neurosyphilis has a special place in the history of neuropsychiatric diseases and the unraveling of the etiology , pathogenesis and control remains one of the instructive lessons in the principles and practice of medicine .
its propensity to affect all social classes is intricately linked to it being a sexually transmitted disease .
the ravages left in its wake as it spread across europe and the subsequent fascinating story of the discovery of penicillin leading to a treatment for syphilis gave impetus for its eradication in the second half of the nineteenth century . despite some measure of success in the control and eradication ,
neurological presentations still occur and can pose a diagnostic dilemma to physicians . as this illness has become rare , opportunities for modern day neuroimaging are few .
we report a case presenting as right sided limb weakness , facial numbness and slurred speech five months prior , diagnosed as a computed tomography ( ct ) scan negative vascular event in which there was subsequent deterioration leading to admission to hospital with features diagnostic of normal pressure hydrocephalus . additionally , clinical findings consistent with dementia paralytica were present and this combination together with ct and magnetic resonance imaging ( mri ) of the modern era are uncommon in the medical literature .
furthermore , to the best of our knowledge , there have been only seven reported cases of neurosyphilis presenting as normal pressure hydrocephalus .
a 46-year - old male presented the emergency department with a history of a decline in his mental status and reduced level of cognition for four months .
he sought medical care five months prior to his presentation for weakness of the right side involving right upper and lower limbs associated with facial numbness and slurred speech .
the ct brain at that time showed diffuse atrophic and small vessel changes with mild hydrocephalus ( figure 1a ) .
he was suspected to have an atherosclerotic cerebrovascular accident and was treated empirically with aspirin , simvastatin and atenolol .
as his condition was worsening he was brought to the emergency room , 5 months later .
he had a progressive decline in his level of cognition , speech and gait was unable to express himself to the extent where his answers became yes or no responses .
there was also significant weight loss of 20 pounds over the preceding three months without any change in appetite .
his prior medication included aspirin , atenolol , simvastatin , which was started since the presumed cerebrovascular event , associated with mild dyslipidemia and hypertension .
however , he had a 20-pack year history of smoking but stopped one year prior , and a history of alcohol use for 25 years .
there was no history of herbal , marijuana , cocaine or other illicit drug use .
there were no recent ill contacts or travel , and he never received blood transfusions or tattoos .
his relatives reported that he only had one sexual partner with whom he had three children and no history of prior sexual transmitted conditions .
he was anicteric , acyanotic , afebrile and his mucus membranes were pink and moist .
he had a glasgow coma scale of 12/15 accounted by the following responses : eye=4 , motor=6 , verbal=2 .
the blood pressure was 120/77 , pulse 65 , respiratory rate 20 , percutaneous oxygen saturation 99% , random blood glucose 147
he had a reduced power of 4/5 on the right upper and lower limb , with associated past pointing and intention tremor .
the patient was able to understand instructions but had severe difficulty executing them as well as expressing himself .
his reflexes were + + + in s1s2 , l3l4 , c5c6 and t1t2 on both right and left sides .
there were involuntary jerky myoclonic movements of both upper and lower limbs , which were worse with movement .
initial laboratory investigations showed normal renal function , liver function , thyroid function , c - reactive protein test , coagulation profile , electrolytes , serum b12 and red cell folate , hiv , hemoglobin electrophoresis , as well as a normal connective tissue screen .
the echocardiogram was normal with an ef 70% with no aortic regurgitation or aortitis / aneurysm .
electroencephalography showed subcortical dysfunction in left frontal lobe , accompanied by cortical irritability in the same area spreading posteriorly to central and temporal regions .
ct brain on admission showed dilated ventricles with an evans index of 0.523 out of proportion to the degree of cerebral atrophy and hypodensities in bifrontal lobes , which appeared to be consistent with edema and microangipathic small vessel disease ( figure 1b ) .
mri imaging on t1,t2,t2 flair , apparent diffusion coefficient ( adc ) and diffusion - weighted imaging ( dwi ) on admission confirmed hydrocephalus , bifrontal white matter hyperintensities , mesotempoaral atrophy and absence of infarctions or gumma ( figure 2a - e ) .
lumbar puncture on presentation and 3 months later showed elevated protein and serological evidence confirming neurosyphilis , with improvement at 3 months following treatment ( table 1 ) .
he was treated with aqueous crystalline penicillin g 4 million units intravenously every four hours , for 14 days .
prior to treatment he had a barthel scale 20 and modified rankin scale of 4 . at the end of 2 weeks of treatment
with improved verbal communication from the patient with treatment , he reported having multiple prior sexual partners , and did not use barrier contraception on all occasions . furthermore , he recalled having an ulcer on his penis several years prior however did not seek medical care .
the clinical manifestations of neurosyphilis can occur at any stage of the infection and include asymptomatic neurosyphilis , acute meningeal syphilis , meningovascular syphilis , paretic neurosyphilis , and tabetic neurosyphilis .
clinical features of the general paresis type are divided into early and late onset features , some of which can be seen in normal pressure hydrocephalus ( nph ) which presents with ataxia , urinary incontinence and dementia .
early symptoms of the paretic type syphilis include irritability , forgetfulness , personality changes , headaches , and changes in sleep habits .
late symptoms include emotional lability , impaired memory and judgment , disorientation , confusion , delusions , and occasionally seizures .
psychiatric manifestations range from depression , to delirium ( hallucinations ) , mania , and psychosis .
common neurological signs include pupillary abnormalities ( argyll  robertson pupils occur in late paresis but are more often observed in tabes dorsalis , dysarthria , and tremors of facial , lingual , and hand muscles .
pathologically , there is atrophy of the frontal and temporal lobe with sparing of the motor , sensory , and occipital cortex .
the lissauer form of the disease , which occurs much less frequently , may also affect the cerebellum and basal ganglia .
the average time from infection to onset of paretic neurosyphilis is between 15 and 20 years .
most untreated patients die within 5 years after the onset of symptoms . to the best of our knowledge
since 1940 , limited case reports have encompassed the study of neurosyphilis in the post - penicillin era .
diagnosis requires clinical documentation of one or more of the characteristic symptoms of idiopathic nph in combination with a brain imaging study demonstrating non - obstructive ventricular enlargement disproportionate to cerebral atrophy .
our patient satisfied the clinical and radiological criteria for nph , which was further confirmed with normal cerebrospinal fluid ( csf ) opening pressures and positive serum and csf serology .
ct findings of patients with neurosyphilis are not well documented and a there are few reports on the mri findings of this disease . in one report
mri is the most sensitive technique in detecting changes in cerebral vasculitis involving large , medium , and small vessels .
most common mri diagnostic features in neurosyphilis are medium contrast enhancement , atrophy , white matter lesions , cerebral infarction , edema . in one study of patients with paretic neurosyphilis ,
the most common findings were generalized cerebral atrophy and foci of increased signal intensity on t2 weighted images .
there was a significant association between the presence of frontal lesions and overall degree of psychiatric morbidity .
those may be observed at any distribution , including subcortical white matter , deep white matter , or gray matter with areas of infarction and hemorrhage .
also , multiple areas of infarction in different vascular regions and of different ages are suggestive of cerebral vasculitis .
common findings include hypointensity on t1 , hyperintensity on t2 , medium contrast enhancement , perilesional edema , meningeal enhancement / dural tail , t1 isointense lesion , t2 isointense lesion , t2 hypointense lesion , cerebral infarction , arteritis , nonspecific white matter lesions involving the frontotemporal lobes , hippocampus and periventricular area , atrophy , gummas , extraaxial enhancement , high signal changes in the bilateral mesial temporal lobes including both hippocampus and amygdala in t2-images and flair and dwi sequence , ventricular dilatation . in neurosyphilis
the most frequently involved arteries are middle cerebral artery and branches of the basilar artery .
our patient had hypointensity on t1 in the left frontal region with hyperinternsity on the left frontal t2 mri with restricted diffusion on dwi image , which support a vasculitic expression of the disease .
the goal of therapy is to inhibit the progression of the infection and reverse the symptoms .
antibodies produced in response to the syphilis antigen results in neuroinflammation , which can contribute to initiation and maintenance of chronic inflammation and tissue damage in neurosyphilis independent of active infection .
data on the treatment of psychiatric symptoms associated with paretic neurosyphilis are very limited . for late disease , especially the parenchymatous forms ,
however with treatment there can be considerable regression of the neurological and psychological signs as in our patient .
repeat lumbar puncture at 3 months showed a fall in protein and vdrl titre correlating with clinical and radiological improvement .
a high index of clinical suspicion is required to diagnose neurosyphilis and commence treatment early . in a illustrative study of 116 patients with general paresis in china , after the initial evaluation
, neurosyphilis was not suspected in 36 percent of patients , which delayed the diagnosis for 1 to 24 months . despite the drastic reduction in the incidence of neurosyphilis with the advent of effective diagnosis and treatment
however , the primary and secondary ( p&s ) syphilis rates have increased almost every year since 20002001 . in 2014 ,
a total of 19,999 p&s syphilis cases were reported , and the national p&s syphilis rate increased to 6.3 cases per 100,000 population , the highest rate since 1994 . during 2013 - 2014 , according to the centers for disease control and prevention , the number of cases of late and late latent syphilis increased 7.9% ( from 21,819 to 23,541 cases ) .
therefore , syphilis remains a global health issue and clinicians are reminded to be vigilant since treatment of this reversible cause of dementia can be most rewarding .
 we report a case of a 46-year - old man presenting with a progressive cognitive decline , ataxic gait , urinary incontinence for 4 months and neuroimaging consistent with normal pressure hydrocephalus . 
 the atypical presentation of a progressively worsening dysphasia and a right hemiparesis dismissed as a vascular event 1 month earlier associated with normal pressure hydrocephalus prompted further investigations confirming neurosyphilis also manifesting as dementia paralytica . 
 treatment using consensus guidelines led to resumption of activities of daily living . 
 neurosyphilis , considered rare in the neuroimaging era , must still be considered a reversible cause of dementia and other neurological manifestations in contemporary neurological practice . 
Introduction
Case Report
Discussion
Conclusions