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PubMed2003

[Clinical features of patients associated with anti-GM1b antibody].

To clarify the clinical features of patients with anti-GM1b antibody, we retrospectively investigated which conditions were associated with anti-GM1b antibody in a large number of patients. Two hundred out of 1,713 patients had anti-GM1b antibody. One hundred and sixty-six (83%) were diagnosed with Guillain-Barré syndrome (GBS) or atypical GBS with preserved deep tendon reflexes. Our study suggests that the anti-GM1b antibody testing is useful for supporting the diagnosis of GBS. In addition, anti-GM1b antibody was detected in 18 patients with Fisher syndrome and the related conditions associated with anti-GQ1b IgG antibody, and 3 patients with ataxic GBS associated with anti-GD1b IgG antibody.

Adult↗
PubMed2003

[Dichloroacetate treatment for adult patients with mitochondrial disease].

We report beneficial and adverse effects of sodium dichloroacetate (DCA) in three adult Japanese patients with mitochondrial disease: a 21-year-old male with involuntary movements, optic atrophy, hearing loss, and convulsions (patient 1), a 28-year-old man with mental deterioration, hemianopia, hearing disturbance, and convulsions (patient 2), and a 50-year-old woman with hearing disturbance, generalized muscle atrophy, and insulin dependent diabetes mellitus (patient 3). A3243G mutation was found in patient 2 and patient 3. Oral administration of DCA improved consciousness level and gait disturbances in patient 1, and ameliorated headaches, easy fatiguability, and muscle cramps in patient 2 and patient 3. DCA normalized high levels of lactate and pyruvate in blood and cerebrospinal fluids in all three patients. In patient 3, daily insulin needs decreased from 38 to 24 units, and urine C peptide increased from an undetectable level to 16 micrograms/day. In patient 1, DCA 23 mg/kg/day had been beneficial without adverse effects and he became free of convulsions for more than 32 months. However, despite of normal lactate and pyruvate, unsteady gait and lethargy developed after 50 mg/kg/day treatment for two months and one month in patient 2 and patient 3, respectively. In both patients, deep tendon reflexes disappeared and Romberg sign became positive. Nerve conduction studies confirmed sensory-dominant polyneuropathy and electroencephalogram showed diffuse slow basic activities. Cessation of DCA resulted in recovery of gait and consciousness, but sensory nerve action potentials did not recover in one month. Long term treatment of 50 mg/kg/day DCA may affect adversely the peripheral and central nervous systems in adult patients. Although effective plasma DCA concentration was previously reported as 25-160 micrograms/ml in patients under 18 years old, plasma DCA concentration of 10.2 micrograms/ml was sufficient in patient 1. We recommend lower dose of DCA in adult patients than in child patients.

Adult↗
PubMed1992

[Adult-onset neuronal ceroid lipofuscinosis--a case report with biological study].

A 32-year-old male was admitted to our hospital complaining of dementia, gait disturbance and blindness. These symptoms developed at the early two decade and were progressive. On admission, his clinical features included dementia (IQ = 69), spasticity, accentuated deep tendon reflexes, ataxia and hypesthesias in his distal limbs. Brain CT scans showed diffuse cerebral atrophy. On light microscopy, many abnormal lipopigments resembling ceroid and lipofuscin were found in Schwann cells of sural nerve and histiocytes of colon. Ultrastructurally, these materials showed lamellar structure like Zebra bodies. Nine lysosomal enzymes, serum very long-chain fatty acids, serum amino acids and urinary oligosaccharides were all normal. Neuronal ceroid lipofuscinosis (NCL) of adult type was diagnosed on the basis of clinical features, radiological and pathological findings, and biochemical studies. Many previous studies suggested that NCL was a disorder with lysosomal dysfunction. We examined lysosomal protein degradation, using 125I-low density lipoprotein (LDL) in cultured fibroblasts from this patient. The degradation of LDL was normal, compared to control subjects. The activities of cathepsin and lysosomal glycosidases, were also normal. The amount of urinary dolichol has been reported to be elevated in the patients with infantile and late infantile types of NCL. However, no elevation was found in the urine of our patient.

Adult↗
PubMed2003

[Evidence-based diagnosis in clinical practice: a patient with hypercholesterolemia].

Evidence-based diagnosis(EBD) is a method for clinical problem solving. The practice of EBD comprises five steps(formulating answerable clinical questions, searching for the best available evidence, critical appraisal of the evidence, application of the evidence to the patients, evaluation of execution of steps 1-4) as well as EBM. I present a case for studying the practice of EBD. The patient is a woman with hypercholesterolemia. It is essential to rule out secondary hypercholesterolemia. A test with high sensitivity is useful to rule out diseases. We call this situation SnNout(a High Sensitivity, a Negative result rules out diagnosis). The level of TSH is SnNout to rule out hypothyroidism with hypercholesterolemia. Clinical score is not SnNout, but may be useful in actual practice. The delay of relaxing phase of tendon reflex is not useful because of low sensitivity. Each clinical findings has a sensitivity and a specificity. It is important for clinicians to consider this fact.

Evidence-Based Medicine↗
PubMedDate not supplied

Classifications of hereditary ataxias. A critical overview.

The classifications of hereditary ataxias (HA) proposed from 1907 to 1984 are reviewed. An analysis is provided of the possible variables in the classification of HA, including inheritance, known metabolic or other cause, localization of pathological lesions, clinical signs, natural history, epidemiology, diagnostic tools. Harding's classification is assumed to be the best clinical tool to support molecular genetics studies. However, we suggest the inclusion of Late Onset Recessive Cerebellar Ataxias in Harding's classification. Some exceptions must be considered for the diagnostic criteria of Friedreich's disease. Early Onset Cerebellar Ataxia with retained tendon reflexes (EOCA) is probably a heterogeneous entity.

Ataxia↗
PubMedDate not supplied

Early onset hereditary ataxias of unknown etiology. Review of a personal series.

Among 300 patients affected by hereditary ataxia, 94 received the diagnosis of Friedreich's disease, 12 of Late Onset Friedreich's disease, 27 of Early Onset Cerebellar Ataxia with retained tendon reflexes, 10 of Progressive Myoclonic Ataxia, 4 of Ataxia with hypogonadism and 2 of Ataxia with hearing loss. Only Friedreich's disease appears clinically homogeneous, whereas the others are not specific entities and each of them probably includes different diseases.

Adolescent↗
PubMed1992

[Migrant sensory neuritis--electrophysiological and pathological study].

Migrant sensory neuritis, which was first proposed by Wartenberg, is very uncommon and only a few case reports have ever been published. We described one case of migrant sensory neuritis and discussed the pathogenesis of this disease. A 44-year-old man noticed numbness in the lateral aspect of the dorsum of the left foot in February 1985. Physically, there was hypoesthesia in the region of left sural nerve with positive Tinel's sign. During the next 4 years, pain, tingling sensation or hypoesthesia appeared in the regions of the right superficial radial nerve, right axillary nerve, left intercostal nerve, left lateral plantar nerve, digital nerve of the right second digit, left saphenous nerve, right superficial peroneal nerve, left superficial radial nerve, bilateral ulnar nerves and bilateral median antebrachial cutaneous nerves one after another in a migrating fashion. Tinel's sign was also positive at the right superficial radial nerve. In some occasions, decreased deep tendon reflexes were observed, but there had been no muscle weakness. Some nerves showed complete recovery, but others showed persistent involvement. Some nerves were affected repeatedly. Laboratory examination failed to clarify underlying disease except for mild liver dysfunction. Electrophysiological study showed reduced amplitude of the sensory nerve action potential (SNAP) of right sural nerve, left ulnar nerve, right superficial radial nerve and digital branch of right median nerve with preserved sensory nerve conduction velocity. SNAP of left sural nerve was absent. These findings mean the cause of the sensory disturbance is axonal degeneration rather than segmental demyelination. There were no abnormalities in motor nerve conduction study.(ABSTRACT TRUNCATED AT 250 WORDS)

Action Potentials↗
PubMed1992

[Abulia: a case of cerebral infarction in the bilateral genua of internal capsules].

A patient was presented with an outstanding symptom of abulia due to cerebral infarcts in the bilateral genua of internal capsules. A 53-year-old woman, generally in good health and active, had no contributory medical history except for hypertension. She was well until August 20, 1988, when she was noted to have become taciturn and absent-minded. In the morning, she got up and went to work as usual. Although she worked without trouble, she hardly talked with her colleagues. After getting home from work, she would lie down without doing any housework, and this was continued on the following day. However, she had no physical problems. She was thus admitted to a hospital on August 22. Lethargy and urinary incontinence were apparent for a few days. Thereafter she became awakeful and could take care of herself. She sat on her bed all the time, and could talk normally with her daughter. She was referred subsequently to the Department of Neurology, Hyogo Prefectural Tsukaguchi Hospital on August 30. On examination, the patient was alert, polite and cooperative with no physical abnormalities except for high blood pressure. Neurological examination indicated the patient to be attentive and well-oriented. Cranial nerves and eye movements were normal except for slight anisocoria and sluggish pupils. There were no muscle weakness, extrapyramidal signs, or cerebellar signs. Deep tendon reflexes were normal. Babinski signs and forced grasping were not noted. A neuropsychological study showed the patient not to be demented, aphasic, or apraxic.(ABSTRACT TRUNCATED AT 250 WORDS)

Akinetic Mutism↗
PubMed1992

[Intramedullary neurinoma. Apropos of a case. Review of the literature].

The authors present a case of intramedullary neurinoma. A 44 years old patient was admitted for lumbar pain and bilateral sciatica. Neurological examination was normal except for dysuria and diminution of Achilles tendon reflexes. Lumbar spine X-rays and spinal cord angiogram were normal, but myelogram and myelo-scan revealed a fusiform enlargement of the conus medullaris. MRI confirmed this aspect. Total removal of an intramedullary tumor was done, using ultrasonography, operating microscope and ultrasonic aspiration. The post-operative course was uneventful, with, as sequelae a perineal hypoesthesia. Histological examination of the excised tumor revealed a neurinoma. The epidemiology, clinical features, radiology, and surgical treatment of such rare intramedullary tumors are discussed.

Adult↗
PubMed1992

[A case of early onset cerebellar ataxia with hearing loss, mental disturbance and primary hypogonadism].

A 14-year-old girl, whose birth and developmental history were normal till the age of 7, was admitted to our hospital because of slowly progressive difficulties in walking, speaking and hearing. She also complained of absence of menstruation. She showed poor school records since the age of 7. On neurological examination, she showed limb and truncal ataxia. There was no nystagmus but slurred speech was found. Muscular power was good and her sensory system was normal. Tendon reflexes were equally present, and plantar reflexes were flexor. Bilateral moderate nerve deafness was also present. Mental deficiency was diagnosed on an intelligence test. Brain CT and MRI showed cerebellar atrophy. Gynecological examination revealed scanty pubic hair and small uterus. Karyotype was 46XX. Endocrinological studies demonstrated high level of FSH, low level of E2, and the normal response to pituitary stimulation with LHRH, indicating the existence of primary hypogonadism. Although the etiology of this multisystem disorder is unknown, it is possible that both nervous and endocrine disorders were genetically determined.

Adolescent↗
PubMed1992

[Influence of treating hypothyroidism and hyperthyroidism upon psychical reaction time].

The aim of the study was answering the question whether determination of psychical reaction time may be useful for the monitoring of treatment of hypothyreosis and hyperthyreosis. The correlation of disease symptoms (diagnostic index for hypothyroidism after Murray and for hypothyroidism according to Crooks), concentration of triiodothyronine, thyroxine, index of free thyroxine in blood serum and Achilles tendon reflex--with the simple reaction time (srt) as well as with the choice reaction time (chrt) before and during treatment was investigated in 18 patients with primary hypothyroidism and in 24 with hyperthyroidism caused by Graves-Basedow's disease. Two control groups comprised 84 healthy persons. Either in hypothyroidism or in hyperthyroidism the srt and chrt was significantly prolonged. The substitution therapy in hypothyroidism resulted in a normalization of both parameters. On the other hand, administration of thiamazole, especially in the early period of treatment of hyperthyroidism, further prolonged the srt and chrt. A shortening of the time of each of the two reactions occurred, however, when the restoration of euthyroidism was achieved, which was accompanied by reduction of thiamazole dosage. The obtained data point to the usefulness of srt and chrt determination for the diagnosis and therapy monitoring of functional disturbances of the thyroid gland.

Adult↗
PubMed1992

[A case of cerebral infarction presenting as retrosplenial amnesia].

We report a 73-year-old right-handed female who presented with an acute amnesic syndrome. On November 18, 1991, she was admitted to a local hospital complaining of sudden-onset vertigo and nausea, but immediately after the admission she developed an amnesic syndrome. On November 27, she was transferred to our hospital for further assessment of her memory disturbance. Neurologically she was normal except for mild right hemianopsia and increased deep tendon reflexes in the extremities. Neuropsychological assessments were performed over 3 weeks. She was always alert, attentive, and cooperative. She had no confabulation. On the Wechsler Adult Intelligence Scale revised (WAIS-R), her total IQ was 110. Frontal, verbal, and perceptual functions and motor performance were normal. She had no signs of a callosal disconnection. Despite these preserved functions, her memory function was obviously disturbed. Several memory betteries showed that her recent memory for both verbal and visual modalities was impaired, while her immediate memory such as digit span was preserved. For remote memory her retrograde episodic memory concerning both personal and public events was almost intact, although she had a profound anterograde amnesia. In particular she recalled her personal information about just-premorbid events in detail. On the other hand, her semantic memory, for example understanding of proverbs, geography, and scientific law, was preserved. Taken together, her procedural memory on learning tasks, such as "Tower of Hanoi" and mirror drawing, was intact. Computed tomography demonstrated a low-density area medial to the trigon of the left ventricle.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1992

[A case of chronic multifocal myositis].

A 61-year-old civil engineer began to have slowly progressive muscle atrophy in the right shoulder and the left arm at 56 years of age. Muscle wasting became manifest in the left thigh at 59 years and in the right thigh at 60 years. He had mild difficulty in climbing and descending stairs. On examination, although he had notable muscle atrophy in the right trapezius and proximal muscles in the upper and lower extremities, his muscle strength was relatively well preserved. The muscle atrophy was asymmetrical; the right periscapular region and the left upper and lower extremities were more markedly atrophic. In addition, multiple foci of the striking muscle atrophy were noted in the upper trunk and the proximal limb muscles. Fasciculation was not present. Deep tendon reflexes were normal with no pathologic reflexes. Except for a moderately elevated serum creatine kinase level of 709 Ul/l (normal 40-170) and mildly elevated serum myoglobin level of 100 ng/ml (normal < 60), no laboratory tests showed abnormal values suggesting an inflammatory process. Motor and sensory nerve conduction velocities were within normal limits. Electromyography disclosed myopathic and neuropathic changes. Computed tomography (CT) of skeletal muscles showed asymmetrical muscle atrophy and patchy low-density foci. In biopsied left quadriceps and right gastrocnemius muscles which showed partially low density on CT, there was marked variation in muscle fiber size, with necrotic and regenerating fibers, an increased number of centrally placed nuclei, and interstitial fibrosis. There were numerous foci of mononuclear inflammatory cellular infiltration, especially around the blood vessels.(ABSTRACT TRUNCATED AT 250 WORDS)

Chronic Disease↗
PubMed1992

[Clinical analysis of 30 cases of stiff-man syndrome].

Thirty cases of SMS, including 22 cases reported previously in China, were analysed clinically manifested by involvement of neck and facial muscles, slurred speech, dysphagia or dyspnea, exaggerated tendon reflexes, ankle clonus and/or Hoffmann's sign. Half of them had past history of infection. Five out of 18 CSF examined showed elevation of protein content, immunoglobulin and white cell count, suggesting the presence of inflammation or demyelination changes in CNS. The pathology of the syndrome is probably located in the spinal cord or brain stem. The criteria for diagnosis are proposed. For treatment, the dosage and way of administration of diazepam should be judged according to the severeness of the disease; nitrazepam and clonazepam are effective, too.

Adolescent↗
PubMed1992

Post-polio syndrome: a review and case report.

A 21-year-old Thai man presented with progressive weakness and atrophy of both hands for 2 years. His left leg was atrophic and weak secondary to poliomyelitis since he was 8 months old. Physical examination showed that there was atrophy of both hands and forearms as well as left leg. Sensation was normal. Deep tendon reflexes were normal except for hyporeflexia of left leg. EMG showed chronic denervation pattern in the recent atrophic muscles and in normal power muscles. Motor and sensory nerve conductions were normal. Biopsy of forearm muscle revealed degeneration and fibrosis of muscle fibers. These findings were compatible with post-polio syndrome (PPS) which presents in patients with late paralysis following poliomyelitis. It is not a form of amyotrophic lateral sclerosis although some clinical similarities exist. Weakness from PPS may lead to musculoskeletal pain and increased functional disability. Proper rehabilitation program is to maintain his functional status and allow him to continue to live as independently as possible by regular exercise without stressing joints and muscles.

Adult↗
PubMed1992

[Acute relapsing sensory neuropathy following upper respiratory infections--a case report].

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.

Acute Disease↗
PubMed1992

[Immunohistochemical studies of paraneoplastic subacute sensory neuropathy--an analysis of antineuronal antibody and infiltrated lymphocytes].

In order to clarify the pathogenesis of paraneoplastic syndrome, immunohistochemical studies were performed in a patient with subacute sensory neuropathy secondary to a small cell lung cancer. The case was a 73-year-old ex-farmer, whose chief complaints were pins and needles sensation of distal limbs and gait difficulty. After 6 weeks prodromata of pain in the upper limbs and numbness in all the limbs, he became unable to stand up without assistance. Neurological examinations on admission revealed marked sensory disturbances with glove and stocking type hypalgesia to pin prick and the loss of position and vibration senses in the distal extremities. His deep tendon reflexes also decreased in all the limbs. A chest X-ray showed a mass in the left upper lung field. A transbronchial lung biopsy of the mass revealed a small cell carcinoma. He was treated with anti-cancer drugs and radiation but he died of pneumonia after 8 months illness. Autopsy revealed a marked demyelination of the entire posterior column of the spinal cord. Dorsal root ganglia were infiltrated by lymphocytes with significant neuronal loss. Immunohistochemically, most of the infiltrated cells around the neurons were classified as CD8+ with fewer CD4+ lymphocytes. No B-lymphocytes were detected in the ganglia. The HLA-ABC and HLA-DR positive cells were found only among the satellite cells, not in the neurons. The serum and CSF from the patient were immunohistologically reacted with the nuclei and cytoplasm of all neurons of human as well as of rats, indicating the presence of anti-Hu type antineuronal antibody in the patient's CSF as well as serum.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1992

[A case of chronic toluene poisoning with myopathic changes].

We reported a 27-year-old man, who had inhaled toluene vapor for 11 years and developed both polyneuropathy and myopathy. He gradually developed progressive muscular weakness in the limbs 2 months after the last inhalation and became nonambulant. Neurological examination revealed distal dominant muscular weakness and hypoactive deep tendon reflexes, especially in the legs. The cranial nerves were intact and neither sensory disturbances nor cerebellar signs were observed. Laboratory studies demonstrated mildly elevated serum CK value, but serum electrolytes and urinalysis were normal. Electromyogram showed a neurogenic pattern in the limbs and also a myogenic in the gastrocnemius muscle. Motor nerve conduction velocities were delayed in the limbs. The sural nerve biopsy specimens showed demyelination, myelin avoids, and axonal swelling compatible with toxic neuropathy. The muscle biopsy of the gastrocnemius revealed necrotic muscle fibers, vacuolated muscle fibers, phagocytosis, a disordered intermyofibrillary network, and small group atrophy on light microscopy. On electron microscopy, two outstanding findings were observed; the first, dilatation and proliferation of the membrane system, and the second, appearance of irregular membrano lamellar structures and vacuoles associated with abnormal membrane system. These histological findings suggest that toluene may affect directly the membrane system of muscle fibers and consequently induce rhabdomyolysis.

Adult↗