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A 67-year-old man was admitted to our hospital in May 2006 because of gait disturbance, delirium and myoclonus along with dermatitis and diarrhea. Those symptoms became worse in 3 months. He had undergone a gastrectomy, including a fundectomy and jejunal pouch interposition, for early gastric cancer at the age of 65 years. He had no habit of drinking alcohol or unbalanced diet. The triad of typical dermatitis, delirium, and diarrhea led to a diagnosis of pellagra, and all the symptoms disappeared after intravenous administration of nicotinate and vitamins. With a gastrectomy, fundectomy performed with jejunal pouch interposition has been regarded as a superior method for postoperative nutrition, but may cause vitamin deficiency. Thus, vitamin deficiency must be considered as a potential cause in neurologic patients who underwent surgical treatment for disorders of digestive tract, regardless of the procedure utilized.

We report two cases of idiopathic carotid-cavernous fistula (CCF) with primary symptoms of headache and diplopia. A 47-year-old woman presented with throbbing headache in her right frontal region followed by right trochlear nerve palsy. Brain magnetic resonance imaging (MRI) was normal but magnetic resonance angiography (MRA) and computed tomographic angiography (CTA) revealed abnormal signals around the right cavernous sinus. CCF was diagnosed by conventional angiography. The symptoms improved naturally but after about 1 year she suddenly exhibited conjunctival congestion. A 41-year-old man complained of fluctuating headache with sudden left abducens nerve palsy. MRI and MRA were normal but CTA showed abnormal signals around the left cavernous sinus. A final diagnosis of CCF was made by conventional angiography and he was transferred to another hospital for stereotactic radiosurgery. Diagnosis of CCF tends to be delayed in cases presenting with only headache and external ophthalmoplegia. However, CCF with cortical vein drainage can lead to cerebral hemorrhage and early correct diagnosis is needed. Our cases showed a dilated superior ophthalmic vein in enhanced CT and an abnormal signal around the cavernous sinus in CTA. Therefore, CTA may be useful as a relatively non-invasive method that can provide diagnostic clues for CCF.

Recently, some standard test batteries such as Boston Diagnostic Aphasia Examination and Western Aphasia Battery are frequently used to assess language abnormalities and to identify individual types of aphasia. Aphasia is, however, a neurologic disorder and differential diagnosis of the type should be made naturally in the neurological examination. Characteristics of verbal output are most important clinically for the classification. The table shows core symptoms of classical aphasic types. The figure presents a systematic approach to aphasia diagnosis based on several different patterns of aphasic output.

Automatic-voluntary dissociation in apraxia was discussed and the following two points were noted 1) This phenomenon is observed for praxis with procedural memory, i.e., learned or skilled movements such as brushing teeth and dressing, but not observed for praxis without procedural memory such as tasks used for testing constructional apraxia. 2) This phenomenon may be understood by dual motor act systems, i.e. implicit and explicit motor systems. Presumably, the former is mainly mediated by medial premotor system that involitionally drives motor procedural memory, while the latter is mainly mediated dorsolateral premotor system that volitionally drives motor procedural memory.

In the long course of Parkinson disease, we encounter the elevation of serum creatine kinase (CK) occasionally. Such elevation was not necessarily accompanied by severe symptoms as malignant syndrome. To delineate the basis of its situation, we selected the patients showing CK-elevation from 697 cases of Parkinson disease who had entered our hospital and their serum CK level had been measured. The cases with common cause of CK-elevation like trauma or myocardial infarction were excluded in advance. Those patients with CK-elevation were investigated with reference to age, gender, severity, duration of illness, dementia, and psychiatric symptoms retrospectively. High CK level was observed in 95 cases who were composed predominantly of advanced male patients. No obvious anticipatory cause of CK-elevation like a modification of anti-parkinson drug was recognized in 65 cases. On the other hand, CK-elevation caused by the modification of anti-parkinson drug was recognized in 10 cases. CK-elevation was observed in patients with dementia, delirium, and hallucination at higher rate. Most of these patients with CK-elevation did not show high fever and did not necessarily meet the criteria of malignant syndrome. However, 9 cases who showed marked increase of CK level over 10 times of upper limit of normal value contained some cases who had features of malignant syndrome. In Parkinson disease, especially in advanced cases dopamine may be unstable controlled in a few locations of their brain. Some situation of the disease may elicit imbalance of dopamine in patients' brain and induce CK elevation as in the similar condition in which neuroleptics are administrated.

We report a 23-year-old female with infantile onset chronic inflammatory demyelinating polyneuropathy. Muscle weakness was noticed when she was 1 year old, and, together with sensory disturbances, gradually progressed in an asymmetric manner. Nerve conduction studies disclosed slowing of conduction velocities, temporal dispersion, and decreased amplitude of compound muscle action potentials, the degrees of which were markedly different amongst different nerves even in the same limbs. The sural nerve biopsy showed various degrees of nerve fiber amongst different fascicles. Marked inter-nerve and intra-nerve differences of involvement and clinical improvement after steroid therapy supported the diagnosis of chronic inflammatory demyelinating polyneuropathy. It should be noted that even after a long clinical course of 23 years, her clinical symptoms remained asymmetrical and multi-focal lesions could be clearly demonstrated.

Bilateral vocal cord abductor paralysis (VCAP) is frequently associated with multiple system atrophy (MSA) and the early clinical manifestation of VCAP is nocturnal inspiratory stridor simulating heavy snoring observed in patients with obstructive sleep apnea syndrome. We examined six MSA patients with nocturnal stridor and four disease controls including sleep apnea syndrome. Vocal cord movements were analyzed by laryngofiberscopy during both wakefulness and sleep induced by intravenous administration of diazepam. The results were as follows: First, the stenotic portion in the upper airway tract was the larynx (the vocal cords) in MSA patients with stridor, while the soft palate or the pharynx in the disease controls. Second, in the MSA patients, while awake-laryngofiberscopy showed abduction restriction suggestive of VCAP in only one of the six patients, sleep-laryngofiberscopy showed obvious paradoxical movement of the vocal cord in all the rests, where the vocal cords abducted in expiration and adducted in inspiration. In addition, there were two patterns in the inspiratory vocal cord position during sleep: one pattern where vocal glottis was still opening at the posterior one-third area and the other pattern where vocal glottis was almost completely closed through total length of the cords. Tracheostomy should be considered in the latter stage of VCAP.

In the nerve conduction study (NCS), the complex repetitive discharge (CRD) which follows M wave is occasionally observed. To understand the origin of CRD, we clinically analyzed the cases with CRD. Among the 1,580 cases examined by NCS, 13 (0.82%) cases including 6 of myotonic dystrophies (MyD), 2 of carpal tunnel syndrome and a case of multiple sclerosis, chronic inflammatory demyelinating polyradiculoneuropathy, compression fracture of a lumbar vertebra, myasthenia gravis (MG) or Isaacs' syndrome had CRD. CRD was divided into forms; one consisted of an attenuating train of the complex of muscle potentials (muscular form) and the other one formed a simple repetition of single motor unit action potential (neural form). MyD, MG, and Isaacs' syndrome showed muscular form, and the others neural form. The pathophysiology of CRD was considered from the sites of lesions of nerve terminals, neuromuscular junctions, or muscle fibers and that the neural form might result from the axonal regeneration of peripheral motor nerve fibers.

The Ellsworth-Howard (EH) test was performed in 16 patients with myotonic dystrophy (DM), who were divided into two groups according to serum calcium level; Group I showing normal serum calcium (8 patients) and Group II with hypocalcemia (8 patients). Patients in Group II were recognized as having pseudohypoparathyroidism (PHP) type II and those in Group I as normal. Therefore, it was suggested that an abnormality of A kinase might be present in Group II patients. We additionally performed the thyrotropin releasing hormone (TRH) tolerance test in the same patients and 7 normal controls to examine an abnormality of C kinase. delta TSH (delta TSH: peak minus pre TSH) values in DM patients were significantly lower than those in normal controls. Moreover, delta TSH values in Group II were significantly lower than those in Group I. It was suggested that the abnormality of C kinase might be present in Group II patients. Taken together, our results indicated that some patients with DM might possess abnormalities of signal transduction of both A and C kinases. We compared the degree of endocrine involvement determined by both the EH test and the TRH tolerance test with expanded DNA fragment (EF) size determined by standard Southern blot analysis using an appropriate cDNA probe (cDNA25 probe). There was significant negative correlation between EF size and the results of the EH test and negative correlation between the EF size and the results of the TRH tolerance test. These findings suggested that EF size might be correlated with disease severity in affected endocrine organs.

We reported a case of myokymia with abnormal F response. A 60-year-old male with chronic alcoholism was admitted to our hospital with complaint of muscle cramp in both legs just after drinking. Neurological examination revealed muscle pain and weakness of the legs, absence of bilateral Achilles tendon reflexes and prominent myokymia in his right quadriceps femoris and left calf muscles. The electrophysiological examination showed reduced conduction velocity, high amplitude, increased number of phases and long duration of F responses suggesting increased excitability of motoneuron pool. The epidural nerve block brought about a disappearance of the myokymia and an improvement of the abnormal features of F response. The myokymia gradually tended to be milder in the clinical course. The relationship between the myokymia and the abnormal F responses indicated that the increased excitability of spinal motoneurons might play an important role on the generation of myokymia of this patient.

We reported a case of neuroleptic malignant syndrome (NMS) associated with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). A 71-year-old woman, who had been diagnosed as hypertension and multiple cerebral infarction, was given sulpiride 150 mg daily for depressive state. Three days after started sulpiride, she developed fever, sweating, difficulty of movement and was admitted to the hospital. The white blood cell count rose to 16,300/mm3 and serum creatine kinase (CK) to 3,063 IU/L. Two days later CK rose to 20,050 IU/L regardless of stopping the drug, so she was transferred to our hospital for further investigation. On admission, it was the 6th day from the onset, she was mute and akinetic accompanied by muscle pain and rigidity in extremities. Serum CK was 1,831 IU/L, Na 122 mEq/L, osmolality 244 mOsm/kg, plasma antidiuretic hormone (ADH) level 6.5 pg/ml and urine Na was 101 mEq/L, osmolality 467 mOsm/kg. Renal and adrenal functions, plasma renin activity were normal. From the history, course and these data, diagnosis of NMS associated with SIADH was made. Intravenous sodium (130-200 mEq/day) and fluids (1,000-1,200 ml/day) were carefully infused. She became active, muscle pain disappeared and rigidity, akinesia decreased. CK, serum Na and osmolality gradually improved to normal. About the transient increase in ADH secretion, we considered that hypothalamic disturbance in NMS might induce leakage of stored ADH from neuroendocrine neurons in it.

We present a case of acute severe demyelination affecting both CNS and PNS with pathological evidence. A 62-year-old man presented with acute onset of coma preceded by common cold-like symptoms. He was diagnosed as acute disseminated encephalomyelopathy (ADEM), and died of brain herniation in two weeks. At autopsy, in addition to severe demyelination of the white matter of the central nervous system, there was widespread acute demyelinating process in the peripheral nervous system. Myelin destruction by macrophage was most conspicuous in the spinal nerve root with preservation of the axon cylinder. So far the association of hypertrophic demyelinating neuropathy was reported in cases of multiple sclerosis. However, there have not been any papers in which substantial involvement of both CNS and PNS in cases of ADEM except for the clinical report by Amit. Antigenic cross-reactivity between CNS and PNS against myelin proteins or other antigens like glycolipid may elicit similar immune responses producing demyelination.

Clinical characteristics of late deterioration in adult cerebral palsy were reported with detailed neurological evaluations and analyses. 10 adult cases, 9 male and 1 female, with cerebral palsy (CP) were included aged from 24 to 58 years on admission. Without marked mental retardation all had been ambulant and completely independent of ADL with residual spasticity and/or dyskinesia of minimal degree until the second or third decade. Late deterioration of functional abilities starting with numbness or pain in upper extremities at age 24-45 (mean: 36.2 y), associated with profound atrophy of the shoulder girdle and hand muscles. Dyskinesia and spasticity markedly aggravated with urinary and respiratory dysfunctions, resulting in tetraplegia in a couple of years. Mentality is generally unaffected, however, severe dementia occurred in one case. Intensive clinical examinations revealed no particular abnormalities except for mild segmental neurogenic changes by needle EMG. Neuroradiological surveys revealed a marked narrowing of upper to middle cervical spinal canal with deformity and shrinkage of the corresponding cord in most cases. Cranial CT scans and MRI were unremarkable except for diffuse cortical atrophy and ventricular dilation. These studies showed that in adult CP an unexpectedly severe deterioration of sensory, motor and/or mental functions may appear even in previously well achieved cases. These dramatic changes of the clinical features of CP after middle age might be suggestive of the degenerating process and precocious aging of the CNS.

Sudomotor function was evaluated by using the sympathetic skin response (SSR) and the sweat response to intradermal acetylcholine (ACh) injection in 69 patients with Parkinson's diseases (PD). The incidence of SSR abnormality (34.8%) was as high as that of orthostatic hypotension (30.4%) and increased with the severity of the illness. Anticholinergic drug did not influence the incidence of SSR abnormality. Therefore, the SSR is useful in evaluating sudomotor efferent pathway in PD patients. Moreover, in all patients, sweat response to ACh showed a reduced number of excitable sweat glands and a low volume of sweat. In a patient in whom sweat response to ACh was markedly impaired, however, the density of acetylcholinesterase-positive unmyelinated fibers in biopsied sural nerve was in normal range. Therefore, this is considered to indicate functional disturbance of the postganglionic sympathetic fibers in PD patients, without morphological changes.

An 18-year-old Japanese female with pseudohypoparathyroidism (PHP) type Ib presented with rhabdomyolysis. The patient, who had been tired after long-distance hiking, experienced paresthesiae and subsequent muscle cramps of the four extremities after drinking a small quantity of alcohol. An occurrence of rhabdomyolysis was confirmed by marked elevation of serum CK activities and muscle necrosis revealed by both CT scanning and biopsy. The exertion and alcohol intake probably induced the initial muscle injury. Subsequently, the muscle necrosis might have worsened the asymptomatic hypocalcemia and decreased 1,25-(OH)2 vitamin D synthesis in PHP type Ib, which resulted in severe muscle cramps and further muscle destruction.

The authors analyzed rhythmical involuntary movements at rest, which appeared as complications in 12 (sporadic 11, hereditary 1) out of a total of 139 cases (sporadic 99, hereditary 40) of olivo-ponto-cerebellar atrophy. These movements tended to be seen in patients with sporadic OPCA of longer illness duration and at more advanced stages. They were distributed over the face, neck and extremities. Palatal myoclonus was observed in only one case. The movements were exacerbated by maintenance of a fixed posture, motion and mental stress, and stopped during sleep. In some cases, clonazepam, trihexyphenidyl or 1-Dopa was effective. In the surface electromyogram, rhythmical grouped discharges of 2-4 Hz were recorded only on agonist muscles or on both agonist and antagonist muscles synchronously, which is characteristic of skeletal myoclonus. However, pathological study of 3 cases with involuntary movements revealed marked putaminal degenerations as compared with 3 uncomplicated cases. This suggests that these rhythmical movements might be related to parkinsonian tremor.

We have reported a 50-year-old woman with HTLV-I associated myelopathy (HAM) who had bilateral abductor vocal cord paralysis. The symptoms and signs were slowly progressive spastic paraplegia, dysuria, inspiratory stridor, and snoring during sleep. She had no hoarseness. Titers of anti-HTLV-I antibody were elevated in both the serum and cerebrospinal fluid. FEV1.0% on the spirogram was reduced to 66%. The fiberscopic examination demonstrated the abductor limitation of the vocal cords during the inspiratory phase. During induced sleep after the intravenous administration of thiopental sodium, this abductor paralysis was worsened, producing a high pitched inspiratory stridor. The adduction was not disturbed at all. Needle electromyogram of the posterior crico-arytenoid muscle which is a sole abductor muscle revealed the high amplitude up to approximately 1.0 mV (normal less than 0.8 mV) with poor interference pattern, indicating neurogenic changes. After 2 months course of prednisolone (60 mg/alternative day), FEV1.0% was recovered to be 92% with the improvement of the gait disturbance, which suggests the abductor vocal cord paralysis is related to HAM. The abductor vocal cord paralysis in HAM would require a careful follow-up observation to protect the respiratory failure in the advanced stage.

A 73-year-old woman suffering from the acute onset monoparesis of her right arm which followed the skin eruption with mild sensory disturbance of right C4-6 level, was reported. Electrophysiological examinations revealed the brachial plexus neuritis and axonal degeneration of the proximal portion, with the evidence of herpes zoster infection. Her paresis of the right arm gradually improved without any medication during her hospital course. It was concluded that herpes zoster should be considered to be one of the causes of acute onset brachial plexopathy.

We reported two cases of brothers demonstrating oculopharyngeal muscular dystrophy (OPMD). The cases had consanguineous parents and five healthy siblings, which suggested the autosomal recessive inheritance. The initial symptom was slowly progressive blepharoptosis with onset in the third decade. On examination, total external ophthalmoplegia was observed in both patients. Additionally, the elder, a 57-year-old man, exhibited dysarthria, dysphagia and muscular weakness with atrophy of the face, bilateral proximal upper limbs and diffuse lower limbs. The younger brother, a 55-year-old man, displayed muscular weakness and atrophy distributed in the face and four limbs. Muscle biopsy of both cases revealed rimmed vacuoles and spheroid bodies in the atrophic and normal-sized fibers. Biochemical study of the biopsy specimens of the elder brother disclosed the myophosphorylase activity reduced to about 40% of the normal value, although in the younger brother, that activity was normal. OPMD is usually inherited in the autosomal dominant mode, and autosomal recessive OPMD is rare. The onset age of our cases was younger than that of the autosomal dominant OPMD. There were some differences in the clinical manifestation between the presented cases, which could be interpreted as phenotypic variation. The elder brother was thought to be associated with McArdle's disease.

An 18-year-old high-school boy presented facial muscle weakness since birth, and then developed wasting around the neck, shoulder girdle, upper arms, and thighs. He was born to un-consanguineous parents. His father had suffered from similar but milder muscle atrophy with predominance on the right side of the face and shoulder girdle since adolescence. His mother and his only sibling were clinically unaffected. Hearing disturbance was detected at the age of 6, and he also noted atrophy of the tongue and the bilateral thighs at the age of 10. The symptom progressed gradually. Neurological examination on admission revealed a well-developed boy (166 cm/60 kg) with a prominent facial diplegia with distinct proximal muscular atrophy of the extremities. Muscles of the tongue, neck, upper arms, shoulder and pelvic girdles, and hamstrings were markedly involved. The anterior tibial muscles were also affected, while the calf muscles were hypertrophic. High arched palate, X legs, mild lordoscoliosis were also noted. Serum CK was slightly increased (424 IU/l), and needle EMG in the extremities including the tongue revealed myopathic changes. Muscle CT demonstrated marked atrophy of the proximal muscles in the lower limbs and hypertrophy of the calf muscles. Audiogram showed bilateral sensorineural hearing disturbance. Muscle biopsy of the gastrocnemius showed myogenic as well as neurogenic changes consisting of atrophic and hypertrophic fibers with interstitial cellular infiltration, and type I fiber predominance. With these family history as well as clinical and laboratory examinations, this case could be diagnosed as "congenital facioscapulohumeral muscular dystrophy".(ABSTRACT TRUNCATED AT 250 WORDS)

In order to elucidate the clinical features and the prognosis for life in the patients with multiple system atrophy (MSA) presenting with vocal cord paralysis (VCP), we studied the correlation between VCP and other neurological findings including cerebellar, pyramidal, extrapyramidal and autonomic nervous signs. Subjects were 48 MSA patients: 23 with VCP and age- and illness duration-matched 25 without VCP. MSA in this paper comprised clinically Shy-Drager syndrome, olivopontocerebellar atrophy, and striatonigral degeneration. MSA patients with VCP had in general more severe neurological findings, compared with those without VCP. Urinary incontinence developed in the relatively early stage of illness and preceded VCP in all patients. VCP developed not only in far-advanced stage but at any time in the course of illness. As to swallowing function when a diagnosis of VCP was established, about half of the patients with VCP needed nasogastric tube feeding and the remaining half tolerated oral feeding. VCP correlated strongly with urinary incontinence but not always with the severity of orthostatic hypotension or extrapyramidal tract sign such as parkinsonism. Five of the eight patients without tracheostomy came to sudden death. The mean duration from making a diagnosis of VCP to death was 1.1 years. In contrast, nine of the 11 patients with tracheostomy were alive and the survival periods after tracheostomy reached a maximum, five years. These facts suggest that the prognosis for life in the patients with VCP depends in part upon whether tracheostomy was carried out or not.(ABSTRACT TRUNCATED AT 250 WORDS)

We studied the energy metabolism of ALS patients under mechanical ventilation and tube feeding. Gas exchanges (O2 and CO2 content in expiratory and inspiratory gas) were measured all day long by DELTATRAC (Datex, Finland) in 11 ALS patients, and energy metabolism during 24 hours was calculated according to the next formula; 5.67 VO2 + 1.60 VCO2-2.17 UN (VO2; O2 consumption l/min, VCO2; CO2 production l/min, UN; urea nitrogen excretion in urine g/day). All patients were clinically stable under continuous mechanical ventilation and tube feeding, and did not have any infection such as pneumonia. The patients were 23-70 years old (mean 49.3), and had total clinical courses of 3-12 years (mean 7.1), and 2-8 year-long courses under mechanical ventilation (mean 4.6). They were classified into the next 3 groups: group I; totally locked-in state (2 patients), group II; complete tetraplegia (6 patients), group III; incomplete tetraparesis (3 patients). Basal metabolic rate (BMR) of each patient was also calculated from Harris-Benedict's formula; male = 66.47 + 13.75W + 5.0H - 6.76A, female = 665.10 + 0.567W + 1.85H - 4.68A (W; weight kg, H; height cm, A; body surface area m2). And the changes of the body weight by month were examined retrospectively in 26 ALS patients with at least 2 year-duration under mechanical ventilation, which include the previous 11 patients. The calorie consumption of 24 hours were 783.3 kcal (group I), 875.3 (group II), 974.9 (group III), which were all lower than BMR (I; -26.8%, II; -17.6%, III; -11.3%).(ABSTRACT TRUNCATED AT 250 WORDS)

A 32-year-old right-handed man suffered a traffic accident with head injury, resulting in loss of spontaneity, right hemiparesis, severe aphasia, and unilateral apraxia, which was noticed on his non-paralyzed left hand. An MRI scan conducted 11 months after onset revealed a large lesion in the left frontal lobe, a small lesion in the right frontal lobe and a striking thinning of the trunk of the corpus callosum with remarkable dilatation of the lateral ventricles. An IMP-SPECT scan, performed 1 year after onset, showed a diffuse hypoperfusion extending to the left temporo-parietal area further than the MRI verified abnormal density areas. The patient's praxic abilities were precisely evaluated 1 year after onset. The performance on the object use task was characterized by content errors. For example, when using a match with his left hand, he always treated with it like a cigarette. The right hand performance of this task was clumsy due to his right hemiparesis but successful. The performance on his left hand did not improved even when the patient was given visual examples. The term "ideational apraxia" proposed by Liepmann, which was characterized by content errors, implies a conceptual deficit. Morlaas defined ideational apraxia as a agnosia of usage. Ideational apraxia has been so far investigated mainly on patients with these difficulties in both hands. On the other hand, left unilateral apraxia has been explained by callosal disconnection. Liepmann and Maas thought that a lesion of the corpus callosum would prevent the space-time engrams in the left hemisphere from reaching the right sensorimotor area necessary to carry out the skilled act with the left hand, thereby inducing apraxia.(ABSTRACT TRUNCATED AT 250 WORDS)

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with central nervous system (CNS) demyelinating lesions has recently been reported to mimic multiple sclerosis (MS). In this paper, a series of patients with CIDP were examined to see if they had CNS involvement. CIDP patients with CNS lesions were then compared to patients with MS with peripheral nervous system (PNS) involvement for similarities. CNS and PNS involvement were detected by clinical symptoms, neurological findings, neuro-otological and neuro-ophthalmological tests, electrophysiological examinations such as electroencephalography, evoked potentials, blink reflex, conventional peripheral nerve conduction studies and electromyography, as well as computed tomography and magnetic resonance imaging (MRI). There were 7 of 17 CIDP patients with CNS involvement, but only 2 of 59 MS patients with PNS lesions were found. The rate of CIDP with CNS involvement (41.2%) was higher than that of MS with PNS lesions (3.4%). The CNS signs and symptoms of 7 CIDP patients were not so constant as their PNS symptoms, and consisted of 1 case with optic neuritis, 4 cases with cerebellar atxia and/or nystagmus, and 3 cases with spinal symptoms. These signs and symptoms are all well known in MS. Prolonged latencies on evoked potentials and high signal white matter lesions on T2 weighted MRI, indicating demyelinating CNS lesions were also similar to those found in MS. The CNS involvement in those patients with CIDP was therefore similar in character to those found in MS, but was far less severe than the PNS finding.(ABSTRACT TRUNCATED AT 250 WORDS)

We described clinical and neuropathological findings of a case of primary cerebral lymphoma with central neurogenic hyperventilation (CNH). A 54-year-old awake woman with a primary cerebral lymphoma presented hyperventilation for two weeks. Arterial blood gas showed severe respiratory alkalosis; PH 7.603, PaCO2, 10.5 mmHg, PaO2 129.8 mmHg, HCO2 10.4 mmol/L, BE -8.0, O2SAT 98.9%. Rebreathing from a paper bag, and intravenous administration of diazepam and sodium bicarbonate failed to alter the respiratory pattern. Consecutive CAT scans indicated that CNH didn't occur when the tumor extensively invaded the cerebral cortex, cerebellum, thalamus and basal ganglia but was initiated when the lymphoma invaded the brain stem. Pathological study showed lymphoma cells invaded the cerebral cortex, cerebellum, thalamus and basal ganglia severely, and the brain stem moderately and recently. Contrary to the cases reported by Plum, Lange and Bateman, the lower medulla was also involved. Possible mechanisms for CNH are discussed in relation to the pathological findings and consecutive CAT scan findings.

Two brothers, 31 and 25 years of age, with distal hereditary motor neuropathy (distal HMN) and choroideremia are reported. Their parents were second cousins. During the first decade, their visual acuity gradually diminished with night blindness, accompanied by weakness of the legs. From the third decade, they noticed weakness of the hands. The neurological signs of both cases could be summarized as follows: bilateral pes cavus, Charcot-Marie-Tooth type distal atrophy and weakness of the four limbs, no sensory deficit, hyperreflexia in the upper limbs and knees, absent ankle jerks and plantar reflexes, and severe visual impairment due to choroideremia. In addition to these signs, the younger patient showed nystagmus and limb kinetic ataxia suggesting cerebellar involvement. Muscle biopsies and EMG studies revealed chronic neurogenic changes. MCV's in the median nerves were normal, but the extensor digitorum brevis muscles were totally denervated. SCV's, the amplitude of sensory action potentials and sural nerve biopsies were normal. In both patients, elevated CSF protein without pleocytosis and high level of serum IgA & E were demonstrated. Associations of choroideremia and abnormal laboratory findings as noted in our cases have not been so far reported in distal HMN, but we suppose these disorders may be genetically related.

We reported an autopsy case of thrombotic occlusion of the superior cerebral vein with hemorrhagic laminar necrosis of the right parietal cortex. A 68-year-old woman was admitted to our hospital because of a severe headache and left hemiplegia of acute onset. There was a past history of hypertension, fever of unknown origin, leukocytopenia and nasal dermatitis. Magnetic resonance images (MRI) disclosed thrombosis of the superior sagittal sinus and of the right parietal cortical vein as well as right parieto-occipital cerebral infarction. Although she improved with mild sequelae, the subsequent MRI showed a recurrent thrombosis of the superior sagittal sinus. Ten months after the onset she died suddenly, presumably due to acute myocardial infarction. Pathologically, thrombotic occlusion of the right parietal cortical vein, recurrent thrombosis of the superior sagittal sinus and old hemorrhagic cortical laminar necrosis of the right parietal region were revealed. Moreover, intracranial arteritis and phlebitis were observed, as well as arteriolitis in the peripheral nerves. In our case, MRI was useful for the diagnosis and following the course of cerebral venous thrombosis. Cerebral noninfective vasculitis may well have caused the venous thrombosis.

Four Japanese cases of postpolio progressive muscular atrophy (PPMA) of late onset were clinically studied to characterize their clinical features and discussed upon the epidemiologic viewpoint. Four male patients were included with mean age of 43.5 (30-59) years on examination, diagnosed as PPMA after the criteria by Alter (1982) and Dalakas (1986). All suffered from polio at age 10 month to 8 years (mean: 3y) between 1931 and 1957 with residual motor paresis in one limb or two. Twenty-eight to 55 (mean: 40.3) years later, subsequent muscle weakness and atrophy appeared in the limb seemingly unaffected by the initial polio attack. Marked fasciculations and occasional myalgias were noticed without sensory disturbance and bulbar as well as upper motor neuron signs. Laboratory examinations revealed moderate elevations of serum CK and protein content in SCF. No significant elevation of polio virus antibody titers was found in both serum and CSF. Electromyography showed neurogenic changes of various degrees by muscle tested. Muscle CT disclosed patchy distribution of atrophied muscles with fatty replacement in all extremities. Spinal cord MRI images were unremarkable. Scatters of small grouped atrophy and fiber type grouping were noticed on muscle biopsy specimens. These findings summarized in our cases are generally compatible with those of hereby reported PPMA cases. On reviewing the western literature and the great epidemic of polio around 1960 in Japan, an increasing number of patients with PPMA can be anticipated in near future; possibly in 10 years.

A 60-year-old female was admitted because of intermittent fever, arthralgia, itching of whole body, pretibial edema, urinary incontinence, pain of both legs and gait disturbance, after an insect bite. On admission, she had fever of 38 degrees C, and nuchal pain and stiffness. Neurological examination revealed spasticity of lower legs and increased deep tendon reflexes of all extremities. Hyperesthesia and hyperalgesia were noted on C2-4 and L5-S5 areas. Leukocyte count was 10,100/mm3 and CRP was 2+. CSF showed no pleocytosis (3/mm3, lymphocyte), but total protein (50 mg/dl) and IgG (10.5 mg/dl) were increased. On T2-weighted images of brain MRI, multiple small high signal areas were shown. The symptom improved markedly by prednisolone, but 3 months later left lateral gaze palsy appeared abruptly. A demyelinating lesion of the pons to the medulla oblongata including the left paramedian pontine reticular formation was suspected, and a corticosteroid pulse therapy was very effective. Serum titer of anti-Borrelia burgdorferi-IgG antibody by indirect immunoperoxidase method was 400 x at first and 1600 x after 3 months. Neuroborreliosis was diagnosed, but high doses of intravenous penicillin were not effective, and an immune-mediated demyelinating mechanism was probably thought to play a role in the pathogenesis of neuroborreliosis.

In vitro immunoglobulin (Ig) synthesis was studied using peripheral blood mononuclear cells (PBM) from 12 patients with myasthenia gravis (MG). In cultured PBM, levels of secreted IgG, IgM and IgA measured by ELISA, as well as the number of IgG-, IgM-, and IgA-secreting cells determined by reversed plaque assay, were higher in MG than in controls; this was not dependent upon the stimulation of PBM with pokeweed mitogen (PWM). PBM from 9 of 12 MG patients synthesized anti-acetylcholine receptor (AChR) antibodies in vitro in the presence of PWM, while PBM from 7 controls did not. Patients whose PBM secreted lower levels of each Ig class also secreted fewer or no anti-AChR antibodies. Levels of anti-AChR antibodies secreted in vitro showed a high degree of correlation with titers of sera from patients (r = 0.86). The results suggest that the process of anti-AChR antibody synthesis by PBM in vitro is related to that of Ig synthesis, and that PBM plays, at least in part, an important role in the production of anti-AChR antibodies in MG.

A 72-year-old man suddenly developed severe muscle weakness following the treatment with pindolol (Calvisken) for three days. Neurological examination on admission disclosed marked proximal muscle weakness with absent deep tendon reflexes. Laboratory data showed significant increase of serum CK, aldolase and myoglobin. Electromyography revealed both neurogenic and myogenic changes in all muscle tested. Skeletal muscle CT showed patchy low density areas in muscles of thigh and leg, especially in the hamstrings. Femoral muscle biopsy demonstrated a granulomatous nodule with multinucleated giant cells in the degenerated muscle fibers showing small-grouped atrophy. By Mb-PAP staining, Mb-negative fibers were randomly distributed among normally stained ones. Leu 1, 3a, 4, HLA-DR positive cells were found adjacent to the granuloma by immunoperoxidase staining. After immediate withdrawal of pindolol and treatment with steroid, he recovered muscle strength and enzyme activities were normalized in a week. Beta blockers have been known to induce muscle cramps or pain and moderate elevation of serum muscle enzymes. However, severe muscle weakness with highly elevated enzyme activities leading to rhabdomyolysis as noticed in the present case was rarely reported in the literature. Underlying sarcoid myopathy might be suspected to exaggerate this unusual case of pindolol-induced rhabdomyolysis. A careful use of pindolol is emphasized.

We have already described that ragged red fiber (RRF), core/targetoid fiber and type 1 fiber predominance were found at autopsy in the diaphragm taken from patients with chronic obstructive pulmonary diseases. The purpose of the present study is to investigate morphological and histochemical changes in the diaphragm in denervating neurologic disorders. The diaphragm in the costal portion was taken from 22 autopsy cases including 4 with amyotrophic lateral sclerosis (ALS), 4 cerebrovascular diseases, 3 Parkinson disease, 2 olivopontocerebellar atrophy. In addition, 4 diaphragm muscles were biopsied at the time of surgery for lung cancer. In the diaphragm we observed not only RRF and core/targetoid fiber but also cytoplasmic body and ring fiber in many cases. These findings were, however, not specific for neurologic disorders. Focal cytochrome c oxidase deficiency was found in muscles with RRF. It should be emphasized that RRF was absent in 3 of 4 cases with ALS and in a case with elevated hemidiaphragm from phrenic nerve paralysis. In the previous report, we suggested that RRF was formed under the relative ischemic state in overworking diaphragm. The relative ischemia means a condition that oxygen (energy) demand for respiratory work exceeds over oxygen supply from the blood in the overworking diaphragm. The reason why no RRF was found in the denervated muscle is that the ischemic state in the denervated muscles is relieved by immobilization after denervation. Karpati et al conformed that denervation prevented ischemic state in the muscle. Other histochemical features in the diaphragm included cytoplasmic body and ring fiber.(ABSTRACT TRUNCATED AT 250 WORDS)

The patient, a 31-year-old married woman, noticed spasticity on walking at the age of 19 accompanied by ataxia, dysarthria and dysphagia. Facial twitching and dystonic movement of extremities have been observed since age 27. A sister of her father showed the similar ataxia and dysarthria, and expired of pneumonia at the age of 45. On admission at the age of 29, neurological examinations revealed nystagmus, marked spasticity with pathological reflexes and clonus, cerebellar ataxia, dysarthria and dysphagia, diffuse muscle wasting, fasciculation in facial musculature, and generalized slow dystonic movement. By neuro-otological studies bilateral MLF syndrome with upward gaze limitation and decreased velocity of saccadic eye movement were detected. Surface EMG at rest showed a dystonic discharges on the extremities. Needle EMG disclosed a systemic neurogenic change with reduced interference and high amplitude potentials. Atrophy of the brainstem was remarkable on the cranial CT and MRI. These abnormal eye movements, especially bilateral MLF syndrome and generalized dystonia seem to be quite unusual in the variety of spinocerebellar degenerations. On reviewing detected clinical descriptions on Joseph disease this case can be probably included.