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We report a patient with a relapsing form of acute ataxic polyradiculoneuritis. The patient developed marked sensory ataxia with mild limb weakness following a respiratory tract infection. Five similar relapses occurred over 18 years. The symptoms reached their maximum in 2 weeks, followed by subsequent gradual improvement over 1-2 months. Sural nerve biopsy showed mild loss of large myelinated fibers.

In a 64-year-old woman with a clinical diagnosis of corticobasal degeneration, a hyperexcitability of central motor tract was demonstrated. She had akinesia, regidity, myoclonic movement, hyperreflexia, forced grasping and clumusiness on the right extremities. The asymmetry of these symptoms was striking. Dopa therapy was not effective. On single photon emission computed tomography (SPECT), blood perfusion was reduced especially on the left side in the frontoparietal area. She had large C-reflex after the median nerve stimulation only on the affected side. The intensity threshold of motor evoked potential by stimulation on the left motor cortex was lower than that on the right motor cortex. Conditioning stimulation of the median nerve on the affected side, greatly increased MEP size as compared to those of normal subjects, at conditioning-test (C-T) intervals of 40-80 ms; whereas, stimulation on the contralateral side did not. On the other hand, SEP did not have a marked increase or asymmetry in size and SEP recovery curve showed no hyperexcitability. These results indicate there is a hyperexcitability of the central motor tract on the affected side.

Magnetic stimulation over the cervical spine using the 8-shaped coil was studied to determine the optimal site and intensity of stimulation. Twenty-one healthy volunteers and 9 patients with acute inflammatory demyelinating polyradiculoneuritis (AIDP) or chronic inflammatory demyelinating polyradiculoneuritis (CIDP) were examined. Each wing of the 8-shaped coil had a 5 cm outer diameter and the field (maximum 2.2 tesla) was produced with MAGSTIM200 (MAGSTIM company). The coil was placed along the line from the seventh cervical prominence to the acromion on the recorded side (C7 standard line) to give orthodromic stimulation to the motor nerve. The coil position was marked at the contact point of each wing. Motor evoked potential (MEP) was recorded from abductor digitorum minimi muscle by using surface electrodes. In normals, the latency of MEP did not change while the coil was moved vertically from 2 cm rostral to 2 cm caudal of C7 standard line and horizontally from the midline to 6 cm lateral. Increasing the stimulus intensity, MEP latency never got shorter when the intensity was over 180% of motor threshold. On the other hand, the MEP amplitude was highest when the stimulation was given at 4 cm lateral from the midline on the C7 standard line, but our equipment were unable to give the supramaximal stimulation. Our conclusion was therefore that the optimal stimulus point was 4 cm lateral from the midline on the C7 standard line, and the minimal latency of MEP was obtained by the stimulation with the intensity beyond 180% of motor threshold.(ABSTRACT TRUNCATED AT 250 WORDS)

We analyzed the antibodies against cerebellum in five patients with paraneoplastic cerebellar degeneration (PCD). Four patients were found to have autoantibodies against rat brain, first one to cerebral 250 kd and 110 kd and cerebellar 110 kd proteins, second one to cerebral and cerebellar 98 kd and 68 kd proteins, third one to cerebellar 58 kd protein and fourth one to 58 kd protein especially in the cytoplasm and nucleus of Purkinje cells. We isolated a cDNA clone from a human cerebellar library to identify the target antigen for fourth antibody. Homology searches revealed a similarity with the zinc finger proteins. Zinc finger proteins are considered to regulate gene expression. Therefore, degeneration of this protein by its antibody may affect the synthesis of proteins in Purkinje cell and may cause cerebellar degeneration.

As a basic study to investigate the involvement of nitric oxide in the pathogenesis of central nervous system diseases, we have established an assay system to measure nitric oxide synthase (NOS) activity by a high performance liquid chromatography monitoring conversion of arginine into citrulline. NOS activity in the rat brain was calcium-dependent and inhibited by L-nitro-arginine, a specific NOS inhibitor. The activities in the discrete brain regions were in the order cerebellum > olfactory bulb > striatum > hippocampus > cerebral cortex > midbrain > hypothalamus > pons.

A 31-year-old man had noticed slowly progressive weakness in his right upper limb girdle. On admission at age 32, fasciculation and muscle atrophy were observed in the right pectoralis major muscle. There was mild muscle weakness in the right triceps brachii muscle. The deep tendon reflexes were normal and the pathologic reflexes were absent. Sensory and autonomic nerve functions were intact. Needle EMG showed fibrillation potentials and positive sharp waves in the right pectoralis major, infraspinatus, extensor carpi radialis, extensor digitorum muscles, the bilateral sternocleidomastoideus and triceps brachii muscles. Motor nerve conduction studies revealed normal conduction velocities and distal latencies as well as no evidence of conduction block or abnormal temporal dispersion. Serum immunoelectrophoresis failed to detect an M protein. Thin-layer chromatography with immunostaining revealed that the serum contained auto antibodies which reacted with asialo-GM1, GM1 and LM1. This case must belong to "proximal lower motor neuron syndrome" proposed by Pestronk et al.

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.

A 47-year-old woman with myasthenia gravis for last 11 years was admitted because of relapsed muscle weakness, hypermenorrhea and thrombocytopenia. Physical and neurological examinations revealed diplopia, proximal muscle weakness and purpuras on the left arm and bilateral legs. Repeated hematological examinations revealed cyclic fluctuation of platelet counts which spontaneously changed from the nadir levels of 12-27 x 10(3)/microliters to the peak levels of 150-400 x 10(3)/microliters. The platelet count reached a nadir at the onset of menstruation. Platelet-associated IgG (PAIgG) was within normal level when platelet count was at an increasing phase. Survival time of autologous platelets was normal when platelet count was at an increasing phase. Megakaryocytes in the bone marrow were apparently normal at the nadir phase. The patient's serum obtained at the nadir of platelet count significantly suppressed megakaryocyte colony forming unit (Meg-CFU) formation in comparison with that after the stabilization of platelet count, suggesting that this cyclic thrombocytopenia was secondary to cyclic hypoproduction of megakaryocytes caused by a suppressive factor. On the other hand muscle weakness showed no cyclic fluctuation. Administration of 60 mg/day prednisolone stabilized the platelet count at about 280 x 10(3)/microliters, abolished hypermenorrhea and gradually improved muscle weakness. These findings suggested autoimmune mechanism in the production of a Meg-CFU-suppressive factor might be involved in the pathogenesis of thrombocytopenia.

A 24-year-old carpenter had the shakes and fever on March 13, 1990. He suffered from watery diarrhea on March 14 and 15. He left muscle weakness in his thumbs and fingers when he drove nails with a hammer on March 24. The weakness reached maximum by the 3rd day of illness. He was admitted to our hospital on day 4. Neurological examination revealed symmetrical weakness localized in the intrinsic hand muscles (MRC grade 2-4). The deep tendon reflexes were preserved. Sensation was intact except for mild disturbance of superficial sense on both plantar areas. Campylobacter jejuni was cultured from his stool. A complement fixation test indicated serologically preceding C. jejuni infection. Whereas maximum motor nerve conduction velocities were not reduced and distal latencies were not prolonged, compound muscle action potential recorded in the thenar and hypothenar muscles were remarkably reduced on day 5. Needle EMG showed neuropathic changes in four limbs. Sensory nerve conduction velocities and action potentials were normal. The weakness gradually improved in association with increased compound muscle action potentials in the thenar and hypothenar muscles. His muscle symptom fully resolved 2 months after the onset of his illness. Thin-layer chromatogram with immunostaining revealed that serum IgG from this patient reacted with GM1, GD1a, GD1b, but did not react with GM2 and GT1b. Enzyme-linked immunosorbent assay showed that anti-GM1, GD1a, GD1b antibodies titer (IgG) decreased concurrently with the clinical improvement.

A 70-year-old woman was admitted to our hospital because of rapidly progressive cerebellar ataxia. Neurological examinations showed saccadic eye movement, downbeat nystagmus, scanning speech, proximal dominant muscle weakness and severe truncal and limb ataxia. Based on these clinical features, she was suspected to have paraneoplastic cerebellar degeneration (PCD), although the malignant tumor was not detected through clinical intensive surveys. Her serum and CSF revealed to have anti-Purkinje cell antibodies immunohistochemically, and western blot analysis showed that they reacted with 58 kd band. In view of previous reports of PCD, she was strongly suspected to have gynecological cancers. The trial laparotomy found early stage fallopian tubal cancer, which had not been detected by CT scan, ultrasonogram and MRI. It is important to detect and characterize these autoantibodies found in the PCD patients for early diagnosis and treatment of underlying cancer.

Using polymerase chain reaction (PCR), a rapid and highly sensitive method was developed for the diagnosis of tuberculous meningitis. The mycobacterium tuberculosis (M. tuberculosis) genome was detected in CSF of 5 of 6 patients with clinically diagnosed tuberculous meningitis, but not present in CSF from 10 patients with other types of meningitis and 10 normal controls. Furthermore, we detected M. tuberculosis in CSF of 2 of 24 patients with clinically possible tuberculous meningitis. This is highly useful method for the rapid diagnosis of tuberculous meningitis over other conventional method.

A 44-year-old man developed numbness in his hands and feet and an unsteady gait following an upper respiratory symptoms. The symptoms progressed rapidly and reached maximum in a few days, followed by a gradual improvement over a few weeks, but his gait remained unsteady. His symptoms progressively increased over 14 years, with 11 relapses of similar episodes. Neurologic examination showed normal limb strength. Deep tendon reflexes were absent. Touch, cold, and pinprick sensation were impaired in his hands and feet. There was a profound loss of position and vibration sense in all extremities. He demonstrated an unsteady and broad-based gait. Electrophysiologic and histologic examination on the peripheral nerves revealed evidences of axonal degeneration without demyelination involving the sensory nerves.

A 47-year-old woman with mixed connective tissue disease (MCTD) was reported. She developed various neuromuscular complications including carpal tunnel syndrome, narrowing of visual field, dysesthesia on face and extremities and weakness before elevation of antibody to RNP. On the examination of biopsied specimens of muscle and peripheral nerve, myositis and demyelination were suspected, respectively. Electroencephalography showed diffuse slowing which was without clinical evidence of the central nervous system involvement and was normalized by the treatment with prednisolone. Present case seems to provide some insights into the neuromuscular complications in MCTD.

A 29-year-old man who had a taste for peanuts frequently showed abnormal behaviors. Physical examination revealed consciousness disturbance associated with flapping tremor, hypotonus in the extremities, and hypoactive deep tendon reflexes. Laboratory examination showed hyperammonemia with mild liver injury. A level of serum citrulline was 14 times higher than the upper limit of normal. A diagnosis of type 2 citrullinemia (Seheki et al) was established by the decreased argininosuccinate synthetase (ASS) in liver with low amount of ASS protein and normal kinetic properties. Diurnal fluctuation of consciousness level in this patient was not correlated with the concentration of ammonia in serum, but with that in cerebrospinal fluid. Main metabolic pathway of ammonia in brain is considered to be localized in astrocytes which have receptors for glucocorticoids. Glucocorticoids induce additional glutamine synthetase activity. Circadian rhythm of cortisol level might play a role in diurnal fluctuation of consciousness level in patients with citrullinemia.

A 26-year-old female developed mental deterioration, general convulsion, cataract and spastic gait in order since her entrance into elementary school. A diagnosis of cerebrotendinous xanthomatosis (CTX) was made because of hypercholestanolemia. At the time of admission, cataract, a mild thickening of Achilles tendons, mental deterioration, spastic paraparesis, truncal ataxia, and bradykinesia were noted. Bilateral slowing of 2 to 7 Hz was recorded in EEG, and brain CT and MRI revealed mild cerebellar atrophy. HVA and 5-HIAA levels in CSF were low. Oral administration of chenodeoxycholic acid, 300 mg per day, resulted in improvement of bradykinesia and EEG abnormality, increase of HVA and 5-HIAA levels in CSF, and decrease of serum cholesterol level in two weeks. Bradykinesia observed in the present case is a rare clinical finding of CTX, and the improvement of bradykinesia soon after the treatment with chenodeoxycholic acid has not been reported yet. This case is important for elucidating the mechanism of neurological disorders in CTX.

A 34-year-old man had noted progressive weakness in his right hand. On admission at age 39, cranial nerves were not involved. Fasciculations were observed in his upper limb girdles. Neurological examination revealed severe wasting and weakness of arms and the right hand, whereas mild in the left hand. The deep tendon reflexes were absent in the upper extremities, but normal in the lower extremities. No sensory disturbances were observed. Motor and sensory nerve conduction velocities were normal, and multifocal conduction block was not observed. EMG showed neuropathic changes in all 4 limbs and sternocleidomastoideus muscles. Serum immunoelectrophoresis failed to detect an M protein. High-performance thin-layer chromatography with immunostaining revealed that his serum IgM reacted with GM1, but not reacted with GM2, GD1a, GD1b, and asialo-GM1.

We reported a case of Guillain-Barré syndrome with autoantibodies against gangliosides GM1 and GD1b, which has not been reported yet. A 25-year-old man was admitted with a 7-day history of acute progressive weakness in the extremities. Two weeks before admission he had suffered from an episode of watery diarrhea. Neurological examination revealed areflexia and tetraparesis without any sensory impairment. Respiration and cranial nerves were not involved. The CSF protein level was 157 mg/dl with normal cellularity on the 14th illness day. The maximum M potential amplitude was 0.1 mV, which was 2% of the lower limit of normal, whereas motor and sensory nerve conduction velocities were normal on day 22. Repeated electrophysiological studies suggested that the predominant process was axonal degeneration. Although he received plasmapheresis at the acute phase, four months after onset he continued to have distal dominant limb weakness with wasting. High-performance thin-layer chromatography with immunostaining revealed that his serum IgG reacted with GM1, GD1b, and asialo-GM1. Enzyme-linked immunosorbent assay showed that anti-glycolipids antibodies titers decreased, concurrent with clinical course. Immunoabsorption study demonstrated that antibodies with anti-GM1 activity were absorbed with liposomes containing purified GD1b, indicating that autoantibodies bind to the galactosyl (beta 1-3) N-acetylgalactosaminyl moiety which is shared by GM1, GD1b, and asialo-GM1. There have been several reports of lower motor neuron diseases with monoclonal IgM antibody to GM1, GD1b, and asialo-GM1. This case suggested that the transient rise in these anti-carbohydrate antibodies may be involved in the pathogenesis of acute axonal degeneration of motor nerve as well as paraproteinemic motor neuron diseases.

To clarify physiological aspects of Machado-Joseph disease (MJD), we studied auditory brainstem response (ABR) and somatosensory evoked potential (SEP) in 17 clinically diagnosed patients with MJD aged 32-64 in Japanese families. ABR was recorded in 13 patients. In 8 patients, ABR were abnormal. In 5 patients, the latency of I wave was normal, but other waves could not be evoked. In the other 3 patients, I-III interpeak latency was prolonged. SEP was recorded in 15 patients. In SEP of median nerve, 11 patients had abnormal findings, and SEP of posterior tibial nerve revealed abnormal findings in all 15 patients. In all patients, responses from Erb's point and popliteal fossa were normal in latency, but other peaks were low in amplitude or absent, and the latency and central conduction time (CCT) were prolonged. The result of ABR indicated the involvement of the brainstem auditory pathways in MJD, and the result of SEP suggested that somatosensory pathways, particularly central pathways, would be involved in the disease process. ABR and SEP can be potential diagnostic methods for detection of subclinical abnormality in MJD patients.

A case of rigid spine syndrome associated with rimmed vacuoles in muscle biopsy is reported. A 36-year-old man was admitted to our hospital because of gait disturbance and limited mortality of the spine. His family was free from any neuromuscular disorders. He was born in normal pregnancy and delivery. His physical development was normal. At age 7, he was unable to run fast. At age 36, he had right hemiparesis and dysarthria. He was diagnosed as cerebral infarction of the left basal ganglia by brain CT. Neurological examination revealed moderate proximal dominant muscular atrophy and weakness. His spine was straight, showing loss of physiological cervical and lumbar lordosis. The neck flexion was limited but the extension was full. And he had contracture of bilateral ankle joint. Laboratory findings were all normal. The electrocardiogram showed negative T wave in V4, V5 and QT interval elongation. The echocardiogram showed diffuse decrease of ventricular wall motion. Respiratory function test revealed decrease of vital capacity. Arterial blood gases on room air showed that the PaO2 and PaCO2 were 70 mmHg and 49 mmHg, respectively. The findings of electromyogram were compatible with myopathic change. Biopsy specimen of the biceps brachii muscle showed marked variation in fiber size, type 1 fiber predominancy and atrophy, and type 2B fiber deficiency. Numerous rimmed vaculoes were found in the same muscle. Four cases of the rigid spine syndrome with rimmed vacuoles have been described. Among them, three patients died in young ages and two suffered from constrictive respiratory failure. In rigid spine syndrome with rimmed vacuole formation, the cardiac and respiratory problems must be taken account intensively.

A 64-year-old male patient of amyotrophic lateral sclerosis (ALS) with frozen gait, axial rigidity and supranuclear upper gaze palsy was reported. We have followed this patient more than four years. He was well until November 1982, when he noticed weakness of left arm. In March 1983, he noticed hypogeusia and in July, he developed dysarthria and frozen gait. On admission, he was alert and oriented. Neurological examination revealed dysarthria, dysphagia and muscular weakness and atrophy in bilateral upper extremities, dominantly in left side. He showed remarkable frozen gait, retropulsion and could not walk. Brain CT showed mild dilatation of the third ventricle. In August 1988, he showed tongue atrophy, and weakness and atrophy of the extremities progressed during these four years. He also showed axial rigidity and frozen gait. Brain CT showed severe third ventricular dilatation and atrophy of tegmentum of the midbrain and cerebellum that were compatible with progressive supranuclear palsy (PSP). Six months later, he developed upper gaze palsy. From these findings, we concluded that this patient had a quite unique clinical features of both ALS and PSP.

A case of juvenile Sandhoff disease with Gowers' local panatrophy was reported. A mentally retarded 39 year-old man had been noticed to have localized skin atrophy on the right leg since 7 years of age, and was also aware of the atrophy of his right leg since his junior high school age. His parents were first cousins. He had multiple localized atrophic skin lesions on the right side of his nape, the right side of lower back, and the right thigh. These skin lesions did not show any signs of sclerosis. Although he was mentally retarded (IQ=44), the neurological examination was normal including funduscopy. Histological examination of the atrophic skin le led that the epidermal basal layer was hyperpigmented and that the dermis and subcutaneous fatty tissue were extremely atrophic. The nerves meandered across the dermis and subcutaneous tissue, and had membranous lipid storage cytosomes in the axon and Schwann cells. Biochemical studies revealed marked deficiency fo leukocyte hexosaminidase activity. Total hexosaminidase activity was decreased to 18% of normal controls, and hexosaminidase B activity was completely deficient. Other lysosomal enzymes in leukocytes were normal in activities. Both parent showed intermediate levels of leukocyte hexosaminidase A and B activities. Electron microscopy analysis of a rectal biopsy specimen showed neuronal accumulation of dense osmophilic deposits, as well as membranous cytoplasmic bodies in Meissner's plexus. On the basis of the patient's age at onset and the above findings, the patient was diagnosed to have juvenile Sandhoff disease. The local panatrophy observed in the present case could be the result of the accumulation of GM2-ganglioside in autonomic nervous system, and may well be a new clinical feature of GM2-gangliosidosis.

We described a case of late-infantile form of galactosialidosis. This male patient was a product of normal pregnancy. His parents were first cousins. He first sat at eight months, walked and talked at two years of age. His gait gradually became unsteady and he was diagnosed as spastic paraparesis at the age of five years. Abnormally slow learning was first pointed out at seven years of age. At the age of nine years, we evaluated him in detail at our university hospital. Physical examination revealed a short stature for his age, slightly coarse face, short neck, funnel chest, genu, pes and hallucis valgus. Corneal clouding, hernia and angiokeratoma were not found. Neurological examination showed mental retardation, bilateral optic atrophy without cherry-red spots, and spastic and slightly ataxic gait. Slight muscular atrophy with weakness was also seen in the extremities, more remarkable in the lower limbs. Deep tendon reflexes were hyperactive with bilateral ankle clonus and no extensor planter response. Routine examination of blood, urine and cerebrospinal fluid were normal except for approximately 10% lymphocytes containing cytoplasmic vacuoles. X-ray films of the backbone exhibited vertebral plana with anterior breaking at the second lumbar vertebra level. The electroencephalography showed the multiple spike and slow wave complexes. Brain CT depicted the atrophy of cerebellum. The activities of sialidase and beta-galactosidase were markedly reduced in white blood cells and cultured skin fibroblasts in this patient. His urinary excretion of sialyloligosaccharides increased.(ABSTRACT TRUNCATED AT 250 WORDS)

An 8-year-old boy developed drowsy state, ataxia and right hemiparesis. Computed tomography and magnetic resonance imaging demonstrated ischemic lesions on bilateral cerebellar hemispheres, right thalamus and left pontine base. Angiography revealed occlusion of the left vertebral artery at the level of the second cervical spine and segmental narrowing of right vertebral artery from the level of the second to fifth cervical spine. Present case and previously reported cases suggest that vertebral artery could be stretched at the level of the second cervical spine and the intima may be injured to produce atherosclerosis.

Only a few cases of carcinomatous neuropathy with tonic pupils have been reported. In the present paper we described a 53-year-old woman with subacute sensory neuropathy who had presented with bilateral tonic pupils. She noticed numbness over the medial aspect of the left thigh in March, 1988, and then developed dysesthesia over the left forearm and hand, mild weakness of left upper and lower extremities and urinary disturbance. Neurological symptoms were subacutely progressive and she was bed-ridden in May. She was admitted to our hospital in June, 1988. On examination, she had body-weight loss of 6 kg during the last six months and general status was otherwise unremarkable. She had anisocoria; the left pupil was larger in daylight than the right, while smaller in dim light. The left pupil scarcely reacted to light, but promptly constricted to near vision. The right pupil constricted normally to light and near vision. An instillation of 0.0625% pilocarpine solution showed supersensitive response of both pupils. An instillation of 1.25% epinephrine solution demonstrated mild dilation of both pupils. Thus, it was conceivable that she had postganglionic ciliary nerve damage characteristic of tonic pupil as well as the lesion of sympathetic nerve innervating pupillary dilator. She had severe sensory ataxia and pseudoathetosis of the hands. Weakness was mild to moderate in extremities. Almost all deep tendon reflexes were absent. All modalities of sensation, particularly on deep sense, were severely involved with sea-level-type distribution below Th7, and over C2 to C3 regions on the left side.(ABSTRACT TRUNCATED AT 250 WORDS)

A 19-year-old man with chronic progressive external ophthalmoplegia with deleted mitochondrial DNA was reported. Neurological examination revealed bilateral external ophthalmoplegia, hearing loss of sensorineural type, short stature, mental retardation, muscle atrophy and weakness in the proximal muscles. Lactate and pyruvate levels were elevated in both serum and cerebrospinal fluid (CSF). Protein concentration was slightly increased in CSF. Electromyogram showed myopathic changes on all the muscles examined. Ragged-red fibers were found in biopsied rectus femoris muscle, stained with modified Gomori trichrome. Scattered cytochrome c oxidase deficient fibers were encountered. The computed tomography of the brain showed mild cerebral and cerebellar atrophy without any abnormal calcification or hypo-lucency. Southern blot analysis of the mitochondrial DNA (mtDNA) extracted from the patient's muscle revealed mixed population of mtDNA, consisting of the normal one and partially deleted one. The size of the deletion was about 4.5-kilobase. The region included the sequences coding for at least four subunits of Complex I, one subunit of Complex IV, two subunits of Complex V and five tRNAs. There may be a "hot area" on the mitochondrial genome that is more prone to be deleted than other regions of mtDNA. Southern blot analysis is usefull for the diagnosis of KSS or CPEO.

Acute autonomic and sensory neuropathy (AASN), one subtype of acute pandysautonomia, in which dorsal root ganglia and autonomic ganglia are involved is uncommon. Little is so far known on central nervous system involvement in AASN. In the present paper we described a rare case of AASN associated with the central nervous system manifestations such as galactorrhea-amenorrhea syndrome and intractable anorexia. A 30-year-old woman rapidly developed burning pain and numbness in her arms and legs as well as orthostatic syncope. She had severe anorexia and no no menstruation from onset. On physical examination, she was emaciated. There was marked orthostatic hypotension with tachycardia. Skin was dry. Moderate galactorrhea was detected. Neurological examination showed prominent paresthesia and dullness of superficial sensation, predominantly to pinprick and thermal stimuli, segmentally over the neck, occipital scalp, and extremities. Deep sensation was intact. She had no weakness or ataxia. Deep tendon reflexes were almost normal. NCV and SEP were normal, while EEG was abnormal. Sural nerve biopsy demonstrated axonal degeneration with the loss of myelinated, predominantly in small-caliber fibers, and unmyelinated fibers. The levels of HVA and MHPG in CSF were decreased. The autonomic nervous function tests revealed postganglionic dysfunction. alpha-adrenergic system was predominantly impaired, while beta-adrenergic system was relatively preserved. The endocrinological studies demonstrated mild or moderate elevation of PRL basal value and hyper-response of PRL and LH for TRH and LH-RH loading test, which suggested disorder of the hypothalamo-hypophysial system. Cranial MRI showed moderate dilatation of the 3rd ventricle.(ABSTRACT TRUNCATED AT 250 WORDS)