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Biomedical subjects

I Sobue

Publications and source records attributed to I Sobue.

At least 19 recordsLinked to original sources
PubMed1992

Central motor and sensory conduction in X-linked recessive bulbospinal neuronopathy.

Central conduction was studied in 12 patients with X-linked recessive bulbospinal neuronopathy (XBSN) using percutaneous electrical cortical, cervical and lumbar stimulation and somatosensory evoked potentials (SEPs). The central motor conduction time from the motor cortex to the cervical and lumbar segments of the spinal cord was normal in XBSN. SEPs, however, were abnormal or central sensory conduction time was prolonged in patients with XBSN. These results are consistent with the clinicopathological findings of XBSN in which the primary sensory neurons are involved as well as the lower motor neurons in the CNS, whereas the upper motor neurons are well preserved.

Adult↗
PubMed1990

[Ageing and central motor conduction time].

The corticomotoneuronal pathway in the aged was studied using the technique of percutaneous electrical stimulation of the brain and the spinal cord. Central motor conduction time (CMCT) from the cerebral motor cortex to the cervical segment of the spinal cord (C-CMCT) was measured in 26 intact volunteers (20-85 years old) by substracting the latency to onset of EMG activity of the thenar muscle after cervical stimulation from the latency to onset of EMG activity of the same muscle after cortical stimulation. The latencies to onset of action potential of the anterior tibial muscle after cortical stimulation and the onset of action potential of the same muscle after lumbar stimulation were also measured in 11 volunteers (aged 46-67 years), and the difference between the two latencies was evaluated as the central motor conduction time from the cortex to the lumbar segment of the spinal cord (L-CMCT). Mean C-CMCT was 5.1 ms. and mean L-CMCT was 12.8 ms. Neither was related to the patient's height or age. There was no difference in either C-CMCT or L-CMCT between males and females. The results suggested that the conduction of the pyramidal tract is not affected by age, and this must be recognised when motor functions in the aged are studied.

Adult↗
PubMed1989

[Analysis of follow-up survey in spinocerebellar degeneration].

Spinocerebellar degeneration (SCD) is associated with other various degeneration of the nervous systems such as the optic tract, pyramidal pathway, extrapyramidal system, nuclei of the brain stem and autonomic nervous system as well as changes of heart. The clinical pattern, also have the great variability. We investigated the mode of progression of clinical symptoms and signs in 214 cases of SCD which were examined 2 times at intervals of about 10 years. 79 of 214 cases were reported to be died at the last examination. 135 alive cases included 3 with the Holmes type, 14 with late cortical cerebellar atrophy (LCCA) 10 with Menzel type, 18 with olive-ponto-cerebellar atrophy (OPCA), 33 with spinocerebellar form (SCF), 6 with Friedreich's ataxia, 18 with hereditary spastic paraparesis (HSP) and 33 with the other type. 79 dead cases included 0 with the Holmes type, 6 with LCCA, 5 with Menzel type, 32 with OPCA, 16 with SCF, 1 with Friedreich's ataxia, 4 with HSP and 15 with the other type. The disability of daily living in SCD revealed slower progression in the advanced stage than in the early stage. Every type of SCD had some different progression of disability each other. In the early stage, Friedreich's ataxia showed the highest progression of disability, but in the advanced stage, Holmes type and the OPCA did. Holmes type showed progression of ataxia without any remarkable change of other systems. LCCA showed increase of abnormality in the eye movements, pyramidal tract and autonomic nervous system in addition to the cerebellar system. OPCA involved multiple systems as ataxia worsening, but Menzel type had no remarkable changes of incidence in eye movement disorder.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1988

Neurotransmitter receptors of the rolling mouse Nagoya: a quantitative autoradiographic study.

The distribution of neurotransmitter and neuromodulator receptors was studied in the brain of the rolling mouse Nagoya (RMN) and in controls, using in vitro receptor autoradiography. Quantitative autoradiography was used to map adenosine A1 (labeled with [3H]cyclohexyladenosine), GABAA [( 3H]muscimol), opiate [( 3H]naloxone), L-glutamate [( 3H]L-glutamate), benzodiazepine [( 3H]flunitrazepam), and muscarinic cholinergic [( 3H]quinuclidinyl benzilate) receptors. In the cerebellar cortex, GABAA and adenosine A1 binding sites were significantly reduced in the RMN, whereas other transmitter binding sites were not significantly altered. Adenosine A1 binding sites were also reduced in the cerebral cortex and caudate-putamen. Benzodiazepine binding was significantly decreased in the cerebral cortex and increased in the CA1 subfield of the hippocampus. These results suggest that neurochemical alterations in the caudate-putamen as well as in the cerebellar cortex play important roles in the ataxia and motor dysfunction of the RMN.

Animals↗
PubMed1986

A family with beta-galactosidase deficiency: three adults with atypical clinical patterns.

Three adult patients in a single family showed severe myoclonus, ataxia, and pyramidal signs. Enzymatic analysis of lymphocytes, plasma, and cultured skin fibroblasts showed marked deficiency of beta-galactosidase activity, more profound with GM1 ganglioside than with another natural substrate, asialofetuin. Other lysosomal hydrolases were normal. Although the physical signs were similar to those of types 1 and 2 GM1 gangliosidosis, none had bony abnormalities.

Adult↗
PubMed1985

A microassay for acid beta-galactosidase activity toward asialofetuin.

To study the enzymatic properties of beta-galactosidase from the patients with a beta-galactosidase deficiency such as GM1 gangliosidosis, determination of enzymatic activity with naturally occurring substrates, asialofetuin in addition to another natural substrate, GM1 ganglioside, is essentially required. With a previously reported, simple and sensitive fluorometric assay for GM1 ganglioside beta-galactosidase using high performance liquid chromatography (HPLC), optimal reaction conditions were determined for the assay of acid beta-galactosidase activity toward asialofetuin in skin fibroblast homogenates. Under these conditions, reduced enzymatic activities could be detected in cultured skin fibroblasts from patients with type 1 and 3 GM1 gangliosidoses and mucopolysaccharidosis IV-B (Morquio B syndrome). This method was applicable to study of the enzymatic properties of the mutant beta-galactosidase and provided an alternative to assays employing radioactive or artificial substrates.

Asialoglycoproteins↗
PubMed1978

Cervical myelopathy due to ossification of the posterior longitudinal ligament: a clinicopathologic study.

A clinicopathologic study was done of a Japanese patient with symptomatic ossification of the posterior longitudinal ligament in the cervical spine. Post-mortem specimen showed characteristic distribution of degenerations at C5-6 segments similar to chronic cervical spondylosis or disk protrusion. The degenerative changes were confined to the ventral two thirds of the posterior and lateral columns. In addition to demyelination, loss of axons, and neuronal cell death, thickening of venular wall and fine capillary neovascularization strongly suggested chronic circulatory insufficiency. Spinal immobilization produced by the ossified ligament may have contributed to the long clinical course, lasting for 30 years. Review of literature showed that the myelopathy can be distinguished clinically and radiographically from that produced by cervical spondylosis.

Aged↗
PubMed1978

Juvenile type of distal and segmental muscular atrophy of upper extremities.

Seventy-one cases of distal and segmental muscular atrophy of the upper extremities with juvenile onset were studied. The clinical features consisted of: juvenile onset, male preponderance, unique distribution of the muscular atrophy in the hand and forearm, tendon reflexes hypoactive in most cases but hyperactive in some, no definite sensory disturbances, no involvement of the cranial nerves, and autonomic nerve disorders in the affected region. There was rapid progression during the 2 to 3 years after onset with a slowly progressive course thereafter. There were no abnormal laboratory findings except for electrophysiological and morphological findings of the affected muscles. The site of lesion was surmised to be from the C5 to T1 spinal segments with intramedullary involvement. The cause is unknown.

Adolescent↗