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Respiratory distress and selective muscle involvement in central core disease: report of a case.

A 10-year-old girl who had suffered life-threatening respiratory distress in late infancy and mild muscle weakness in the extremities was found to have central core disease (CCD). Her developmental milestones were markedly delayed and she became ambulant at the age of 6 years. On physical examination, she had a high-arched palate, nasal tone vocalization, a positive Gowers' sign, waddling gait, and decreased deep tendon reflexes. On magnetic resonance imaging (MRI) examination, the thigh, calf, arm, and paravertebral muscles were selectively involved, especially in the paravertebral, biceps brachii, gluteus maximus and medius, vastus lateralis and medialis, sartorius, adductor magnus, tibialis anterior, soleus, lateral head of gastrocnemius, and peroneus longus muscles. Two biopsy specimens from the left biceps brachii and rectus femoris muscles showed the common histochemical findings of CCD, including type 1 fiber atrophy and predominance, and core structures. Proliferation of adipose tissue was only present in the biceps brachii muscle. The patient improved clinically in muscle strength after three year follow-up. Muscle MRI may provide a non-invasive evaluation of gross muscle involvement in CCD.

Arm↗

Residual electroneurographic modifications in subjects with n-hexane induced polyneuropathy: a follow-up study.

A group of 90 subjects affected by n-hexane polyneuropathy were studied again after at least one year from cessation of occupational exposure to the neurotoxic agent. Neurological examinations, conduction velocities and distal latencies in sensory (sural and median) and motor (peroneal and ulnar) nerves were evaluated in order to study the evolution of the disease. The percentage of subjects with abnormal leg deep tendon reflexes, leg cutaneous sensitivity or vibration sensation was statistically higher in subjects who had ceased n-hexane exposure for less than 10 years than in the other subjects; however, no differences between these two groups were found for electrophysiological parameters. A complete recovery of motor nerve velocities was found. On the contrary, sensory nerve velocities, sensory nerve distal latencies, as well as ulnar nerve latency, were statistically different from controls, even if they were also statistically different from the values which had been recorded at the time of diagnosis. We conclude that in n-hexane polyneuropathy motor nerve components improve, while the sensory nerves are more seriously affected and do not completely recover, even after more than 10 years without exposure to the toxic agent.

Adult↗

[Idiopathic segmental anhidrosis].

We described two cases of idiopathic segmental anhidrosis. Case 1 was a 47-year-old man, who noticed anhidrosis on the right side of face and chest during body heating. Case 2 was a 54-year-old woman, who complained of anhidrosis on the left side of face and upper chest during exercise. Both cases had neither somatic neurological deficit nor autonomic failure except for anhidrosis pupil size and deep tendon reflexes were normal. Reflex sweating to pilocarpine was exaggerated in the anhidrotic areas in both cases, suggesting lesions in the preganglionic sudomotor nerves. Abnormal laboratory finding was elevated serum rheumatoid factor level in only case 2. Segmental anhidrosis was static for 1.5 year and one year, respectively. The previous literature contains similar four cases. Idiopathic segmental anhidrosis may be an abortive form of Ross' syndrome (tonic pupil, hyporeflexia and segmental anhidrosis).

Female↗

[Autosomal recessive early-onset parkinsonism with diurnal fluctuation (AR-EPDF)--clinical characteristics].

Among heterogeneous diseases manifested by parkinsonism beginning early in life, there is a disease presenting with marked diurnal fluctuation of symptoms, called autosomal recessive early-onset parkinsonism with diurnal fluctuation (AR-EPDF). To identify the characteristics of this condition as a disease entity, we examined the clinical manifestations of AR-EPDF patients (Group I, n = 42) in comparison with those of early-onset parkinsonism patients without diurnal fluctuation (Group II, n = 34). Family history suggesting autosomal recessive inheritance was noted in 85.7% of Group I patients and 17.6% of Group II. The male-to-female ratio was 1: 1.8 in Group I, and 1:0.89 in Group II. Age at onset showed a standard distribution with an average of 25.6 years (SD: +/- 7.7) in Group I and an average of 32.7 with an increasing pattern toward 40 years in Group II. The initial symptom was dystonic gait disturbance in 42.9% of Group I and 5.9% of Group II, parkinsonian gait in 19.9% of Group I and 2.9% of Group II, and tremor in 28.6% of Group I and 41.2% of Group II. The main clinical feature was parkinsonism in both groups. Diurnal fluctuation of parkinsonism was remarkable in all but one (97.6%) of Group I, while it was not observed in Group II. Dystonic postures were noted in 79.4% of Group I and in 37.1% of Group II; hyperactive tendon reflexes in 74.3% of Group I patients and in 20% of Group II. Autonomic symptoms were mild in both groups. None of the Group I patients had dementia while two of Group II did. Levodopa was markedly effective in both groups. Dopa-induced dyskinesia was observed in 96.8% of Group I and in 61.8% of Group II. As for progression of the disease, the Hoehn-Yahr stage of patients on medication was evaluated as 2.2 +/- 0.7 (mean +/- SD) in Group I and 3.1 +/- 1.1 in Group II for a period of 10 to 20 years of onset, 2.5 +/- 0.8 in Group I and 3.3 +/- 0.5 in Group II for 20 to 30 years, and 3.2 +/- 0.9 in Group I and 4.3 +/- 0.6 in Group II after 30 years. There were significant differences between the two groups in the frequency of positive family history, in the mean and distribution of age at onset, in the incidence of dystonic gait as the initial symptom, and in the incidences and medians of the variables including dystonia, hyperreflexia and dopa-induced dyskinesia, as well as in the progression of the disease. Thus, we have successfully characterized the clinical features of AR-EPDF and demonstrated that diurnal fluctuation is a cardinal symptom of this disease. Reported pathologic studies on AR-EPDF showed the nigral lesion characterized by non-Lewy body type degeneration and the occurrence of melanin-poor neurons. These pathologic findings as well as the clinical manifestations differentiate AR-EPDF from Parkinson's disease and from autosomal-dominant familial parkinsonism. Low melanin-content of the nigral neurons is also a striking feature of hereditary progressive dystonia with diurnal fluctuation, in which unlike AR-EPDF there is no neuronal loss in the substantia nigra.

Adult↗

[Dissociated adduction nystagmus in a patient with hereditary spinocerebellar degeneration].

A 66-year-old woman with a 10 years' history of autosomal dominant hereditary spinocerebellar degeneration developed an atypical nystagmus. Neurological examination revealed cerebellar ataxia, absent tendon reflex, supranuclear upward gaze palsy, saccadic pursuit and nystagmus. Nystagmus was a dissociated type and appeared only in lateral gaze and the amplitude was prominent in adducting eyes. Brain MRI showed cerebellar hemispheric and vermal atrophy, and pontine atrophy. Neuro-otological examination revealed normal caloric test, but impaired optokinetic nystagmus and visual suppression test. The dissociated adduction nystagmus of this case may correspond to gaze evoked nystagmus and latent hypofunction of the abducens motor neurons.

Aged↗

[A late onset familial amyloidotic polyneuropathy (FAP) with a novel variant transthyretin characterized by a basic-for-acidic amino acid substitution (Glu61-->Lys)].

A 64-year-old man has suffered from intractable diarrhea since January 1990. He noticed numbness and weakness in the distal portion of four extremities in the following several months. His symptoms were gradually progressive. In June 1992, neurological examination revealed mild muscular atrophy and weakness in the proximal and distal portions of four extremities. There were paresthesia and severe impairment of superficial sensations in the lower limbs, lower half of the trunk and upper limbs. All deep tendon reflexes were reduced or absent. Autonomic dysfunctions such as orthostatic hypotension, impotence and diarrhea were evident. On sural nerve biopsy, myelinated fibers showing axonal degeneration were predominantly seen, and densities of both myelinated and unmyelinated fibers were markedly decreased. No amyloid deposits were found in the endoneurium. Amyloid deposition was identified in the gastric mucosa by Congo red staining and immunostaining with anti-transthyretin (TTR) antibody. Edman degradation showed one amino acid substitution of Lys for Glu at position 61 in the TTR-peptides from the serum. Direct DNA sequencing revealed a new point mutation in the 61st codon of TTR gene. The same point mutation of TTR gene was identified in the DNAs from his 67-year-old brother and 63-year-old sister and one of the paternal cousins, a 64-year-old woman, although their clinical symptoms and signs were negative. Clinical features such as late onset of the symptoms and signs and presence of carriers in their sixties in this family are unique and atypical as compared with those of more frequent Val30-->Met FAP families. A variant TTR, characterized by a Glu61-->Lys substitution (a basic-for-acidic amino acid substitution) found in this family, has not been reported in the literature. In the case of the examination of the patients with autonomic and sensory symptoms and signs of unknown etiology, amyloidotic polyneuropathies, including FAP even in the absence of the family history, should be differentiated. When FAP is highly suspected, the combination of family study and DNA analysis of a possible variant TTR is indispensable for the establishment of the diagnosis.

Age of Onset↗

[A 91-year-old man with a stroke, hypertension, and renal failure].

We report a 91-year-old man who had a stroke and died of renal failure. He had been treated for hypertension since 20 years before the onset of the present illness. In addition, he was operated on a gastric cancer 17 years previously. Otherwise he was doing well until May 29, 1991 (when he was 87-year-old) when he had sudden onset of dysarthria and right facial weakness. He was admitted to our hospital. On admission, general physical examination was unremarkable, and neurologic examination revealed a mentally sound man with slight dysarthria, right facial weakness, orolingual dyskinesia, and dysequilibrium in which he showed difficulty in tandem gait; however, no cerebellar ataxia was noted. A cranial CT scan revealed leukoaraiosis with multiple low density areas in the cerebral white matter. His BUN was 37 mg/dl and Cr 2.2 mg/dl. His neurologic symptoms cleared within the next few weeks and he was discharged with ticlopidine 100 mg q.d.. He had been doing well after the discharge except for gradual worsening of his renal function; his BUN was 65 mg/dl and Cr 3.27 mg/dl in April of 1994. On March 10, 1995, he fell down and hit his back; he became unable to walk because of pain, and he was admitted again on March 16, 1995. On admission, his blood pressure was 170/80 mmHg. There was an 1 + pitting pretibial edema; otherwise general physical examination was unremarkable. Neurologic examination revealed an alert and oriented man, however, Hasegawa's dementia scale was 23/30. Higher cerebral functions as well as cranial nerves were intact. He showed some unsteadiness of gait, however, no motor weakness or ataxia was noted. Deep tendon reflexes were diminished, but Chaddock sign was positive bilaterally. Vibration was diminished in the feet, however, pain and touch sensations were intact. Laboratory examination revealed a compression fracture of the twelfth thoracic vertebra. Blood count and chemistries were as follows; Hb 7.6 g/dl, Hct 23.3%, TP 6.0 g/dl, Alb 3.6 g/dl, BUN 87 mg/dl, Cr 4.53 mg/dl, T-Chol 174 mg/dl, HDL-Chol 49 mg/dl, Glu 156 mg/dl, Na 142 mEq/L, K 5.4 mEq/L, Cl 115 mEq/L. A urine specimen contained 1 + protein and 1 + glucose, and the sediments contained hyaline casts. A cranial CT scan was essentially same as that taken four years ago. His hospital course was complicated with pneumonia, congestive heart failure, and progressive renal failure. He was treated with intravenous fluid, chemotherapy, and other supportive measures, however, he expired from respiratory failure on April 30, 1995. He was discussed in a neurologic CPC, and the chief discussant arrived at the conclusion that the patient had Binswanger's disease in the brain, benign nephrosclerosis from arteriolosclerosis due to hypertension, congestive heart failure, and pneumonia. Opinions were divided regarding the question as to whether or not this patient had Binswanger's disease. Although his cranial CT scan revealed leukoaraiosis, his dementia and gait disturbance was only mild until his fall on March, 1995. Clinical features did not conform to those of Binswanger's disease. Postmortem examination of the right hemisphere revealed wide spread atherosclerosis and arteriolosclerosis. The kidney showed benign nephrosclerosis due to arteriolosclerosis. Sclerotic changes were also seen in the coronary arteries and the left middle cerebral artery with 70% stenosis. Myelin stain showed diffuse myelin pallor of the cerebral white matters with scattered small infarcts. Arterioles in the white matter showed arteriolosclerosis. Small infarcts were also seen in the putamen and in the thalamus. This patient appeared to have had circulatory disturbance of the white matter which is the basic abnormality causing Binswanger's disease. However, white matter changes in this patient were not quite severe enough to make a pathologic diagnosis of Binswanger's disease.

Aged↗

[Critical illness polyneuropathy].

Critical illness polyneuropathy (CIP) is a recently identified entity for which reliable data is unavailable and of which the prevalence is unknown. Although the percentage of patients suffering CIP is low, more cases, including subclinical ones, are likely to be detected through the use of electroneurography. Any aged patient may be affected by CIP. It usually occurs a long stay in the intensive care unit (ICU) and is generally associated with sepsis, severe trauma and so-called multiple organ failure. The main clinical sign is distal weakness in the lower extremeties, although finding one or more of the following signs is not uncommon: quadriparesis, quadriplegia, difficult weaning, loss of osteo-tendon reflexes and muscle atrophy. The pathophysiology of CIP is unknown, although such mechanisms as cell dehydration, increased proteolysis and the activation of certain cytokins have been suggested. Before diagnosing CIP, other neuromuscle diseases and several recently described toxic myopathies must be ruled out. Electroneuromyographic study of axonal lesions may be of great utility, whereas analyses have low specificity. Histologic examination, when possible, allows axonal lesions (but never demyelinization) to be observed along with the non specific changes of myopathy. Although no specific treatment is available, the long-term prognosis is good if the underlying disease that gave rise to ICU admission is controlled, and if rehabilitation therapy is started early.

Age Factors↗

[Neuroradiological findings of sporadic olivopontocerebellar atrophy with marked laterality and degenerative changes in the corticopontine tract].

We report a case of sporadic olivopontocerebellar atrophy (OPCA) with marked laterality of cerebellar atrophy and degenerative changes in the corticopontine tract. A 35-year-old man was admitted to our hospital for evaluation of titubation, gait disturbance, dysarthria, and urinary and fecal incontinence. Neurological examination showed a wide based gait, slurred speech, truncal ataxia, slightly saccadic ocular movement, and finger-to-nose incoordination, greater on the right than the left. Deep tendon reflexes were hyperactive and preserved with the right side greater than the left. Bilateral Babinski signs were present, and the patient had neurogenic bladder without orthostatic hypotension. Cranial MRI showed atrophy of the cerebellum with right dominance and of the pons. On T2- and PD-weighted images, high-intensity areas were detected at the left internal capsule, crus cerebri and ventral pons. These findings were compatible with the right dominance of the clinical symptoms. The high intensity area detected at the posterior internal capsule was more extensive than that seen in patients with motor neuron disease. This finding may coincide with the degenerative changes in the corticopontine tract. Moreover, 99mTc-HMPAO-SPECT showed the crossed cerebello-cerebral diaschisis (CCCD) pattern, which indicates the decreased CBF in the right cerebellar hemisphere and the left frontal lobe. These findings may reflect degenerative changes in the corticopontine tract in OPCA.

Adult↗

[Status and selenium intake as related to the thyroid gland in the population of Znojmo].

In a group of 360 people aged 6-65 years of both sexes from the Znojmo area the author investigated the selenium status and intake by serum analyses (246 cases), urine analyses (356 cases) and hair analyses (28 analyses in middle-aged men). By correlation analysis the author investigated the relationship of selenium and metabolic and peripheral thyroid parameters. From the low selenium concentration in all investigated materials (42 micrograms Se/l serum, 8.2 micrograms Se/urine, 7.2 micrograms Se/g creatinine in urine and 0.23 microgram Se/g hair) the author concludes that there is selenium deficiency in the investigated population, the primary cause being a low dietary selenium intake (average adult intake 17 to 25 micrograms Se/day). From significant, though loose correlations between selenium concentrations and thyroid parameters (size, texture, number of nodes), serum concentrations of thyroid hormones (and their ratios resp.), as well peripheral parameters of hormone actions (Achilles tendon reflex, pulse rate or anthropometric variables) the conclusion is drawn that the selenium deficiency is so marked that it interferes in the investigated population with the regulation of the organism by thyroid hormones. This effect may be caused by changes of hormone formation in the thyroid gland due to the concentration of Se-dependent peroxidase, but in particular changes of their metabolism in the circulation and periphery, as selenium participates in the active centre of deiodase I in the formation of metabolically active triiodothyronine, and selenium deficiency has an impact also on deiodase II activity in some specialized tissues.

Adolescent↗

[Fulminant cerebello-brainstem encephalitis with polyradiculitis following probable Epstein-Barr virus infection].

Epstein-Barr virus (EBV) infection is occasionally accompanied by acute neurological impairment. The pathogenesis of neurological manifestations with EBV infection consists of primary inflammations of EBV infection, and secondary immunologic reactions. However, their clinical course and prognosis are usually favorable. Here we report a patient with fulminant neurological involvement in association with EBV infection. The patient was a 44-year-old man. One morning he developed ataxic gait and speech following flu-like symptoms. He noticed double vision in the afternoon. He had disturbance of consciousness, bilateral ptosis with mydriasis, opthalmoplegia, facial diplegia, bulbar palsy, and weakness of muscles in extremities and respiratory system on the next day. He required mechanical ventilatory support for a month. His symptoms began to improve gradually two weeks after the onset. Two month later, neurological examinations disclosed severe cerebellar ataxia of the four extremities and ocular movement, cerebellar speech, and moderate weakness in his limbs. Moderate cerebellar ataxia and diminished deep tendon reflexes remained for 8-months. Although he had no physical manifestations of infectious mononucleosis, DNA of EBV was identified in the cerebrospinal fluid (CSF) by the polymerase chain reaction method. From these results, we diagnosed his condition as a cerebello brainstem encephalitis with polyradiculitis associated with EBV infection. The cell counts and protein content of CSF gradually normalized in the early stage of his illness, but CSF protein increased again, and had the peak of 275 mg/dl in about one month. In spite of normalized CSF cell counts, his neurological symptoms persisted. CT scan and MRI studies of the brain and the spinal cord were repeated, but demonstrated no significant abnormalities. Clinical course and CSF findings revealed that his fulminant neurological symptoms were most likely produced by the secondary immunologic reactions following the primary inflammations by EBV infection.

Adult↗

[A case of Creutzfeldt-Jakob disease (CJD) started with monoparesis of the left arm].

A 72-year-old man developed a sudden weakness in his left hand on October 5, 1991. He was admitted two weeks thereafter. Physical examination revealed minimal weakness, and clumsiness of the fingers on his left hand. Exaggerated tendon reflexes and spasticity were also noted only on his left upper limb. He had neither dementia nor psychiatric symptoms. Subsequently he developed weakness in his left leg on November 17. Within 12 days he developed left facial weakness, and myoclonic movements on the left side. By December 2, he developed spastic tetraparesis with bilateral facial palsy, and generalized myoclonic jerks. A few days after that he started to show decorticate posture. From December 16, his mental status deteriorated rapidly, and he became mute, and uncooperative within a week. His clinical course can be summarized as stepwise progression similar to a cerebrovascular accident. Electroencephalography was normal on admission, but periodic synchronous discharge developed in January 1992. Brain CT that showed only mild brain atrophy at first was considered to be compatible with his age, changed to have severe brain atrophy in March 1992. He died of pneumonia on May 24, 1992 after eight months of progressive clinical course. Autopsy was done. The brain weighed 930 grams. Macroscopically there was prominent cortical atrophy. Microscopic examination revealed severe spongy state throughout the cerebral cortex. Typical spongiform changes were confined to the hippocampus. The cerebral white matter appeared to be normal. In the cerebellar cortex, the granular cell layer disappeared and Purkinje's cells were reduced in number. Kuru plaques were not seen. The cerebellar white matter, dentate nucleus, and brainstem seemed to be normal. The spinal cord was not examined. There were no pathological changes to indicate cerebrovascular accident, except for a lacuna in the right basal ganglion and a small angionecrosis in the pons. Western blotting test using Anti-APC (amyloid plaque core) antibody was positive. Neuropathological changes of the present case were consistent with those of CJD. However, the sudden onset of monoparesis without dementia or ataxia is rare as the initial symptom of this disease. The subsequent clinical course with stepwise progression of hemiplegia, which was mimicking a progressive stroke, was also rare for CJD. In comparison to typical case of CJD, this case had a different clinical onset as acute monoparesis. We can find such cases of CJD presenting as stroke in 5.6% in the previous English literatures.

Aged↗

[Two siblings with sarcoidosis diagnosed by younger sister's central nervous symptoms].

We report two siblings with sarcoidosis; the younger sister had symptoms of central nervous system, and both sisters had subcutaneous mass lesions in the gluteal region. Case 1. A 30-year-old woman presented with two episodes of right leg paresis. On admission, neurological examination revealed right leg weakness, spasticity of both legs, increased deep tendon reflexes in all extremities, urinary disturbance and hearing loss of the right ear, but she had no meningeal signs. Serological studies were normal including angiotensin converting enzyme. Cerebrospinal fluid revealed elevated protein to 340 mg/dl, mild pleocytosis, decreased glucose. CSF culture was negative, and cytology showed no malignant cells. Enhanced MR imaging showed diffuse leptomeningeal enhancement in both the brain around basal meninges and the whole spinal cord. Case 2. A 34-year-old woman (the elder sister of Case 1) presented with visual disturbance. She had been diagnosed to have bilateral iritis at Hiroshima Red Cross Hospital before visiting our hospital. Neurological examination and serological studies were normal. In both cases, left gluteal subcutaneous mass was detected and its biopsy revealed characteristic sarcoid nodules and confirmed the diagnosis of sarcoidosis. A tendency of familial occurrence and positive associations of the specific HLA antigens in sarcoidosis have been reported. Though the diagnosis of neurosarcoidosis has been difficult without extraneurological signs, sarcoidosis should be considered as a differential diagnosis in all the patients with myelopathy, and enhanced MRI and measure of CSF angiotensin converting enzyme seem to be useful for diagnosis and evaluation of drug effect during the course of steroid therapy.

Adult↗

[A 36-year-old woman with acute onset left hemiplegia and anosognosia].

We report a 36-year-old woman with right hemiplegia, anosognosia, and rapidly deteriorating course. She was well until the end of January, 1995 when she had an onset of fever, sputum, and cough. A 5 x 5 tumor was found in her left lower lobe. She was admitted to the Pulmonary Medicine on May 24, 1995 when she was 36-year-old. General physical examination was unremarkable. Bone scintigraphy revealed increased uptake in the skull, sternum, right scapula, vertebrae, right femur, and in ribs. Cranial CT scan revealed a large mass lesion in the right frontal subcortical region with central low density and peripheral high density areas, and small low density lesions in the right thalamic area and in the right posterior frontal region; ring enhancement was observed in the latter two lesions. On the second day of admission, she noted left-sided weakness which improved by corticosteroid treatment. On June 17, there was a sudden onset of left hemiparesis and a neurologic consultation was asked. Upon neurologic examination, she appeared somnolent but could understand verbal commands. She showed constructional apraxia, neglect of the left hemisphere, and anosognosia. Cranial nerves were unremarkable. Motor-wise, she showed flaccid left hemiplegia. Deep tendon reflexes were exaggerated on the left and the plantar response was extensor bilaterally. Nuchal stiffness was noted. Her cranial CT scan on June 17 revealed enlargement of the right frontal mass lesion. The subsequent course was complicated by DIC and progressive worsening of her consciousness. On June 18, she was comatose and pupillary light reflex was lost. She developed Cheyne-Stokes respiration and expired on that evening. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that the patient had a primary adenocarcinoma in the lung with multiple metastases including the brain. The fulminant terminal course was ascribed to hemorrhage within the tumor and subsequent central type of transtentorial herniation. Opinions were divided regarding the cause of hemorrhage; some participants thought hemorrhage was caused by DIC. Post-mortem examination revealed an adenocarcinoma arising at the S6 segment of the left lung with multiple organ metastases. In the brain, a huge hemorrhagic metastasis was found in the right frontal lobe and a non-hemorrhagic metastasis in the right thalamic region. Probably, the size of the metastases influenced the occurrence of hemorrhage. The direct cause of the death was transtentorial herniation.

Adenocarcinoma↗

[Guillain-Barré syndrome: clinical features, immune mechanisms, and therapies].

Guillain-Barré syndrome (GBS) is an acute, inflammatory, demyelinating disease of the peripheral motor nerves and nerve roots. The cause of GBS is unknown; it is though to be immune-mediated. The following seven clinical features usually distinguish this syndrome: 1) the neurological symptoms are preceded 1 to 3 weeks by an antecedent event. 2) symptoms and signs progress rapidly for several days up to 2 weeks, followed by a period of stability before gradual improvement to normal function after several months, 3) there is symmetric weakness, 4) there is a loss of tendon reflexes, 5) cerebrospinal fluid protein is elevated, 6) conduction velocity in motor nerves is reduced and F-waves are absent, and 7) most patients recover functionally. There are variants of this common presentation, such as acute motor axonal neuropathy and acute motor and sensory axonal neuropathy, of the many variants, the most aberrant is Fisher syndrome. The clinical criteria for diagnosis of Guillain-Barré syndrome and the electrodiagnostic criteria for demyelination of the peripheral nerve are described. Circulating antineural antibodies directed at a number of antigens have been demonstrated in GBS, occasionally against P2 protein (P2), galactocerebroside, and ganglioside GM1. We studied peripheral blood samples from patients with acute phase GBS using flow cytometry. The proportion of activated T cells (CD4 + HLA-DR +) was increased in comparison to the proportion in healthy controls. The helper-inducer (CD4 + CD29 +) subset was also increased; the suppressor-inducer (CD4 + CD45RA +) subset was neither increased nor decreased. Activated T cells and helper-inducer cells may play a crucial role in the onset of GBS. Lymphocytes sensitized to P2 were demonstrated in the acute stage of GBS, but not in any other neurological diseases. The lymphocytes recognized the P2 residues 60-78. We also demonstrated a significant relationship between GBS and IILA-A2, B61, Cw3, and DR6 antigens. Early plasmapheresis has been proved to be useful for patients with GBS. Intravenous immunoglobulin therapy appears to be as effective as plasmapheresis. Corticosteroids can no longer be considered useful therapy for GBS. Two randomized, controlled trials, one using conventional doses of prednisolone and high-dose intravenous methylprednisolone have shown no benefit.

Humans↗

[A 29-year-old man with diabetes insipidus and cerebellar ataxia and development of spinal cord swelling 15 years after the onset].

We report a 29-year-old man with diabetes insipidus and cerebellar ataxia who developed spinal cord swelling 15 years after the onset. He was well until 14 years of the age when he noted dizziness. Two years after there was an onset of gait disturbance and slurred speech. He also noted polydipsia and polyuria. He was evaluated at the neurosurgery service of our hospital when he was 17 years of the age. Neurologic examination at that time revealed memory loss, horizontal nystagmus, cerebellar ataxic gait, dysmetria and decomposition more on the left. Cranial CT scan revealed a mass lesion involving the left subthalamic region and the head of the caudate area. Spinal fluid was unremarkable, however, human chorionic gonadotropin was increased to 27 mIU/ml. He was treated by radiation therapy (3,000 rads for total brain area and 5,460 rads for focal region). His CT scan and memory loss improved, however, cerebellar ataxia was unchanged. Three years after the radiation, he started to show choreic movement in his neck and left upper extremity. He was admitted to our service in August 14, 1995 when he was 29 years of the age. On admission, he was alert but disoriented to time; calculation was also poor. Higher cerebral functions were intact. The optic fundi were normal without papilledema. Visual field appeared intact. Gaze nystagmus was observed in all the directions, but more prominent in the horizontal direction. Speech was slurred. Otherwise, cranial nerves were unremarkable. Motor wise, he showed marked truncal and gait ataxia; he was unable to walk because of ataxia. Muscle atrophy and marked weakness was noted in both upper extremities more on the left side. Deep tendon reflexes were diminished in the upper extremities but active in the lower extremities. He was polyuric; urinary specific gravity was low. Spinal fluid contained 6 cells/cmm and 113 mg/ dl of protein; Queckenstedt was positive. MRI revealed swelling of the cervical cord; in addition, the entire cervical region and the medullar oblongata appeared as high signal intensity areas. No mass lesion was noted in the supratentorial structures but the third ventricle was markedly enlarged. Surgical biopsy was performed on the cervical lesion. The patient was discussed in neurologic CPC, and the chief discussant arrived at the conclusion that the patient had germinoma with syncytiotrophoblastic giant cells in the diencephalic region which appeared to have been cured by radiation therapy; he thought that the cervical lesion was the seeding of germinoma. Cerebellar ataxia was ascribed to the remote effect of germinoma. Most of the participants thought that the original tumor was germinoma and the cervical lesion was its spread. Some participants thought that his ataxia was caused by germinoma cells involving the medulla and the inferior cerebellar peduncles. Histologic observation of the biopsied tissue from the spinal cord revealed the typical two cell patterned germinoma. Most of the tumor cells were not stained for an antibody against HCG, but some tumor cells were positively stained. Germinoma is very radio-sensitive; this patient showed T2 high signal lesion involving the medulla oblongata and cervical cord continuously. Probably, tumor cells in the lower brain stem escaped radiation, and gradually spread to the spinal cord over many years. At the time of operation, the surface of the spinal cord was free from tumor cells. Therefore, tumor cells invaded the spinal cord continuously from the medulla oblongata. He was treated with cervical radiation, and his neurologic as well as radiologic findings showed marked improvement.

Adult↗

[A 64-year-old woman with severe headache and progressive disturbance of consciousness].

We report a 64-year-old woman who developed nausea, headache, and consciousness disturbance. She was well until four years before the onset of her neurologic illness when (April of 1990 at her 59 years of the age) she was found to have an early cancer in her anterior wall of the lower stomach. Subtotal gastrectomy was performed and the operative result was reported as curative. Four years after the surgery (December of 1994 at her 64 years of the age), she noted suboccipital headache and nausea which had become progressively worse and she was admitted to our service on May 24, 1995. On admission, she appeared chronically ill but general physical examination was unremarkable with normal vital signs. Neurologically she was alert and not demented, and the higher cerebral functions were intact. Cranial nerves were also unremarkable. She was able to walk in tandem and on heels. No motor weakness or ataxia was noted. Deep tendon reflexes were moderately increased, however, no Babinski sign was noted. Although she had headache, no meningeal signs were seen. Slight superficial and vibratory sensory loss was noted in both feet. Routine blood work was again unremarkable except for slight increase in CEA to 8.3 ng/dl (N < 5 ng/dl). The opening pressure of lumbar CSF was 180 mm H2O and the CSF contained 39 cells/microliter, 79 mg of protein, and 10 mg/dl of glucose. Approximately half of the cells were atypical malignant cells. Plain CT was unremarkable, however, tentorial border showed enhancement after contrast infusion. FGS showed no malignant tumors in the stomach. She was treated with intravenous glycerol and whole brain radiation, however, she continued to complain of severe headache, and her sensorium started to be disturbed one month after the admission. Follow-up cranial CT scan revealed enlargement of the lateral and the third ventricles. Her consciousness progressively deteriorated and she became comatose three months after the admission. Repeated cranial CT scan showed enlargement of the ventricles, but no mass lesions were seen within the brain. She developed respiratory arrest on September 25 of the same year. She was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had a gastric cancer with meningeal seeding developing meningeal carcinomatosis. The cause of deep coma was ascribed to damage of cerebral cortical areas secondary to metastatic carcinoma cells and fibrinous materials in the surface of the brain. Postmortem examination revealed thickening and clouding of leptomeninges of the cerebral convexity. On histologic observation, patchy areas of fibrous thickening were seen in the cerebral leptomeninges; in such areas, adenocarcinomatous cells were seen scattered. The basal meninges were free of carcinoma cells, however, leptomeninges of the cerebellum and brain stem tegmentum contained scattered carcinoma cells. The lateral and the third ventricles were enlarged, however, insides of the brain were free of pathologies; the ependymal layer were intact. In the stomach no carcinoma cells were remaining. Pneumonic changes were seen in the right upper and the left lower lobes which appeared to be the direct cause of her death. No evidence of tentorial herniation was noted. The cause of her deep coma was not clearly determined, however, combination of hydrocephalus and cortical malfunction due to leptomeningeal carcinoma cell infiltration and fibrinous material accumulation appeared to have played a role.

Adenocarcinoma↗

[Axonal motor neuropathy associated with anti-SGPG antibody].

We report a case of axonal motor neuropathy associated with anti-sulfated glucuronic paragloboside (SGPG) antibody which has not been reported yet. A 49-year-old man was admitted with asymmetrical patchy weakness which started in his upper extremities. The deep tendon reflexes were absent in the upper extremities, but normal in the lower extremities. There was a slight decrease in the vibratory sensation in the distal portions of the lower extremities. The general laboratory tests including the protein level of the cerebrospinal fluid revealed no abnormalities. Motor conduction studies showed the low amplitudes of CMAP and no demonstrable conduction block in the limbs. Sensory conduction studies showed no abnormalities except for slightly decreased amplitude of SNAP in the limbs. Electromyography showed active denervation in the upper extremities. Serial electrophysiological studies suggested that the predominant process was axonal degeneration of the motor nerves. Thin-layer chromatography with immunostaining showed that his serum IgM reacted with SGPG. Treatment with cyclophosphamide and corticosteroids was unsuccessful. In this case, this anti-SGPG antibody may be involved in the pathogenesis of chronic axonal degeneration of the motor nerves.

Autoantibodies↗