The answer is 2, an EMG, which would be the diagnostic test to confirm the diagnostic suspicion of ALS, the imaging tests both CT, but especially MRI would help us to rule out, but would not confirm the diagnosis.

The answer is [HIDDEN], an EMG, which would be the diagnostic test to confirm the diagnostic suspicion of ALS, the imaging tests both CT, but especially MRI would help us to rule out, but would not confirm the diagnosis.

A 65-year-old woman consults for weakness in the right hand that has spread in a few months to other muscle territories of both arms and legs, with distal predominance. On examination there is atrophy and fasciculations in different metameric territories with preserved sensitivity. There is a bilateral Babinski's sign, what is the diagnostic test that would confirm the suspected diagnosis?

[A case presenting manifestations of bulbospinal muscular atrophy with senile onset, rapid progression and marked asymmetry].

A 64-year-old man was admitted to our department because of muscle cramp, atrophy and weakness of the limbs together with difficulty in walking, which had gradually progressed from age 60. About 1 year prior to admission, he had noticed hand tremor and gynecomastia. On admission, neurological examination revealed diffuse muscle atrophy and weakness of the extremities, which were more obvious on the right side with preponderance in the right leg. Bilateral postural hand tremor was also more prominent on the right hand. Fasciculations were observed both in the extremities and tongue. The remaining cranial nerves and cerebellar functions were intact. Sensation was normal except for slightly decreased vibratory sense in the distal part of the legs. Deep tendon reflexes including jaw jerk were increased with the exception of hyporeflexia of the right leg. Babinski sign was negative bilaterally. Blood examination disclosed slight elevation of CK and fasting glucose level of 110 mg/dl. Glucose tolerance test showed a diabetic pattern. CSF examination showed total protein of 74 mg/dl and IgG of 12 mg/dl. On a series of endocrinological studies, there was no significant elevation of androgen and estrogen both in serum and urine except for slight elevation of serum E1 level. Serum LH and FSH, however, were markedly high, which responded far beyond the normal range following to 0.1 mg injection of LH-RH. These results suggested that gynecomastia might be caused by dysfunction of the hypothalamus-hypophysis system. Brain CT and spine MRI showed no abnormality. Muscle biopsy obtained from the right quadriceps femoris revealed neurogenic abnormalities.(ABSTRACT TRUNCATED AT 250 WORDS)

Progressive muscular atrophy

Signs and symptoms As a result of lower motor neuron degeneration, the symptoms of PMA include: muscle weakness muscle atrophy fasciculations Some patients have symptoms restricted only to the arms or legs (or in some cases just one of either). These cases are referred to as flail limb (either flail arm or flail leg) and are associated with a better prognosis. Diagnosis PMA is a diagnosis of exclusion, there is no specific test which can conclusively establish whether a patient has the condition. Instead, a number of other possibilities have to be ruled out, such as multifocal motor neuropathy or spinal muscular atrophy. Tests used in the diagnostic process include MRI, clinical examination, and EMG. EMG tests in patients who do have PMA usually show denervation (neuron death) in most affected body parts, and in some unaffected parts too. It typically takes longer to be diagnosed with PMA than ALS, an average of 20 months for PMA vs 15 months in ALS.

[The diagnosis of amyotrophic lateral sclerosis supported by motor evoked potential and brain MRI studies].

A 57-year-old man developed severe muscle weakness and atrophy of the upper extremities within a five-month period. Neurological examination revealed severe weakness and atrophy in the scapular muscles and proximal and distal muscles of the upper extremities. Fasciculations were also observed in the various muscles of the upper extremities. There was neither muscle weakness, atrophy nor fasciculation in either his face, neck muscles or lower extremities. He had no pseudobulbar or bulbar signs. Tendon reflexes were mildly hyperactive in the jaw and lower extremities, and normal in the upper extremities. There were no pathological reflexes, spasticity or sensory disturbances. The needle EMG study revealed denervation potentials in all muscles of the upper extremities examined. The nerve conduction study revealed no findings of the conduction block. Cervical spine X-rays revealed the narrowing of the spinal foramens at the left C3/C4 and bilateral C4/C5, C5/C6, and C6/C7 intervertebral levels. In addition, magnetic resonance imaging (MRI) revealed compressions of the cervical cord at C4/C5 and C5/C6 intervertebral levels. These clinical and neuroradiological findings resembled those of the cervical spondylotic amyotrophy (CSA). However, the motor evoked potential (MEP) study revealed the pyramidal tract dysfunction above the levels of the pyramidal decussation. Furthermore, brain MRI revealed abnormal foci in both internal capsules which were characterized by hyperintense relative to cortical gray matter on T2-weighted images and still hyperintense to white matter on proton-density-weighted images. In addition, T2-weighted images demonstrated a low signal within the motor cortex and hyperintense lesions in the white matter of the precentral gyri. These MRI findings indicated the degeneration of the pyramidal tract and corresponded to those found in the patients with amyotrophic lateral sclerosis (ALS) which have been recently reported. It has been difficult to distinguish ALS from CSA. However, MEP and brain MRI studies were useful for distinguishing these two diseases in this patient. In addition, this patient showed typical MRI findings suggesting the degeneration of the pyramidal tract, although this patient had a relatively short course of illness and did not show obvious physical findings suggesting pyramidal tract dysfunction.

[Neurological CPC.57. An 80-year-old woman with four years history of muscle atrophy involving lower extremities predominantly on the right side].

We report an 80-year-old woman with progressive muscular atrophy predominantly involving her right lower extremity. She was well until 1992 (75 years of age) when she noted an onset of weakness in her right leg which had got progressively worse. She was admitted to our service in July 1994. On admission, general physical examination was unremarkable. She was alert and well oriented without dementia. Higher cerebral functions were normal. Cranial nerves also appeared intact. She dragged her right leg in walking. Mild to moderate weakness (2/5 to 4/5) was noted in muscles in her right lower extremity more in the distal part. Deep tendon reflexes were within normal limits, and the plantar response was flexor bilaterally. Sensation was intact. Laboratory examinations were also unremarkable except for slight increase in CK which was 470 IU/l. CSF was also normal. EMG revealed neurogenic changes in the lower extremities. She was admitted to Aoki Hospital on October 21, 1994, by that time, her weakness in the right lower extremity had gotten worse in that the muscle strength of the right extensor hallucis longus was 0 and tibialis anterior 2; muscle atrophy was also prominent in her right leg; the right ankle jerk could not be elicited. In the subsequent course, weakness and atrophy appeared in her left lower extremity, however, upper extremities and cranial nerves had never been affected. Babinski sign was always negative. In February 1996, she developed delusional ideation of self persecution, and showed difficulty in communication with medical staffs. She developed fever of 38.7 degrees C on June 13, 1996 expired on the next day. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that the patient had a form of spinal muscular atrophy. Opinions were divided between ALS and spinal muscular atrophy. Post-mortem examination revealed marked loss of anterior horn neurons in the lumbar area with astrogliosis. Bunina bodies were seen in some of the remaining neurons. No myelin pallor was noted in the pyramidal tracts, however, atrophy and loss of Betz cells were noted in the motor cortex. Other cortical areas were unremarkable. The neuropathologist arrived at the conclusion that the patient had ALS. This patient was unique in that she had asymmetric atrophy and weakness limited to the lower extremities. This is quite unusual as ALS of four years duration. In addition, the patient developed some mental change which was thought to represent dementia by some participants. But no clear morphologic changes were seen to account for her mental change.

[Spinal meningioma as differential diagnosis of diabetic polyneuropathy].

A 70 year old woman had suffered from diabetes mellitus type 2 since she was 52. Three years before the surgery she had begun to experience weakness together with altered sensitivity in the right leg, which was regarded as having been caused by diabetic polyneuropathy. During the admission examination the level for algesia on the right-hand side was at about D 11, a distal paraparesis of the leg (3-4 degrees, Janda's classification), more intense on the right, hyperactive deep tendon reflexes, Babinski's reflex on both sides, and depressed abdominal cutaneous reflexes. The sensitivity to vibrations on the Malleolus medialis on both sides was 0/8. The patient could walk only with the help of a Rollator. Over the three-year period following onset of symptoms the following tests were carried out: motor nerve conduction speeds of the N. tibialis and N. peronaeus, electromyogram of the N. tibialis anterior and the M. gastrocnemius, somatosensory evoked potentials (SSEP) of the N. tibialis, which indicated a lesion in the peripheral nerves or nerve roots. Cranial computed tomography (CCT), CT scan of the lumbar spine (L3-S1) and angiological investigation elicited no significant pathological findings. An MRI of the thoracal spine showed a vertebra-sized dorsal tumor pressing on the spinal cord from left to right. By means of microsurgery the spinal tumor was completely removed. Suspected meningeoma was confirmed by histological analysis. During the post-surgical period, the incomplete paraplegia quickly regressed, and 7 weeks after the removal of the spinal meningeoma the patient was able to climb stairs. In case of slowly-developing paresis of the legs in diabetic patients, diabetic polyneuropathy should not be diagnosed without careful consideration, and rare spinal tumors should be considered as part of the differential diagnosis, especially if the blood glucose level is normal, and intensive physiotherapy brings no improvement in the patient's condition.

[Familial spastic paraplegia with severe amyotrophy of the hands. (Silver syndrome?)].

Familial spastic paraplegia (FSP) with severe muscular atrophy of hands and feet is exceptional. Autosomal dominant forms were initially described by Silver in 1966. We report two cases, from the same Tunisian family, presenting FSP with severe amyotrophy of the hands. A brother and his sister, aged respectively 37 and 36 years old, presented practically the same clinical picture. Their parents were cousins. The female patient was hospitalized. Both patients developed gait disorders around the age of three years. Muscular atrophy of the hands arose much later, around the age of 20 years. The neurological examination disclosed a spastic gait with distal amyotrophy, severe in the hands and moderate in the feet. Sensitivity was preserved and there was no fasciculation. The spinal cord and cerebral MRI was normal. Electromyography (EMG) showed a neurogenic pattern in the distal muscles. Stimulation of the median, ulnar and sciatica nerves was ineffective. The somatosensory evoked potentials (EP) were delayed (upper limb) or desynchronised (lower limb). The auditory and visual EP were normal. The cerebrospinal fluid contained 1 mononuclear cell/mm3 and 10 mg protein/100 ml. Abnormalities of the cranio-vertebral junction, Arnold-Chiari malformation, syringomyelia and familial juvenile amyotrophic lateral sclerosis (ALS) were excluded and the diagnosis of Silver's syndrome was evoked.

[A patient with motor neuron syndrome clinically similar to amyotrophic lateral sclerosis, presenting spontaneous recovery].

We report a patient with motor neuron syndrome similar to amyotrophic lateral sclerosis (ALS) and with spontaneous recovery. At the age 40, the woman developed progressive muscular weakness, atrophy and fasciculation in extremities. She also noted a dyspnea, tongue atrophy and dysphagia. A neurological examination 6 months after onset revealed i) a tongue atrophy and fasciculation, ii) diffuse muscule weakness and atrophy in face, neck and extremities, and iii) marked hyperreflexia in the four limbs and bilateral Babinski reflex, but iv) neither sensory disturbance nor ophthalmoplegia. Electromyogram (EMG) detected such denervation potentials as fibrillation potentials, fasciculation potentials, positive sharp waves and polyphasic or giant MUPs diffusely in the limb muscles. Peripheral nerve conduction study detected neither conduction block nor delay. Thus, she was diagnosed as suffering from ALS. However, since approximate 1 year after onset, her muscle weakness has gradually been getting better. Simultaneously, the dyspnea and dysphagia gradually improved. Two years after onset, an EMG examination detected chronic denervation potentials in the left musculus sternocleidomastoideus and a few on-going denervation potentials in the left musculus extensor carpi radialis, but no denervation potentials in other limb muscles. Fasciculation potentials were found in tongue muscles. Thus, the present case was thought to have a reversible motor neuron syndrome clinically quite similar to ALS. A mild increase in IgE (346 U/ml) and a low-titer IgM-class anti-GM1 antibody were found in her serum though its pathological significance was uncertain. Any immunological aberrance may account for the pathogenesis. It should be noted that clinically diagnosed cases of ALS may rarely recover spontaneously.

An unusual cause of dementia: essential diagnostic elements of corticobasal degeneration-a case report and review of the literature.

Corticobasal degeneration (CBD) is an uncommon, sporadic, neurodegenerative disorder of mid- to late-adult life. We describe a further example of the pathologic heterogeneity of this condition. A 71-year-old woman initially presented dysarthria, clumsiness, progressive asymmetric bradykinesia, and rigidity in left arm. Rigidity gradually involved ipsilateral leg; postural instability with falls, blepharospasm, and dysphagia subsequently developed. She has been previously diagnosed as unresponsive Parkinson's Disease. At our clinical examination, she presented left upper-arm-fixed-dystonia, spasticity in left lower limb and pyramidal signs (Babinski and Hoffmann). Brain MRI showed asymmetric cortical atrophy in the right frontotemporal cortex. Neuropsychological examination showed an impairment in visuospatial functioning, frontal-executive dysfunction, and hemineglect. This case demonstrates that association of asymmetrical focal cortical and subcortical features remains the clinical hallmark of this condition. There are no absolute markers for the clinical diagnosis that is complicated by the variability of presentation involving also cognitive symptoms that are reviewed in the paper. Despite the difficulty of diagnosing CBD, somatosensory evoked potentials, motor evoked potentials, long latency reflexes, and correlations between results on electroencephalography (EEG) and electromyography (EMG) provide further support for a CBD diagnosis. These techniques are also used to identify neurophysiological correlates of the neurological signs of the disease.

La Belle Indifference -- History and Physical

Hoover's sign (63% Sensitivity & 100% specificity): This test is commonly used to separate organic from the nonorganic cause of weakness or paralysis. An examiner's hand is placed below the heel of the affected leg, and the patient is asked to flex the hip of the normal leg against resistance. In organic disorders, there should not be any pressure on the examiner's hand on the affected side, while pressure is felt in patients with FNSD/CD. [20] Variable Strength (63% sensitivity and 97% specificity): The weakness is inconsistent with variable force at different locations.

Neurology_Adams

In patients with neurologic signs, nerve conduction studies disclose reduced amplitude of the ulnar sensory potentials. There may be decreased amplitude of the median motor evoked potentials as well, a mild but uniform slowing of the median motor conduction velocity, and a prolongation of the F-wave latency. Concentric needle examination of affected hand muscles reveals large-amplitude motor units, suggesting collateral reinnervation. Somatosensory evoked potentials may be a useful adjunct to the conventional nerve conduction and EMG studies (Yiannikas and Walsh). Brachial artery MR angiography is usually reserved for patients with a suspected arterial occlusion, an aneurysm, or an obvious cervical rib. The place of venography in the diagnostic workup is uncertain, for a number of otherwise normal individuals can occlude the subclavian vein by fully abducting the arm.

Neurology_Adams

The biceps and brachioradial reflexes on one or both sides may be depressed, sometimes in association with an increase in the triceps and finger reflexes. The hand or forearm muscles may undergo atrophy; in a few cases, the atrophy of hand muscles is severe. In such cases, the spondylotic compression, as judged by MRI or CT myelography, may be confined to the high cervical cord, well above the levels of the motor neurons that innervate these muscles. In patients with sensory loss, pain and thermal sensation often appear to be affected more than tactile sense. An unexpected Babinski sign has already been mentioned and a few fasciculations may be seen, especially in proximal arm muscles. Another unusual feature in advanced stages of cervical cord compression is the appearance of mirror movements of the hands, in which effortful attempts to make refined movements of the fingers of one hand, causes the opposite hand to move similarly.

Neurology_Adams

Monoplegia with Muscular Atrophy This is more frequent than monoplegia without muscular atrophy. Long-continued disuse of one limb may lead to atrophy, but it is usually of lesser degree than atrophy caused by lower motor neuron disease (denervation atrophy). In disuse atrophy, the tendon reflexes are retained and nerve conduction studies are normal. With denervation of muscles, there may be visible fasciculations and reduced or abolished tendon reflexes in addition to paralysis. The location of the lesion (in nerves, spinal roots, or spinal cord) can usually be determined by the pattern of weakness, by the associated neurologic symptoms and signs, and by special tests—MRI of the spine, examination of the cerebrospinal fluid (CSF), and electrical studies of nerve and muscle. If the limb is partially denervated, the EMG shows reduced numbers of motor unit potentials (often of large size) as well as fasciculations and fibrillations.

Tests of motor function in patients suspected of having mild unilateral cerebral lesions.

Though various textbooks describe clinical manoeuvres that help detect subtle motor deficits, their sensitivity, specificity and predictive values have not been determined. We investigated the sensitivity, specificity and predictive values of various manoeuvres in order to determine the most sensitive and reliable test or combination thereof. Straight arm raising (Barré), pronator drift, Mingazzini's manoeuvre, finger tap, forearm roll, segmental strength and deep tendon reflexes were tested in 170 patients with (86) and without (84) a proven lesion in the motor areas confirmed by computed tomography. Segmental motor strength bad good specificity (97.5%) but poor sensitivity (38.9%) and negative predictive value (NPV) (58.7%). The forearm roll had a similar profile. Finger tap had a sensitivity of 73.3% and a specificity of 87.5%. Barré and pronator testing had a sensitivity and specificity of 92.2% and 90.0% respectively. Hyperreflexia had a sensitivity of 68.9% and a specificity of 87.5%. An abnormality of pronator, reflexes or finger tap had a sensitivity of 97%, and when these three tests were positive, specificity was 97%. When all six tests were positive, the positive predictive value was 100%, when all six tests were negative the NPV was 100%. The detailed segmental examination has very good specificity for detecting motor deficits, but the sensitivity and NPV are unacceptably low. Pronator drift with finger tap and reflexes is the most reliable and time-effective combination of tests for the detection of subtle motor lesions, and could replace the segmental motor examination as a screening for motor lesions.

How to Localize Neurologic Lesions by Physical Examination -- Clinical Significance

Examination of the motor system of a limb includes checking for muscle bulk and fasciculation, muscle tone at joints, the power of muscle groups, deep tendon reflexes, clonus, plantar response, and coordination. In cases of a lower motor neuron type weakness, there is early muscle wasting, fasciculations, hypotonia, hyporeflexia, and a normal plantar response. On the other hand, the upper motor neuron type of weakness is characterized by normal muscle bulk, hypertonia, hyperreflexia, clonus, and an extensor plantar response (positive Babinski’s sign). Furthermore, the preservation of deep tendon reflexes distinguishes myopathy from neuropathy.

Hirayama's disease: an Italian single center experience and review of the literature.

Hirayama's disease (HD), is a benign, self-limited, motor neuron disease, characterized by asymmetric weakness and atrophy of one or both distal upper extremities. In the present study we report the clinical, electrophysiological and MRI features of a group of Italian patients, with review of the literature. Moreover we propose an optimized MRI protocol for patients with suspected or diagnosed HD in order to make an early diagnosis and a standardized follow up. Eight patients with clinical suspicion of Hirayama disease underwent evaluation between January 2007 and November 2013. All patients underwent standard nerve conduction studies (NCS), electromyography (EMG) and motor/sensory evoked potentials (MEP/SEP). Cervical spine MRI studies were conducted with a 1.5 Tesla MRI scanner in neutral and flexion position, including sagittal T1-weighted sequences and sagittal and axial T2-weighted sequences. The following diagnostic features were evaluated: abnormal cervical curvature, localized cervical cord atrophy in the lower tract (C4-C7), presence of cord flattening (CF), intramedullary signal hyperintensity on T2 weighted sequences, anterior shifting of the posterior wall of the cervical dural sac (ASD) and presence of flow voids (EFV) in the posterior epidural space during flexion. All patients complained of weakness in hand muscles as initial symptoms, associated with hand tremor in three of them and abnormal sweating of the hand palm in two of them. No sensory deficits and weakness at lower limbs were reported by any patients. Distal deep tendon reflexes at upper limbs were absent in all patients with the absence of the right tricipital reflex in one of them. Deep tendon reflexes at lower limbs were normal and no signs of pyramidal tract involvement were present. The clinical involvement at onset was unilateral in six patients (three left-sided and three right-sided) and bilateral asymmetric in two of them, with the right side more affected. With the progression of the disease all patients but one experienced weakness and wasting of hand muscles and forearm bilaterally, but still asymmetric. The duration of the progression phase of the disease ranged from eight months to three years. In all patients, NCS and EMG findings were consistent with a spinal metameric disorder involving the C7-T1 myotomes bilaterally; sensory conduction and electrophysiologic features at lower limbs were normal. MEP and SEP were normal and we did not observe the disappearance of the spinal potential during the neck flexion in any of the patients. MRI is the best diagnostic tool in the diagnosis of HD; it can confirm clinical diagnosis and exclude other conditions responsible for the neurological deficits leading to a correct patient management and therapy, limiting arm impairment. On MRI all patients had loss of the normal cervical lordosis (100%). Five patients had loss of attachment of posterior dural sac and anterior dural shift on flexion MRI with presence of flow voids from venous plexus congestion (62.5%); three patients had no anterior dislocation of the dural sac and no epidural vein congestion. Two patients showed localized cord atrophy, one at C5-C6 and the other at C6-C7 level (25%). Three patients had T2 intramedullary hyperintensities (37.5%) and cord flattening (CF) was present in 5 patients of 8 (62.5%). HD is a rare entity and a self-limited condition, but it has to be early differentiated from other diseases that may determine myelopathy and amyotrophy to establish a correct therapy and limit arm impairment. MRI is very important to confirm the clinical suspect of HD and a standardized MRI protocol using axial and sagittal images in both neutral and flexing position is needed, in order to diagnose and follow up affected patients.

[Regarding the clinical diagnosis of the  monotopical  spinal forms of multiple sclerosis. The value of the fan sign in the adult].

We wish to discuss the value of the clinical history and examination in orientation of the diagnosis of probable multiple sclerosis (MS). We report the two year study of a woman who over the previous ten years had had three episodes of paraesthesia, with pins and needles in her left leg and other parts of the left side of her body, although never affecting head or neck. She also complained of tiring more than usual. In an outpatient clinic she was found to have a syndrome affecting the upper segments of the spinal cord, mainly involving the right side and resembling an incomplete Brown Sequard type syndrome. There were increased clinical muscle and deep reflexes. The most marked was that of the right deltoid (C5), bilateral fanning of the toes when the Babinski reflex was tested, Barré positive in the right leg, pins and needles and dysaesthesia on the left to an undetermined level. Function was well preserved when compared with the clinical signs found. The case was considered to be of monotopical MS. Spinal magnetic resonance findings confirmed the clinical diagnosis. We emphasise the value of careful clinical investigation directed towards the diagnosis of probable MS. We draw attention to the diagnostic value of the dissociation between the severe clinical alterations and the functional performance, which was surprisingly well maintained. Also we report the originality of the presence of bilateral fanning sign supporting the diagnosis of MS, occurring in a disease of adult life.

[A 54-year-old man with progressive proximal muscle atrophy and gynecomastia].

We report a 54-year-old man with progressive proximal muscle atrophy and gynecomastia. The patient had an insidious onset of weakness in his lower extremities at age 14, in that he noted a difficulty in standing up from a chair. Soon after he noted some difficulty in climbing up stairs. At age 35, he noted weakness in his arms; his weakness slowly progressed in that he became unable to walk or stand alone before 40 years of age. He also noted gynecomastia at that age. He was admitted to our hospital for the work up on September 16, 1993, when he was 54-year-old. On admission, he was alert and oriented; his BP was 150/70 mmHg; he had bilateral gynecomastia, however, no other skeletal deformities were found. On neurologic examination, he was mentally sound without dementia, and his higher cerebral functions were normal. Cranial nerves also appeared intact without facial atrophy, dysarthria, or dysphagia; no atrophy was noted in the tongue. He had marked muscle atrophy in both upper and lower extremities more marked in the proximal portions; muscle strength was approximately in the range of 2/5 to 3/5 in the proximal parts, and 4/5 in the distal parts in both upper and lower extremities. No fasciculation was noted; muscle tone was flaccid; no ataxia was present. Deep reflexes were either lost or markedly diminished. No Babinski sign was noted. Sensation was intact. Laboratory examination revealed normal blood counts; serum CK was slightly increased to 131 IU/l; ECG showed complete right bundle branch block; EMG revealed no active units in the right biceps brachii, deltoid, quadriceps femoris, and triceps surae muscles; in other muscles tested, motor unit potentials of low amplitude and short duration were seen; in the right tibialis anterior muscle, however, motor unit potentials with an amplitude up to 6 m V were also seen. Nerve conduction velocities were normal. A diagnostic procedure was performed. He was discussed in the neurological CPC, and the chief discussant arrived at the conclusion that this patient had Becker type of progressive muscular dystrophy. In her differential diagnosis, the possibility of Kennedy-Alter-Sung syndrome was discussed because this patient had gynecomastia. However, the discussant excluded that possibility because of absence of both bulbar symptoms and typical neurogenic changes in his EMG. The diagnostic procedure was a muscle biopsy on the left tibialis anterior muscle. Histologic observation on HE stained specimens revealed marked inequality in the muscle fiber diameters, increase in endomysial nuclei, proliferation of connective tissue, and fiber splitting.(ABSTRACT TRUNCATED AT 400 WORDS)

Distal Acquired Demyelinating Symmetric Neuropathy Associated with Decreased Electrical Excitability of the Femoral Nerves.

<bIntroduction:</b There are many phenotypic variants of chronic inflammatory demyelinating polyneuropathy. <bMethods:</bAn Ancient Greek aryvallos painted c. 480-450 BC, now on display at the Louvre museum, was meticulously studied regarding its painted surface, which presents an outpatient clinic in Ancient Greece. Other Ancient Greek works of art presenting medical activities have been also evaluated in order to reach informed conclusions regarding medical practice of that period. <bCase report:</b We report a rare case of the distal phenotype of chronic inflammatory demyelinating polyneuropathy with a subacute onset and rapidly progressive course. A 58-year-old male had distal, symmetric, predominantly motor impairment without ataxia and tremor. After a three-month duration of the disease, the patient had already complete paresis of the feet with absence of compound muscle action potentials (CMAPs) over the feet and lower leg muscles, but preserved proprioception and sural sensory nerve action potential. Cerebrospinal fluid protein level was elevated to 3.4 g/L. Demyelinating neuropathy was predominantly in the proximal segment of the nerves. Low amplitude of CMAPs was recorded hardly over the vastus medialis and rectus femoris muscles, while weakness and atrophy in these muscles were not. The patient was refractory to treatment. He died three years after disease onset. <bConclusion:</bWe described a new clinical-electrophysiological phenomenon, which was characterized as a decrease in the evoked electrical excitability at the femoral nerve stimulation site (decreased CMAP), while the natural physiological conduction of the impulse from the motor neuron to the muscle was not blocked (preserved muscle strength).

Characteristics of C6-7 myelopathy: assessment of clinical symptoms and electrophysiological findings.

This is a single-center retrospective study. The objective of this study was to study the clinical symptoms and electrophysiological features of C6-7 myelopathy. This study was conducted at the Department of Orthopedic surgery, Yamaguchi University Graduate school of medicine, Japan. A total of 20 patients with cervical compressive myelopathy were determined by spinal cord-evoked potentials or a single level of obvious magnetic resonance imaging (MRI)-documented cervical spinal cord compression. Neurological examinations included manual muscle testing and investigation of deep tendon reflex, including Hoffmann sign, and of sensory disturbance areas. Motor-evoked potentials (MEPs), compound muscle action potentials (CMAPs) and F-wave were recorded from bilateral abductor digit minim and abductor halluces muscles. Central motor conduction time was calculated as follows: MEPs latency-(CMAPs latency+F latency-1)/2 (ms). Eighteen patients (90%) had negative Hoffmann sign. Eight patients (40%) had no sensory disturbance in the upper limbs and 8 patients (40%) had no muscle weakness in the upper limbs. We determined that patients had cervical myelopathy when their central motor conduction time measured in abductor digit minim was longer than 6.76 ms (+2 s.d.). Using this definition, the sensitivity for myelopathy was 42.8%. Patients with C6-7 myelopathy may lack clinical symptoms in their hands and central motor conduction time measured in abductor digit minim tended to be less prolonged, and it only showed symptoms in their lower limbs as gait disturbance. Surgeons should bear in mind the possibility of disorders of caudal C6-7 when they encounter patients with no or few symptoms in their hands and with leg weakness or numbness.

[A 65-year-old woman with dysarthria, dysphagia, weakness, and gait disturbance].

We report a 65-year-old woman with progressive dysarthria, dysphagia, weakness, and gait disturbance. The patient was well until 59 years of age (January of 1986) when she noted bilateral ptosis. One year later, she noted a gradual onset of difficulty in speech (articulation). Her speech slowly deteriorated and she noted weakness in chewing power and difficulty in swallowing in addition. In October 1987, she developed emotional incontinence. In January of 1988, she started to drag her left foot. She was admitted to our hospital on June 13 of 1988. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed no dementia; her higher cerebral functions appeared intact. Ptosis was present bilaterally more on the right. She showed difficulty in opening her eyes on command; no contraction of the frontal muscles was seen upon attempted eye opening. There was a moderate limitation in the vertical gaze. Forced laughing and crying were seen. Facial muscles were moderately weak without apparent atrophy. The movement of the soft palate was very weak, and swallowing disturbance was more prominent for liquid staff. The tongue appeared somewhat small, however, no fasciculation was noted. Her step was small and the posture was stooped. Retropulsion was present, however, Romberg's sign was absent. No muscle atrophy was apparent, however, diffuse mile to moderate muscle weakness was noted in all four limbs. Cerebellar sign was absent. Deep tendon reflexes were exaggerated bilaterally, and Babinski sign was present on the left side. Sensation was intact. Routine blood tests were unremarkable as was a cranial CT scan. Her ptosis did not improve after 10 mg of edrophonium injection. CSF was also normal. She was transferred to another hospital but her neurological disabilities further progressed. In 1989, she was totally unable to move her limbs; she could only move her eyes; still consciousness was clear without dementia. She developed respiratory difficulty and expired on July 25, 1992. She was discussed in a neurological CPC, and the opinions were divided into ALS and primary lateral sclerosis (PLS). The chief discussant arrived at the conclusion that the patient might have had the pyramidal form of ALS. Postmorten examination revealed marked myelin pallor in the anterior as well as lateral corticospinal tracts. Pyramidal tract degeneration was prominent starting at the level of the cerebral peduncle and was continued to be seen until the level of lumbar cord. The number of anterior horn cells showed only slight decrease in the cervical level, however, it was normal in the lumbar cord.(ABSTRACT TRUNCATED AT 400 WORDS)

[Value of preoperative investigations in the so-called "cervical myelopathy" (author's transl)].

The particular value of clinical, radiological and electromyographical features is compared in 42 patients with motor deficit related to cervicarthrosic myelopathy or amyotrophic lateral sclerosis. The initial onset of the disease was identical (motor deficit and long tracts pathways involvement). Three different groups were identifyed according to the evolution: -- Group I: (13 cases): true lateral amyotrophic sclerosis which were not operated on. -- Group II (10 cases): myelopathy called "cervicarthrosic" because of radiological findings which were operated on but had the same steady worsened course as a lateral amyotrophic sclerosis. -- Group III (19 cases): cervical myelopathy which had surgery. The operation brought about stabilization or fairly good recovery over the 18 months following at least. From a clinical aspect, the "Lhermitte sign" or objective sensitive deficit are strongly significant for cervical myelopathy. On the contrary, diffuse fasciculations specially in the tongue seem to be mostly found in lateral amyotrophic sclerosis, whereas they are restricted into the paralysed area in cervical myelopathy. Electromyographic examination is decisive: simple activity with high frequency motor units (increased amplitude and polyphasic waves) or "preponderant potentials" into a cranial nerve territory or three segments of the lower limbs are frequently found in lateral amyotrophic sclerosis. These electromyographic features are less significant in the upper limbs. The neuroradiological findings lonely cannot assert definitely the cervicarthrosic origin of the myelopathy but visualize the conflicting situation between the spinal cord and the cervical canal and allow to choose the surgical procedure.

Clinical utility of reflex studies in assessing cervical radiculopathy.

We prospectively studied the diagnostic utility of upper limb segmental reflexes in patients with suspected cervical radiculopathy (CR). Fifty-three patients (29 men and 24 women), referred for electrodiagnostic testing, were positive for at least one of four clinical criteria for CR: abnormal (1) history, (2) motor (myotomal) examination, (3) sensory (dermatomal) examination, and (4) changes in deep tendon reflexes (DTR). All underwent electrodiagnostic assessment, needle electrode examination (NEE), specialized segmental reflexes (heteronymous and Hoffman's reflexes [H reflexes]), and neuroimaging. The clinical diagnosis was supported in all 32 patients who entered the study with two or more clinical signs for CR. Abnormal NEE was found in 90% of subjects with three clinical signs, 59% with two signs, and only 10% of those with one sign. H reflexes demonstrated a sensitivity of 72% and specificity of 85% for detection of CR and were particularly helpful when forming conclusions in the 21 subjects with only one clinical sign for CR. Specialized segmental H-reflex studies of the upper limb were as sensitive and specific as neuroimaging (magnetic resonance imaging).

[A 63-year-old woman with muscle weakness, myotonia, and parkinsonism].

We report a 63-year-old woman who presented myotonia and parkinsonism. The patient was well until 15 years of the age when she noted that the ring finger of her left hand at times flexed when she did not intend to do so. She noted weakness in her left upper extremity at the age of 40, and difficulty in relaxing her hand grip at 45. She had an onset of tremor in her right foot at age 50, which was followed by difficulty in gait and hand writing. She was admitted to Juntendo University Urayasu Hospital when she was 63-year-old. Her mother, two sisters, and a son were affected with similar muscle weakness and myotonia. Although some of them developed stooped posture in the late stage of the disease, none of them had overt parkinsonism. General physical examination was unremarkable. Neurologic examination revealed an alert and oriented woman with some recent memory loss. She had bilateral ptosis, facial weakness, and a masked face. Myerson's sign was present. Her speech was small and monotonous. The sternocleidomastoid muscles were markedly atrophic and weak. The remaining of the cranial nerves were intact. She walked in small steps with freezing with support. She showed bradykinesia, retropulsion, and resting tremor in her right leg. Slight distal dominant weakness was noted in both upper and lower extremities more on the left. No cerebellar signs were noted. Muscle stretch reflexes were within normal limits in the upper extremities and diminished in the lower limbs. Sensation was intact. Routine laboratory findings were unremarkable. Cranial CT scan and MRI revealed slight cortical atrophy and leukoaraiosis. She responded to levodopa and she became able to walk by herself. She was transferred to another hospital one month after her admission. She had several bouts of airway obstruction with one episode of respiratory arrest. She expired six month after the transfer. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that this patient suffered from myotonic dystrophy and Parkinson's disease which set in later years. Postmortem examination on the iliopsoas muscle revealed uneven muscle fiber diameters, central nuclei, and type 1 fiber predominance; the pathologic finding was consistent with myotonic dystrophy. The substantia nigra showed marked cell loss and Lewy bodies in the remaining neurons. The finding was consistent with Parkinson's disease. In myelin stain, diffuse myelin pallor was noted in the cerebral white matter which was the pathologic substrate of leukoaraiosis in this patient. Combination of these two disorders have never been reported in the literature to our knowledge. It appears to be that the coincidence is just a by-chance phenomenon, but it seems interesting to note that accelerated aging process appears to be present in both myotonic dystrophy and Parkinson's disease.

[A case of subacute necrotizing lymphadenitis complicated with brachial plexus neuritis].

A 22-year-old female noted a low grade fever and swelling of the cervical lymph nodes in May 1997, and later developed a dry cough. She was diagnosed to have interstitial pneumonitis, and then administration of corticosteroids alleviated her symptoms. On February 6, 1998, however, a high fever recurred and her swollen cervical lymph node on the right side was biopsied on February 9, 1998. A histological examination revealed an increased number of histiocytes and karyorrhexis of the lymphocytes in the paracortical areas, and she was therefore diagnosed to have histiocytic necrotizing lymphadenitis. She could not fully elevate her arm on February 16, 1998. On admission, her cervical lymph node was swollen on the left side. A neurological examination revealed a marked weakness of the right deltoid muscle, moderate weakness of the right latissimus dorsi, triceps and brachioradialis muscles and also a mild weakness of the serratus anterior, supra- and infra-spinatus, and biceps brachii muscles. The muscle power of the other muscles were normal and no muscle atrophy was evident. Winging of the right scapula was observed. The deep tendon reflexes were normal in all four limbs, and her sensation was also normal. No cerebellar sign was found. The Jackson, Spurling, Allen, Morley and Adson tests were all negative. ESR was mildly elevated to 18 mm/hr, but CRP was negative. RF, ANA and anti-SS-A and SS-B antibodies were positive, whereas LE-test, direct and indirect Coombs tests and other autoantibodies were negative. Needle EMG disclosed fasciculation potentials in the right triceps muscle and polyphasic waves in the right deltoid muscle. MRI showed gadolinium-enhancement of the right brachial plexus. Although an abnormal accumulation of gallium was detected in the right parotid and bilateral submandibular glands, no sicca symptoms were found and the Schirmer test findings were normal. Oral prednisolone (50 mg/day with gradual tapering) alleviated both her symptoms and the gadolinium-enhancement of the right brachial plexus. As a result, her right upper limb paresis was thus considered to have been caused by right brachial plexus neuritis, which was probably associated with histocytic necrotizing lymphadentis. Although acute cerebellar ataxia and meningitis have previously been reported to be complicated with histiocytic necrotizing lymphadenitis, this is the first report to describe the complication of peripheral neuritis with this condition.

[A 64-year-old man with recurrent blurred vision and an abdominal mass].

We report a 64-year-old man with recurrent bouts of blurred vision who died after developing an abdominal mass. He was well until June of 1985 when he was 59-years-old when he had an acute onset of loss of vision in his right eye. He was treated by prednisolone with a complete remission. In August of 1986, he had another bout of blurring of vision in his left eye. Once he lost his left vision completely, from which he showed slow recovery. In January of 1987, he developed blurring of his right eye and loss of pain and touch sensation in his right leg. Since then he repeated loss of vision in his right or left eye five times, and he was admitted to our hospital in May of 1990. On admission, he was alert and oriented. General physical examination was unremarkable. Neurologic examination revealed bilateral optic nerve atrophy. He could not discriminate light or dark by either eye. Other cranial nerves were unremarkable. He could walk in a wide-base only with support; spasticity was noted in his left leg. Muscle strength was preserved. Deep reflexes were exaggerated in both legs with extensor plantar reflex bilaterally. Pain and touch sensation was decreased in the left leg by 30%, and vibration was diminished in both feet. Position sense was preserved. Routine blood counts and chemistries were unremarkable. Cranial MRI scans revealed multiple high-signal intensity lesions in both pontine bases, basal ganglia, thalami, and in the deep cerebral white matters. He was treated with oral prednisolone, plasmapheresis, lymphocytapheresis, and then immuran. His vision showed only slight recovery to discriminate light and dark. In October of 1990, slight weakness appeared in his both legs. In December of that year, he developed nausea, and a fiber colonoscopic study revealed a stenosis in the transverse colon. In March of 1991, he developed anemia and liver dysfunction. In July of that year, jaundice appeared, and his serum bilirubin was increased. In October, his leg weakness became more prominent, and his cranial CT scans at that time revealed a low density change in the right cerebellum in the right superior cerebellar artery territory; in addition, multiple low density spots were scattered to be seen in both cerebral hemispheres including the basal ganglia and thalamic areas with ventricular dilatation and cortical atrophy.(ABSTRACT TRUNCATED AT 400 WORDS)

[Motor neuropathy with conduction blocks].

A series of 4 patients with pure, chronic and progressive motor neuropathy whose main clinical characteristics were asymmetric and distal weakness of the upper limbs, myokymia and fasciculations is presented. There were no sensory impairment and amyotrophy was observed in only one case. This picture suggested the diagnosis of motor neuron disease (MND). However, neurophysiologic examination demonstrated the presence of multifocal conduction blocks (CB) of the motor axons which were preferentially located in the proximal nerve segments and always at points atypical to nerve compression. The peripheral sensitive conductions and the somatosensory evoked potentials were normal, even through the nerve segments where the CB were located. Since this is a treatable potentially reversible syndrome, this motor neuropathy with CB should be included in the differential diagnosis of MND.

Cerebral palsy masking spinal muscular atrophy.

Spinal muscular atrophy (SMA) is an autosomal recessive anterior horn cell disease that results in progressive muscular weakness and atrophy without sensory involvement. A wide clinical spectrum that ranges from early death to essentially normal adult live exists. We describe a case of two 12 years olds, who represent two of three surviving non-identical quadruplets, born at 25 weeks gestational age. A diagnosis of hypotonic cerebral palsy (CP) was made in early childhood and early intervention services were initiated. At 3 years of age, MRI's showed white matter changes. Both briefly attained Gross Motor Functional Classification Scale (GMFCS) 3 status, but by 12 years of age their ambulatory abilities had decreased to Level 4. Physical Medicine and Rehabilitation (PM&R) physicians were consulted. On exam, distal lower extremities atrophy, hypotonia, hyporeflexia, and muscle weakness were noted. Neither child had upper motor neuron signs or spasticity. Cognition was normal. Neuromuscular disorder was suspected and genetic testing confirmed spinal muscular atrophy in both patients. While prior MRI/CT demonstrated static encephalopathy, recognition of symptoms and signs consistent with neuromuscular disease should have led to a secondary diagnosis. Therapeutic and surgical treatment decisions may have differed. Fragmentation of care and lack of a comprehensive team approach also contributed to the delay in recognition of their dual diagnosis.

Hoover's sign: Clinical relevance in Neurology.

Hoover's sign was described by Dr. Charles Franklin Hoover more than 100 years back to differentiate between the organic and functional weakness of pyramidal origin. This test is usually performed in the lower limbs and is valuable when on bedside one is not sure about the nature of hemiparesis. A subject with hemiparesis of organic cause while asked to flex the hip of normal leg against resistance will not exert pressure on the hand of examiner placed under the heel on the affected side while in hysterical weakness heightened pressure will be felt on the examiner's hand. The presumed genesis of this sign could be the crossed extensor reflex or the principle of synergistic contraction. It is a useful clinical test in differentiating functional and organic paresis with moderate sensitivity (63%) and high specificity (100%), but there are some limitations which should be kept in mind while evaluating a patient.

Coronavirus Disease 2019-Related Acute Ischemic Stroke: A Case Report.

Coronavirus disease 2019 (COVID-19) is an active worldwide pandemic with diverse presentations and complications. Most patients present with constitutional and respiratory symptoms. Acute ischemic stroke remains a medical emergency even during the COVID-19 pandemic. Here we present a case of a patient with COVID-19 who presented with acute ischemic stroke in the absence of common risk factors for cerebrovascular accidents. A 70-year-old male patient, with no prior comorbidities, presented to the emergency department (ED) with fever, cough, and shortness of breath for four days, and altered level of consciousness and right-sided weakness with the sensory loss for one day. On examination, the patient had a score of 8/15 on the Glasgow coma scale (GCS). There was a right-sided sensory loss and weakness in both upper and lower limbs with a positive Babinski's sign. The pulmonary examination was remarkable for bilateral crepitation. On blood workup, there was leukocytosis and raised c-reactive protein (CRP). D-dimer, ferritin, thyroid-stimulating hormone (TSH), vitamin B12, and hypercoagulability workup were normal. Transthoracic echocardiography was also normal. COVID-19 polymerase chain reaction (PCR) detected the virus. Chest x-ray showed infiltrations in the left middle and both lower zones of the lungs in the peripheral distribution. Computed tomography (CT) scan of the chest showed peripheral and mid to basal predominant multilobar ground-glass opacities. CT scan of the head showed a large hypodense area, with a loss of gray and white matter differentiation, in the left middle cerebral artery territory. Magnetic resonance imaging (MRI) of the head showed abnormal signal intensity area in the left parietal region. It appeared isointense on T1 image and hyperintense on T2 image. It also showed diffusion restriction on the diffusion-weighted 1 (DW1) image with corresponding low signals on the apparent diffusion coefficient (ADC) map. These findings were consistent with left middle cerebral artery territory infarct due to COVID-19. The patient was intubated in the ED. He was deemed unfit for thrombolysis and started on aspirin, anti-coagulation, and other supportive measures. Patients with COVID-19 should be evaluated early for neurological signs. Timely workup and interventions should be performed in any patient suspected of having a stroke to reduce morbidity and mortality.

Abductor sign: a reliable new sign to detect unilateral non-organic paresis of the lower limb.

To test a new neurological sign, the "abductor sign," which can distinguish between organic and non-organic leg paresis using synergic movements of the bilateral hip abductors. The subjects were 33 patients presenting with paresis of one leg, 17 of organic origin and 16 of non-organic origin (hysteria). To test the abductor sign, the examiner told the patient to abduct each leg, and opposed this movement with his hands placed on the lateral surfaces of the patient's legs. The leg contralateral to the abducted one showed opposite actions for organic paresis and non-organic paresis: for example, when the paretic leg was abducted, the sound leg stayed fixed in organic paresis, but moved in the hyperadducting direction in non-organic paresis. Hoover's sign was used for comparison in the same patients. The abductor sign gave the correct classification for all 33 cases. Hoover's sign was reliable if the results were carefully interpreted, but it was non-diagnostic for 16 patients because of strong hip extensors and in two because of strong hip flexors. Two patients with non-organic paresis succeeded in tricking the examiner by pretending full effort to lift the paretic leg. The abductor sign is a useful test to detect non-organic paresis, because (1) it is difficult for a hysterical patient to deceive the examiner, (2) the hip abductor is one of the most commonly involved muscles in pyramidal weakness, and (3) the results are easily visible as movement or non-movement of the unabducted leg.

Hirayama Disease Presenting as 4-Limb Paresthesia.

Hirayama disease is a rare clinical entity that presents typically as a unilateral, slowly progressive arms weakness, mostly occurring in young men. We report a case of Hirayama disease in a 20-year-old man presenting with a 4-year history of progressive paresthesia starting in his left arm, progressing to the right arm 1 year later. Four months before the presentation, he experienced bilateral foot paresthesias. Examination revealed weakness of the abductor digiti minimi, hallux extension weakness, and postural tremor bilaterally. He had hypersensitivity to pinprick in both hands with ulnar and median distribution. Sensory examination in the legs was normal. He had a postural tremor in both hands, which worsened on neck flexion. Spinal fluid analysis, including oligoclonal band testing, was normal. Electromyography demonstrated bilateral chronic C7 and C8 radiculopathies. Laboratory tests were normal. Flexion-extension magnetic resonance imaging demonstrated laxity of the dura and ligamentum flavum, with compression of cervical cord, maximal at C5-C6 in neck flexion. Laxity of the posterior dura during neck flexion has been postulated to lead to asymmetric lower cervical cord atrophy. Involvement of all 4 limbs is rare, and the condition can be mistaken for progressive multiple sclerosis.

[A case of amyotrophic lateral sclerosis with bilateral vocal cord paralysis necessitating tracheotomy].

Vocal cord movement disorders are increasingly recognized in patients with amyotrophic lateral sclerosis (ALS). We describe a patient with limb-onset ALS who developed vocal cord paralysis. A 74-year-old Japanese male consulted our clinic with a 6-month history of weakness in both arms. His family history was unremarkable. There were fasciculations and mild atrophy of the tongue and both arms. In the legs, muscle strength was almost normal but widespread fasciculations were present. All tendon reflexes were hypoactive and pathological reflexes were absent. Thereafter, he developed weakness of the legs and showed increased eating time. Babinski sign was positive bilaterally at this stage. The forced vital capacity dropped from 90% at the initial evaluation to 62% of the predicted value 14 months later. Two years after disease onset, the patient developed aspiration pneumonia with hoarseness and had difficulty clearing his throat of phlegm. Laryngoscopy demonstrated severe vocal cord paresis on both sides, particularly in the abductor muscles possibly leading to obstruction. Tracheotomy was performed because of the risk that the patient could choke to death. A review of the literature suggests that severe impairment of vocal cord abduction could be a prelude to sudden death in ALS. Follow up by laryngoscopic examination is necessary.