuals may develop changes in social style, and in religious and political
beliefs, with repetitive movements, hoarding, changes in ea ting behavior, and hyperoral-
ity. In later stages, loss of sphincter contro l may occur. Cognitive decline is less prominent,
and formal testing may show relatively few deficits in the early stages. Common neuro-
cognitive symptoms are lack of planning and organization, distractib ility, and poor judg-
ment. Deficits in executive function, such as poor performance on tests of mental | dsm5.pdf |
0d579a69ebcf-0 | 616 Neurocognitive Disorders
flexibility, abstract reasoning, and response inhibition, are present, but learning and mem-
ory are relatively spared, and perceptual-mot or abilities are almost always preserved in
the early stages.
Individuals with language-variant major or mild frontotemporal NCD present with pri-
mary progressive aphasia with gradual onse t, with three subtypes commonly described:
semantic variant, agrammatic/n onfluent variant, and logopenic variant, and each variant
has distinctive features and corresponding neuropathology.
“Probable” is distinguished from “possibl e” frontotemporal NCD by the presence of
causative genetic factors (e.g., mutations in the gene coding for microtubule-associated pro-
tein tau) or by the presence of distinctive at rophy or reduced activity in frontotemporal re-
gions on structural or functional imaging.
Associated Features Supporting Diagnosis
Extrapyramidal features may be prominent in some cases, with an overlap with syn-
dromes such as progressive supranuclear pa lsy and corticobasal dege neration. Features of
motor neuron disease may be present in some cases (e.g., muscle atrophy, weakness). A
subset of individuals develop visual hallucinations.
Prevalence
Major or mild frontotemporal NCD is a common cause of early-onset NCD in individuals
younger than 65 years. Population prevalen ce estimates are in th e range of 2–10 per
100,000. Approximately 20%–25% of cases of frontotemporal NCD occur in individuals
older than 65 years. Frontotemporal NCD accoun ts for about 5% of all cases of dementia in | dsm5.pdf |
0d579a69ebcf-1 | unselected autopsy series. Prevalence estima tes of behavioral variant and semantic lan-
guage variant are higher among males, and prevalence estimates of nonfluent language
variant are higher among females.
Development and Course
Individuals with major or m ild frontotemporal NCD commonly present in the sixth de-
cade of life, although the age at onset varies from the third to the ninth decades. The dis-
ease is gradually progressive, with median survival being 6–11 years after symptom onset
and 3–4 years after diagnosis. Survival is shorter and decline is faster in major or mild fron-
totemporal NCD than in typical Alzheimer’s disease.
Risk and Prognostic Factors
Genetic and physiological. Approximately 40% of individuals with major or mild fron-
totemporal NCD have a family history of early-onset NCD, and approximately 10% show an
autosomal dominant inheritance pattern. A number of genetic factors have been identified,
such as mutations in the gene encoding the microtubule asso ciated protein tau (MAPT), the
granulin gene (GRN), and the C9ORF72 gene. A number of families with causative muta-
tions have been identified (see the section “D iagnostic Markers” for th is disorder), but many
individuals with known familial transmission do not have a known mutation. The presence
of motor neuron disease is associated with a more rapi d deterioration.
Diagnostic Markers
Computed tomography (CT) or structural magnetic resonance imaging (MRI) may show
distinct patterns of atrophy. In behavioral-variant major or mild frontotemporal NCD,
both frontal lobes (especially the medial frontal lobes) and the anterior temporal lobes are | dsm5.pdf |
0d579a69ebcf-2 | both frontal lobes (especially the medial frontal lobes) and the anterior temporal lobes are
atrophic. In semantic language–variant majo r or mild frontotemporal NCD, the middle,
inferior, and anterior temporal lobes are at rophic bilaterally but asymmetrically, with the | dsm5.pdf |
e27a7bf27674-0 | Major or Mild Frontotemporal Neurocognitive Disorder 617
left side usually being more affected. Nonfluent la nguage–variant major or mild fronto-
temporal NCD is associated with predominantly left posterior fr ontal-insular atrophy.
The logopenic variant of major or mild fron totemporal NCD is associated with predomi-
nantly left posterior perisylvian or parietal atrophy. Functional imaging demonstrates hy-
poperfusion and/or cortical hypometabolism in the corresponding brain regions, which
may be present in the early stages in the abse nce of structural abno rmality. Emerging bio-
markers for Alzheimer’s disease (e.g., cerebrospinal fluid amyl oid-beta and tau levels, and
amyloid imaging) may help in the differential diagnosis, but the dist inction from Alzhei-
mer’s disease can remain difficult (the logopeni c variant is in fact often a manifestation of
Alzheimer’s disease).
In familial cases of frontotemporal NCD, the identification of genetic mutations may
help confirm the diagnosis. Mutations associated with frontote mporal NCD include
the genes encoding microtubule-associated protein tau (MAPT) and granulin (GRN),
C9ORF72, transactive response DNA-binding protein of 43 kDa (TDP-43, or TARDBP),
valosin-containing protein (V CP), chromatin modifying protein 2B (CHMP2B), and fused
in sarcoma protein (FUS).
Functional Consequences of Major or Mild
Frontotemporal Neurocognitive Disorder
Because of the relative early age at onset of the disorder, the disorder oftens affects work-
place and family life. Because of the involvem ent of language and/or behavior, function is | dsm5.pdf |
e27a7bf27674-1 | often more severely impaired relatively early in the course . For individuals with the be-
havioral variant, prior to diagnostic clarific ation there may be significant family disrup-
tion, legal involvement, and problems in the workplace because of socially inappropriate
behaviors. The functional impairment due to behavioral change and language dysfunc-
tion, which can include hyperorality, impuls ive wandering, and other dishinhibited be-
haviors, may far exceed that due to the co gnitive disturbance and may lead to nursing
home placement or institutionalization. Thes e behaviors can be severely disruptive, even
in structured care settings, particularly wh en the individuals are otherwise healthy, non-
frail, and free of othe r medical comorbidities.
Differential Diagnosis
Other neurocognitive disorders. Other neurodegenerative diseases may be distinguished
from major or mild frontotemporal NCD by th eir characteristic features. In major or mild
NCD due to Alzheimer’s disease, decline in learning and memory is an early feature.
However, 10%–30% of patients presenting with a syndrome suggestive of major or mild
frontotemporal NCD are found at autopsy to have Alzheimer’s disease pathology. This oc-
curs more frequently in individuals who present with progressi ve dysexecutive syn-
dromes in the absence of behavioral changes or movement disorder or in those with the
logopenic variant.
In major or mild NCD with Lewy bodies, co re and suggestive features of Lewy bodies
must be present. In major or mild NCD due to Parkinson’s disease, spontaneous parkin-
sonism emerges well befo re the cognitive decline. In majo r or mild vascular NCD, depend-
ing on affected brain regions, there may also be loss of executive ability and behavioral | dsm5.pdf |
e27a7bf27674-2 | ing on affected brain regions, there may also be loss of executive ability and behavioral
changes such as apathy, and this disorder should be considered in the differential diagno-
sis. However, history of a cerebrovascular event is temporally related to the onset of cog-
nitive impairment in major or mild vascular NCD, and neuroimaging reveals infarctions
or white matter lesions sufficient to account for the clinical picture.
Other neurological conditions. Major or mild frontotemporal NCD overlaps with pro-
gressive supranuclear palsy, corticobasal degeneration, and motor neuron disease
clinically as well as pathologically. Progressive supranuclear palsy is characterized by | dsm5.pdf |
acd424b40587-0 | 618 Neurocognitive Disorders
supranuclear gaze palsies and axial-predominant parkinsoni sm. Pseudobulbar signs may
be present, and retropulsion is often prom inent. Neurocognitive assessment shows psy-
chomotor slowing, poor working memory, and executive dysfunction. Corticobasal degen-
eration presents with asymmetr ic rigidity, limb apraxia, postural instability, myoclonus,
alien limb phenomenon, and cortical sensory loss. Many individuals wi th behavioral-variant
major or mild frontotemporal NCD show features of motor neuron disease, which tend to
be mixed upper and predominantly lower motor neuron disease.
Other mental disorders and medical conditions. Behavioral-variant major or mild fron-
totemporal NCD may be mistaken for a primary mental disorder, such as major depression,
bipolar disorders, or schizophrenia, and individuals with this variant often present initially
to psychiatry. Over time, the development of progressive neurocognitive difficulties will
help to make the distinction. A careful medi cal evaluation will help to exclude treatable
causes of NCDs, such as meta bolic disturbances, nutritional deficiencies, and infections.
Major or Mild Neurocognitive Disorder
With Lewy Bodies
Diagnostic Criteria
A. The criteria are met for major or mild neurocognitive disorder.
B. The disorder has an insidious onset and gradual progression.
C. The disorder meets a combination of core diagnostic features and suggestive diagnos-
tic features for either probable or possibl e neurocognitive disorder with Lewy bodies.
For probable major or mild neurocognitive disorder with Lewy bodies, the indi-
vidual has two core features, or one suggestive feature with one or more core features. | dsm5.pdf |
acd424b40587-1 | vidual has two core features, or one suggestive feature with one or more core features.
For possible major or mild neurocognitive disorder with Lewy bodies, the individ-
ual has only one core feature, or one or more suggestive features.
1. Core diagnostic features:
a. Fluctuating cognition with pronounced variations in attention and alertness.
b. Recurrent visual hallucinations that are well formed and detailed.
c. Spontaneous features of parkinsonism, with onset subsequent to the develop-
ment of cognitive decline.
2. Suggestive diagnostic features:
a. Meets criteria for rapid eye movement sleep behavior disorder.
b. Severe neuroleptic sensitivity.
D. The disturbance is not better explained by cerebrovascular disease, another neurode-
generative disease, the effects of a substance, or another mental, neurological, or sys-
temic disorder.
Coding note: For probable major neurocognitive disorder with Lewy bodies, with behav-
ioral disturbance, code first 331.82 (G31.83) Lewy body disease, followed by 294.11
(F02.81) probable major neurocognitive disorder with Lewy bodies, with behavioral distur-
bance. For probable major neurocognitive disorder with Lewy bodies, without behavioral
disturbance, code first 331.82 (G31.83) Lewy body disease, followed by 294.10 (F02.80)
probable major neurocognitive disorder with Lewy bodies, without behavioral disturbance.
For possible major neurocognitive disorder with Lewy bodies, code 331.9 (G31.9) possible
major neurocognitive disorder with Lewy bodies. ( Note: Do not use the additional code for | dsm5.pdf |
acd424b40587-2 | Lewy body disease. Behavioral disturbance cannot be coded but should still be indicated
in writing.) | dsm5.pdf |
3d8530893195-0 | Major or Mild Neurocognitive Disorder With Lewy Bodies 619
For mild neurocognitive disorder with Lewy bodies, code 331.83 (G31.84). ( Note: Do not
use the additional code for Lewy body disease. Behavioral disturbance cannot be coded
but should still be indicated in writing.)
Diagnostic Features
Major or mild neurocognitive disorder with Lewy bodies (NCDLB), in the case of major
neurocognitive disorder (NCD), corresponds to the condition known as dementia with
Lewy bodies (DLB). The disorder includes not only progressive cognitive impairment
(with early changes in complex attention and ex ecutive function rather than learning and
memory) but also recurrent complex visual hallucinations; and concurrent symptoms of
rapid eye movement (REM) sleep behavior diso rder (which can be a very early manifes-
tation); as well as hallucinations in other sensory modalities, depression, and delusions.
The symptoms fluctuate in a pattern that can resemble a delirium, but no adequate under-
lying cause can be found. The variable pres entation of NCDLB symptoms reduces the like-
lihood of all symptoms being observed in a brief clinic visit and necessitates a thorough
assessment of caregive r observations. The use of assessment scales specifically designed to
assess fluctuation may aid in diagnosis. Anot her core feature is spontaneous parkinson-
ism, which must begin after the onset of cognit ive decline; by convention, major cognitive
deficits are observed at least 1 year befo re the motor symptoms. The parkinsonism must
also be distinguished from neuroleptic-induce d extrapyramidal signs. Accurate diagnosis
is essential to safe treatment planning, as up to 50% of individuals with NCDLB have se- | dsm5.pdf |
3d8530893195-1 | vere sensitivity to neuroleptic drugs, and these medications should be used with extreme
caution in managing the psychotic manifestations.
The diagnosis of mild NCDLB is appropriate for individuals who present with the core
or suggestive features at a stage when cognitive or functional impairments are not of suf-
ficient severity to fulfill criteria for major NC D. However, as for all mild NCDs, there will
often be insufficient ev idence to justify any si ngle etiology, and use of the unspecified di-
agnosis is most appropriate.
Associated Features Supporting Diagnosis
Individuals with NCDLB frequently experience repeated falls and syncope and transient
episodes of unexplained loss of consciousness. Autonomic dysfunction, such as ortho-
static hypotension and urinary incontinen ce, may be observed. Auditory and other
nonvisual hallucinations are common, as are sy stematized delusions, delusional misiden-
tification, and depression.
Prevalence
The few population-based prev alence estimates for NCDLB available range from 0.1% to
5% of the general elderly popu lation, and from 1.7% to 30.5 % of all dementia cases. In
brain bank (autopsy) series, the pathological lesions known as Lewy bodies are present in
20%–35% of cases of dementia. The male-to- female ratio is approximately 1.5:1.
Development and Course
NCDLB is a gradually progressive disorder with insidious onset. However, there is often
a prodromal history of confusional episodes (delirium) of acute onset, often precipitated
by illness or surgery. The distinction between NCDLB, in which Lewy bodies are primar- | dsm5.pdf |
3d8530893195-2 | ily cortical in location, and major or mild NC D due to Parkinson’s disease, in which the pa-
thology is primarily in the basal ganglia, is the order in which th e cognitive and motor
symptoms emerge. In NCDLB, th e cognitive decline is manifest ed early in the course of ill-
ness, at least a year before the onset of mo tor symptoms (see the section “Differential Di- | dsm5.pdf |
51c1f56013e3-0 | 620 Neurocognitive Disorders
agnosis” for this disorder). Disease course may be characterized by occasional plateaus
but eventually progresses through severe deme ntia to death. Average duration of survival
is 5–7 years in clinical series. Onset of symptoms is typically observed from the sixth
through the ninth decades of life, with most cases having their onset when affected indi-
viduals are in their mid-70s.
Risk and Prognostic Factors
Genetic and physiological. Familial aggregation may occur, and several risk genes have
been identified, but in most cases of NCDLB, there is no family history.
Diagnostic Markers
The underlying neurodegenerative disease is primarily a synucleinopathy due to alpha-
synuclein misfolding and aggregation. Cognitiv e testing beyond the use of a brief screen-
ing instrument may be necessary to define de ficits clearly. Assessment scales developed to
measure fluctuation can be useful. The associated condition REM sl eep behavior disorder
may be diagnosed through a formal sleep study or identified by questioning the patient or
informant about relevant symptoms. Neurolep tic sensitivity (challenge) is not recom-
mended as a diagnostic marker but raises suspicion of NCDLB if it occurs. A diagnosti-
cally suggestive feature is low striatal dopa mine transporter uptake on single photon
emission computed tomography (SPECT) or positron emission tomography (PET) scan.
Other clinically useful markers potentially include relative preservation of medial tempo-
ral structures on computed tomography (C T)/magnetic resonance imaging (MRI) brain
scan; reduced striatal dopamine transporter uptake on SPECT/PET scan; generalized low
uptake on SPECT/PET perfusion scan with re duced occipital activity; abnormal (low up- | dsm5.pdf |
51c1f56013e3-1 | take) MIBG myocardial scintigraphy suggest ing sympathetic denervation; and prominent
slow-wave activity on the electroencephalo gram with temporal lobe transient waves.
Functional Consequences of Major or Mild
Neurocognitive Disord er With Lewy Bodies
Individuals with NCDLB are more functionally impaired than would be expected for their
cognitive deficits when contrasted to individuals with other neurodegenerative diseases,
such as Alzheimer’s disease. This is largely a result of motor and autonomic impairments,
which cause problems with toileting, transfer ring, and eating. Sleep disorders and prom-
inent psychiatric symptoms may also add to functional di fficulties. Consequently, the qual-
ity of life of individuals with NCDLB is often significantly worse than that of individuals
with Alzheimer’s disease.
Differential Diagnosis
Major or mild neurocognitive diso rder due to Parkinson’s disease. A key differenti-
ating feature in clinical diagnosis is the tempor al sequence in which the parkinsonism and
the NCD appear. For NCD due to Parkinson’s disease, the individual must develop cog-
nitive decline in the context of established Parkinson’s disease; by convention, the decline
should not reach the stage of major NCD until at least 1 year after Parkinson’s is diagnosed.
If less than a year has passed since the onset of motor symptoms, the diagnosis is NCDLB.
This distinction is clearer at the major NCD level than at the mild NCD level.
The timing and sequence of parkinsonism an d mild NCD may be more difficult to de-
termine because the onset and clinical presentation can be ambiguous, and unspecified
mild NCD should be diagnosed if the othe r core and suggestive features are absent. | dsm5.pdf |
8c58997002e8-0 | Major or Mild Vascular Neurocognitive Disorder 621
Comorbidity
Lewy body pathology frequent ly coexists with Alzheimer’s disease and cerebrovascular
disease pathology, particularly among the olde st age groups. In Alzheimer’s disease, there
is concomitant synuclein pathology in 60% of cases (if amygdala-restricted cases are in-
cluded). In general, there is a higher rate of Lewy body pathology in individuals with de-
mentia than in older individuals without dementia.
Major or Mild Vascular Neurocognitive Disorder
Diagnostic Criteria
A. The criteria are met for major or mild neurocognitive disorder.
B. The clinical features are consistent with a vascular etiology, as suggested by either of
the following:
1. Onset of the cognitive deficits is temporally related to one or more cerebrovascular
events.
2. Evidence for decline is prominent in complex attention (including processing
speed) and frontal-executive function.
C. There is evidence of the presence of cere brovascular disease from history, physical
examination, and/or neuroimaging consider ed sufficient to account for the neurocog-
nitive deficits.
D. The symptoms are not better explained by another brain disease or systemic disorder.
Probable vascular neurocognitive disorder is diagnosed if one of the following is pres-
ent; otherwise possible vascular neurocognitive disorder should be diagnosed:
1. Clinical criteria are supported by neuroimaging evidence of significant parenchymal in-
jury attributed to cerebrovascula r disease (neuroimaging-supported).
2. The neurocognitive syndrome is temporally related to one or more documented cere-
brovascular events.
3. Both clinical and genetic (e.g., cerebral aut osomal dominant arteriopathy with subcortical | dsm5.pdf |
8c58997002e8-1 | infarcts and leukoencephalopathy) evidence of cerebrovascular disease is present.
Possible vascular neuroc ognitive disorder is diagnosed if the clinical criteria are met
but neuroimaging is not available and the tem poral relationship of the neurocognitive syn-
drome with one or more cerebrovascular events is not established.
Coding note: For probable major vascular neurocognitive disorder, with behavioral dis-
turbance, code 290.40 (F01.51). For probable major vascular neurocognitive disorder,
without behavioral disturbance, code 290.40 (F01.50). For possible major vascular neuro-
cognitive disorder, with or without behavioral disturbance, code 331.9 (G31.9). An addi-
tional medical code for the cerebrovascular disease is not needed.
For mild vascular neurocognitive disorder, code 331.83 (G31.84). ( Note: Do not use an
additional code for the vascular disease. Behavioral disturbance cannot be coded but
should still be indicated in writing.)
Diagnostic Features
The diagnosis of major or mild vascular neurocognitive diso rder (NCD) requires the es-
tablishment of an NCD (Criterion A) and the determination that cere brovascular disease is
the dominant if not exclusive pathology that accounts for the cognitive deficits (Criteria B
and C). Vascular etiology may range from large vessel stroke to micr ovascular disease; the | dsm5.pdf |
da59f1d82beb-0 | 622 Neurocognitive Disorders
presentation is therefore very heterogeneous, stemming from the types of vascular lesions
and their extent and location. The lesions may be focal, multifocal, or diffuse and occur in
various combinations.
Many individuals with major or mild vascul ar NCD present with multiple infarctions,
with an acute stepwise or fluctuating dec line in cognition, and intervening periods of
stability and even some improvement. Others may have gradual onset with slow pro-
gression, a rapid development of deficits follo wed by relative stabilit y, or another complex
presentation. Major or mild vascular NCD wi th a gradual onset an d slow progression is
generally due to small vessel disease leading to lesions in the white matter, basal ganglia,
and/or thalamus. The gradual progression in these cases is often punctuated by acute
events that leave subtle neurological deficits. The cognitive deficits in these cases can be at-
tributed to disruption of cortical-subcortical circuits, and complex attention, particularly
speed of information processing, and exec utive ability are likely to be affected.
Assessing for the presence of sufficient cerebrovascular disease relies on history, phys-
ical examination, and neuroimaging (Criterion C). Etiological certainty requires the dem-
onstration of abnormalities on neuroimaging. The lack of neuroimaging can result in
significant diagnostic inaccuracy by overlooking “silent” brain infarction and white mat-
ter lesions. However, if the neurocognitive im pairment is temporally associated with one
or more well-documented strokes, a probable diagnosis can be made in the absence of neu-
roimaging. Clinical evidence of cerebrovascular disease includes documented history of
stroke, with cognitive decline temporally assoc iated with the event, or physical signs con- | dsm5.pdf |
da59f1d82beb-1 | stroke, with cognitive decline temporally assoc iated with the event, or physical signs con-
sistent with stroke (e.g., hemi paresis; pseudobulbar syndrome , visual field defect). Neuro-
imaging (magnetic resonance imaging [MRI] or computed tomography [CT]) evidence of
cerebrovascular disease comprises one or more of the following: one or more large vessel
infarcts or hemorrhages, a strategically placed single infarct or hemorrhage (e.g., in angu-
lar gyrus, thalamus, basal forebrain), two or mo re lacunar infarcts outside the brain stem,
or extensive and confluent white matter lesions. The latter is often termed small vessel dis-
ease or subcortical ischemic changes on clinical neuroimaging evaluations.
For mild vascular NCD, history of a single stroke or extensive white matter disease is gen-
erally sufficient. For major vascular NCD, two or more strokes, a strate gically placed stroke,
or a combination of white matter disease and one or more lacunes is generally necessary.
The disorder must not be better explained by another disorder. For example, promi-
nent memory deficit early in the course mi ght suggest Alzheimer' s disease, early and
prominent parkinsonian features would suggest Parkinson's disease, and a close associa-
tion between onset and depression would suggest depression.
Associated Features Supporting Diagnosis
A neurological assessmen t often reveals history of stroke and/or transient ischemic epi-
sodes, and signs indicative of brain infarctions. Also commonly associated are personality
and mood changes, abulia, depression, and emotional lability. The development of late-
onset depressive symptoms accompanied by psychomotor slowing and executive dys- | dsm5.pdf |
da59f1d82beb-2 | onset depressive symptoms accompanied by psychomotor slowing and executive dys-
function is a common presentation among olde r adults with progressive small vessel isch-
emic disease (“vascular depression”).
Prevalence
Major or mild vascular NCD is the second most common cause of NCD after Alzheimer’s
disease. In the United States, population prevalence estimates for vascular dementia range
from 0.2% in the 65–70 years age group to 16% in individuals 80 years and older. Within
3 months following stroke, 20%–30% of individuals are diagnosed with dementia. In neu- | dsm5.pdf |
17f6aabd9c7b-0 | from 0.2% in the 65–70 years age group to 16% in individuals 80 years and older. Within
3 months following stroke, 20%–30% of individuals are diagnosed with dementia. In neu-
ropathology series, the prevalence of vascular dementia increases from 13% at age 70 years
to 44.6% at age 90 years or older, in comp arison with Alzheimer’s disease (23.6%–51%) and
combined vascular dementia and Alzheimer’s disease (2%–46.4%). Higher prevalence has | dsm5.pdf |
aaeb490ce2bd-0 | Major or Mild Vascular Neurocognitive Disorder 623
been reported in African Americans compared with Caucasians, and in East Asian countries
(e.g., Japan, China). Prevalence is higher in males than in females.
Development and Course
Major or mild vascular NCD can occur at an y age, although the prevalence increases ex-
ponentially after age 65 years. In older indivi duals, additional pathologies may partly ac-
count for the neurocognitive de ficits. The course may vary fr om acute onset with partial
improvement to stepwise decline to progressive decline, with fluctuations and plateaus of
varying durations. Pure subcortical major or mild vascular NCD can have a slowly pro-
gressive course that simulates major or mild NCD due to Alzheimer’s disease.
Risk and Prognostic Factors
Environmental. The neurocognitive outcomes of vascular brain injury are influenced by
neuroplasticity factors such as education, physical exercise, and mental activity.
Genetic and physiological. The major risk factors for major or mild vascular NCD are the
same as those for cerebrovascular disease, incl uding hypertension, diab etes, smoking, obesity,
high cholesterol levels, high homocysteine levels, other risk factors for atherosclerosis and ar-
teriolosclerosis, atrial fibrillati on, and other conditions increasing the risk of cerebral emboli.
Cerebral amyloid angiopathy is an important risk factor in which amyloid deposits occur
within arterial vessels. Another key risk factor is the hereditary condition cerebral autosomal
dominant arteriopathy with subcortical infarcts and leukoencephalopathy, or CADASIL.
Diagnostic Markers
Structural neuroimaging, using MRI or CT, ha s an important role in the diagnostic pro- | dsm5.pdf |
aaeb490ce2bd-1 | cess. There are no other established biomar kers of major or mild vascular NCD.
Functional Consequences of
Major or Mild Vascular Neurocognitive Disorder
Major or mild vascular NCD is commonly asso ciated with physical deficits that cause ad-
ditional disability.
Differential Diagnosis
Other neurocognitive disorders. Since incidental brain infarctions and white matter le-
sions are common in older individuals, it is important to consider other possible etiologies
when an NCD is present. A history of memory deficit early in the course, and progressive
worsening of memory, language, executive function, and perceptual-motor abilities in the
absence of corresponding focal lesions on br ain imaging, are suggestive of Alzheimer’s
disease as the primary diagnosis. Potential bi omarkers currently being validated for Alz-
heimer’s disease, such as cerebrospinal fluid levels of beta-amyloid and phosphorylated
tau, and amyloid imaging, may prove to be helpful in the differential diagnosis. NCD with
Lewy bodies is distinguished fr om major or mild vascular NCD by its core features of fluc-
tuating cognition, visual hallu cinations, and spontaneous parkinsonism. While deficits in
executive function and language occur in majo r or mild vascular NC D, the insidious onset
and gradual progression of behavioral features or language impairment are characteristic
of frontotemporal NCD and are no t typical of vascular etiology.
Other medical conditions. A diagnosis of major or mild vascular NCD is not made if
other diseases (e.g., brain tumor, multiple sc lerosis, encephalitis, toxic or metabolic disor-
ders) are present and are of su fficient severity to account for the cognitive impairment. | dsm5.pdf |
542b5cb9ccd1-0 | 624 Neurocognitive Disorders
Other mental disorders. A diagnosis of major or mild vascular NCD is inappropriate if
the symptoms can be entirely attributed to delirium, although delirium may sometimes be
superimposed on a preexisting major or mild vascular NCD, in which case both diagnoses
can be made. If the criteria for major depressive disorder are met and the cognitive impair-
ment is temporally related to the likely onse t of the depression, major or mild vascular
NCD should not be diagnosed. However, if the NCD preceded the development of the de-
pression, or the severity of the cognitive impairment is out of proportion to the severity of
the depression, both should be diagnosed.
Comorbidity
Major or mild NCD due to Alzheimer’s dise ase commonly co-occurs with major or mild
vascular NCD, in which case both diagnoses should be made. Major or mild vascular NCD
and depression frequently co-occur.
Major or Mild Neurocognitive Disorder
Due to Traumatic Brain Injury
Diagnostic Criteria
A. The criteria are met for major or mild neurocognitive disorder.
B. There is evidence of a traumatic brain injury—that is, an impact to the head or other
mechanisms of rapid movement or displacement of the brain within the skull, with one
or more of the following:
1. Loss of consciousness.
2. Posttraumatic amnesia.
3. Disorientation and confusion.
4. Neurological signs (e.g., neuroimaging demonstrating injury; a new onset of sei-
zures; a marked worsening of a preexisting seizure disorder; visual field cuts; an-
osmia; hemiparesis).
C. The neurocognitive disorder presents imm ediately after the occurrence of the trau-
matic brain injury or immediately after recovery of consciousness and persists past the | dsm5.pdf |
542b5cb9ccd1-1 | matic brain injury or immediately after recovery of consciousness and persists past the
acute post-injury period.
Coding note: For major neurocognitive disorder due to traumatic brain injury, with behavioral
disturbance: For ICD-9-CM, first code 907.0 late effect of intracranial injury without skull frac-
ture, followed by 294.11 major neurocognitive disorder due to traumatic brain injury, with be-
havioral disturbance. For ICD-10-CM, first code S06.2X9S diffuse traumatic brain injury with
loss of consciousness of unspecified duration, sequela; followed by F02.81 major neurocog-
nitive disorder due to traumatic brain injury, with behavioral disturbance.
For major neurocognitive disorder due to traumatic brain injury, without behavioral distur-
bance: For ICD-9-CM, first code 907.0 late effect of intracranial injury without skull fracture,
followed by 294.10 major neurocognitive disorder due to traumatic brain injury, without be-
havioral disturbance. For ICD-10-CM, first code S06.2X9S diffuse traumatic brain injury with
loss of consciousness of unspecified duration, sequela; followed by F02.80 major neurocog-
nitive disorder due to traumatic brain injury, without behavioral disturbance.
For mild neurocognitive disorder due to traumatic brain injury, code 331.83 (G31.84).
(Note: Do not use the additional code for traumatic brain injury. Behavioral disturbance
cannot be coded but should still be indicated in writing.) | dsm5.pdf |
26d4a670d6fc-0 | Major or Mild Neurocognitive Disorder Due to Traumatic Brain Injury 625
Specifiers
Rate the severity of the neuroc ognitive disorder (NCD), not the underlying traumatic brain
injury (see the section “Development and Course” for this disorder).
Diagnostic Features
Major or mild NCD due to traumatic brain injury (TBI) is caused by an impact to the head,
or other mechanisms of rapid movement or di splacement of the brain within the skull, as
can happen with blast injuries. Traumatic brain injury is defined as brain trauma with spe-
cific characteristics that include at least one of the following: loss of consciousness, post-
traumatic amnesia, diso rientation and confusion, or, in more severe cases, neurological
signs (e.g., positive neuroimaging, a new onset of seizures or a marked worsening of a pre-
existing seizure disorder, visual field cuts, anosmia, hemiparesi s) (Criterion B). To be at-
tributable to TBI, the NCD must present either immediately after the brain injury occurs or
immediately after the individu al recovers consciousness afte r the injury and persist past
the acute post-injury period (Criterion C).
The cognitive presentation is variable. Difficulties in the domains of complex attention,
executive ability, learning, an d memory are common as well as slowing in speed of infor-
mation processing and disturbances in social cognition. In more seve re TBI in which there
is brain contusion, intracranial hemorrhage, or penetrating injury, there may be additional
neurocognitive deficits, such as aphasia, neglect, and constructional dyspraxia.
Associated Features Supporting Diagnosis
Major or mild NCD due to TBI may be accompanied by disturbances in emotional function | dsm5.pdf |
26d4a670d6fc-1 | Major or mild NCD due to TBI may be accompanied by disturbances in emotional function
(e.g., irritability, easy frus tration, tension and anxiety, affective lability); personality
changes (e.g., disinhibition, apathy, suspic iousness, aggression); physical disturbances
(e.g., headache, fatigue, slee p disorders, vertigo or dizziness, tinnitus or hyperacusis, pho-
tosensitivity, anosmia, reduced tolerance to psychotropic medication s); and, particularly
in more severe TBI, neurolog ical symptoms and signs (e.g., seizures, hemiparesis, visual
disturbances, cranial nerve deficits) an d evidence of orthopedic injuries.
Prevalence
In the United States, 1.7 million TBIs occur a nnually, resulting in 1.4 million emergency de-
partment visits, 275,000 hospitalizations, and 52,000 deaths. About 2% of the population
lives with TBI-associated disabi lity. Males account for 59% of TBIs in the United States.
The most common etiologies of TBI in the Unit ed States are falls, vehicular accidents, and
being struck on the head. Collisions and blows to the head that occur in the course of con-
tact sports are increasingly re cognized as sources of mild TB I, with a concern that repeated
mild TBI may have cumulatively persisting sequelae.
Development and Course
The severity of a TBI is rated at the time of injury/initial assessment as mild, moderate, or
severe according to the thresholds in Table 2.
The severity rating of the TBI itself does not necessarily correspon d to the severity of
the resulting NCD. The course of recovery fr om TBI is variable, depending not only on the | dsm5.pdf |
26d4a670d6fc-2 | specifics of the injury but also on cofactors, such as age, prior history of brain damage, or
substance abuse, that may favor or impede recovery. | dsm5.pdf |
aa53889845a8-0 | 626 Neurocognitive Disorders
Neurobehavioral symptoms tend to be most severe in the immediate aftermath of the
TBI. Except in the case of severe TBI, the typi cal course is that of complete or substantial
improvement in associated neurocognitive, neurological, and psyc hiatric symptoms and
signs. Neurocognitive symptoms associated with mild TBI tend to resolve within days to
weeks after the injury with complete resoluti on typical by 3 months. Other symptoms that
may potentially co-occur with the neurological symptoms (e.g., depression, irritability,
fatigue, headache, photosensitivity, sleep dist urbance) also tend to resolve in the weeks
following mild TBI. Substantial subsequent deterioration in these areas should trigger con-
sideration of additional diagnoses. However, repeated mild TBI may be associated with
persisting neurocognitive disturbance.
With moderate and severe TBI, in addition to persistence of neurocognitive deficits,
there may be associated neurophysiological, emotional, and behavi oral complications.
These include seizures (particula rly in the first year), photosen sitivity, hyperacu sis, irritabil-
ity, aggression, depression, sl eep disturbance, fatigue, apat hy, inability to resume occu-
pational and social functioning at pre-injury level, and deterioration in interpersonal
relationships. Moderate and seve re TBI have been associated with increased risk of depres-
sion, aggression, and possibly neurodegenerative diseases such as Alzheimer’s disease.
The features of persisting majo r or mild NCD due to TBI will vary by age, specifics of
the injury, and cofactors. Persisting TBI-related impairment in an infant or child may be re-
flected in delays in reaching developmental mi lestones (e.g., language acquisition), worse | dsm5.pdf |
aa53889845a8-1 | flected in delays in reaching developmental mi lestones (e.g., language acquisition), worse
academic performance, and possibly impaired social development. Among older teenag-
ers and adults, persisting symptoms may incl ude various neurocognitive deficits, irrita-
bility, hypersensitivity to light and sound, e asy fatigability, and mood changes, including
depression, anxiety, hostility, or apathy. In older individuals with depleted cognitive re-
serve, mild TBI is more likely to result in incomplete recoveries.
Risk and Prognostic Factors
Risk factors for traumatic brain injury. Traumatic brain injury rates vary by age, with
the highest prevalence among individuals younge r than 4 years, older adolescents, and in-
dividuals older than 65 years. Falls are the mo st common cause of TBI, with motor vehicle
accidents being second. Sports concussions are frequent causes of TBI in older children,
teenagers, and young adults.
Risk factors for neurocognitive disorder after traumatic brain injury. Repeated con-
cussions can lead to persistent NCD and neur opathological evidence of traumatic enceph-
alopathy. Co-occurring intoxication with a substance may incr ease the severity of a TBI
from a motor vehicle accident, but whether into xication at the time of injury worsens neu-
rocognitive outcome is unknown.
Course modifiers. Mild TBI generally resolves within a few weeks to months, although res-
olution may be delayed or incomplete in the context of repeated TB I. Worse outcome fromTABLE 2 Severity ratings for tr aumatic brain injury
Injury characteristic Mild TBI Moderate TBI Severe TBI
Loss of consciousness <30 min 30 minutes–24 hours >24 hours
Posttraumatic amnesia <24 ho urs 24 hours–7 days >7 days | dsm5.pdf |
aa53889845a8-2 | Posttraumatic amnesia <24 ho urs 24 hours–7 days >7 days
Disorientation and confusion
at initial assessment
(Glasgow Coma Scale
Score)13–15 (not below 13
at 30 minutes)9–12 3–8 | dsm5.pdf |
ef21048a1e93-0 | Substance/Medication-Induced Major or Mild Neurocognitive Disorder 627
moderate to severe TBI is associated with older ag e (older than 40 years) and initial clinical pa-
rameters, such as low Glasgow Coma Scale score; worse motor function; pupillary nonreac-
tivity; and computed tomography (CT) evidence of brain injury (e.g., petechial hemorrhages,
subarachnoid hemorrhage, midline shift, obliteration of third ventricle).
Diagnostic Markers
Beyond neuropsycholog ical testing, CT scanning may reveal petechial hemorrhages,
subarachnoid hemorrhage, or evidence of contusion. Magn etic resonance image scanning
may also reveal hyperintensities suggestive of microhemorrhages.
Functional Consequences of Major or Mild
Neurocognitive Disorder Due to Traumatic Brain Injury
With mild NCD due to TBI, individuals may re port reduced cognitive efficiency, difficulty
concentrating, and lessened abilit y to perform usual activities. With major NCD due to TBI, an
individual may have di fficulty in independent living an d self-care. Prominent neuromotor
features, such as severe incoordination, ataxia , and motor slowing, may be present in major
NCD due to TBI and may add to functional diffi culties. Individuals with TBI histories report
more depressive symptoms, and these can amp lify cognitive complaints and worsen func-
tional outcome. Additionally, loss of emotional control, including aggre ssive or inappropriate
affect and apathy, may be present after more se vere TBI with greater neurocognitive impair-
ment. These features may compound difficulti es with independent living and self-care.
Differential Diagnosis | dsm5.pdf |
ef21048a1e93-1 | Differential Diagnosis
In some instances, severity of neurocognitive symptoms ma y appear to be inconsistent
with the severity of the TBI. After previously undetected neurologic al complications (e.g.,
chronic hematoma) are excluded , the possibility of diagnoses such as somatic symptom
disorder or factitious disorder need to be considered. Posttraumatic stress disorder (PTSD)
can co-occur with the NCD and have overlappi ng symptoms (e.g., difficulty concentrat-
ing, depressed mood, aggressiv e behavioral disinhibition).
Comorbidity
Among individuals with substa nce use disorders, the neurocognitive effects of the sub-
stance contribute to or compound the TBI-as sociated neurocognitive change. Some symp-
toms associated with TBI may overlap with sy mptoms found in cases of PTSD, and the two
disorders may co-occur, especia lly in military populations.
Substance/Medication-Induced
Major or Mild Neurocognitive Disorder
Diagnostic Criteria
A. The criteria are met for major or mild neurocognitive disorder.
B. The neurocognitive impairments do not occur exclusively during the course of a delir-
ium and persist beyond the usual duration of intoxication and acute withdrawal.
C. The involved substance or medication and duration and extent of use are capable of
producing the neurocognitive impairment.
D. The temporal course of the neurocognitive deficits is consistent with the timing of sub-
stance or medication use and abstinence (e.g., the deficits remain stable or improve
after a period of abstinence). | dsm5.pdf |
831acef0b754-0 | 628 Neurocognitive Disorders
E. The neurocognitive disorder is not attributable to another medical condition or is not
better explained by another mental disorder.
Coding note: The ICD-9-CM and ICD-10-CM codes for the [specific substance/medica-
tion]-induced neurocognitive disorders are indi cated in the table below. Note that the ICD-
10-CM code depends on whether or not there is a comorbid substance use disorder present
for the same class of substance. If a mild substance use disorder is comorbid with the sub-
stance-induced neurocognitive disorder, the 4th position character is “1,” and the clinician
should record “mild [substance] use disorder” before the substance-induced neurocognitive
disorder (e.g., “mild inhalant use disorder wi th inhalant-induced major neurocognitive disor-
der”). If a moderate or severe substance use disorder is comorbid with the substance-
induced neurocognitive disorder, the 4th position character is “2,” and the clinician should
record “moderate [substance] use disorder” or “severe [substance] use disorder,” depending
on the severity of the comorbid substance use disorder. If there is no comorbid substance
use disorder, then the 4th position character is “9,” and the clinician should record only the
substance-induced neurocognitive disorder. For some classes of substances (i.e., alcohol;
sedatives, hypnotics, anxiolytics), it is not permissible to code a comorbid mild substance
use disorder with a substance-induced neurocog nitive disorder; only a comorbid moderate
or severe substance use disorder, or no substance use disorder, can be diagnosed. Behav- | dsm5.pdf |
Subsets and Splits