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Biomedical subjects

G Sobue

Publications and source records attributed to G Sobue.

At least 217 records · Page 12Linked to original sources
PubMed1996

[Long-term maintenance therapy of multifocal motor neuropathy by weekly administration of human immunoglobulin].

High-dose intravenous immunoglobulin therapy for multifocal motor neuropathy (MMN) exhibits a beneficial, but temporary effect in most cases. We experienced a patient with MMN having a high titer of anti-GM1 ganglioside antibody. He was weekly administered human immunoglobulin, and his muscle strength was successfully improved and maintained at the improved level after high-dose human immunoglobulin infusion. The pattern of improvement and maintenance by weekly administration of human immunoglobulin was variable among the muscles; e.g. only a minimal improvement was observed in the most severely involved brachioradial muscles. Successive measurements with a hand-held dynamometer revealed in detail the changes in the strength of each muscle during treatment. Anti-GM1 ganglioside antibody titers did not significantly change during the treatment, but a slight improvement in motor conduction amplitudes and velocities was observed in mildly affected nerves. Our findings suggest that weekly administration of human immunoglobulin is a safe and effective therapy for MMN and that the anti-GM1 ganglioside antibody titer may not be a good indicator for this therapy.

Adult↗
PubMed1996

[A case of X-linked bulbar and spinal muscular atrophy with impaired neuromuscular transmission].

A 43-year-old man suffered from weakness of the upper limbs and easy fatigability. Low frequency (3 Hz) repetitive nerve stimulation (RNS) showed a marked waning with a maximum decrement of 20% of M wave amplitude. This remarkable waning was partially reduced by an administration of 60 mg bromopyridostigmine. High frequency RNS (30 Hz), however, did not induced waxing. Tensilon test and anti-AchR antibody were negative. The CAG repeat in androgen receptor gene was abnormally expanded ((CAG)n = 47), which confirmed a diagnosis of X-linked bulbar and spinal muscular atrophy. (BSMA). In some ALS patients, impaired neuromuscular transmission has been reported. The impaired neuromuscular transmission in ALS occurs in cases with rapidly progressive muscle wasting, suggesting that this abnormality is related to rapidly degenerating motor nerve endings. However, impaired neuromuscular transmission as seen in this patient has not been well documented in BSMA.

Adult↗
PubMed1996

[Clinical features of anterior inferior cerebellar artery territory infarcts--a study of ten patients].

Clinical features of the anterior inferior cerebellar artery (AICA) territory infarcts were investigated in ten patients, ranging in age from 38 to 76 years. In all patients, there were MR images of infarction located in the area supplied by the AICA. The lesion was on the left side in 6 patients and right side in 4. The lesion of brain stem including the middle cerebellar peduncle was found in 7 patients and that extended to the cerebellum was in 3 patients. The main ipsilateral neurological signs were the VII and VIII cranial nerves palsy and cerebellar ataxia. The V and VI cranial nerves palsy. Horner's syndrome, and dysphagia were also present. The main contralateral sign was superficial sensory disturbance, but no hemiplegia. The underlying pathology included chiefly hyperlipidemia, hypertension, and diabetes mellitus. Cerebral angiography was performed in 8 patients, most of which was observed severe arteriosclerosis suggesting poor hemodynamics in the vertebral and basilar arteries. The prognosis was relatively good, but the VII, VIII, and V cranial nerves palsy and contralateral superficial sensory disturbance remained as the sequelae. As mentioned above, there were various neurological findings and MR images in AICA territory infarcts. Especially there were some patients whose lesion extended to the upper medulla and neurological findings were similar to the Wallenberg syndrome. It is important that one investigates not only axial slices but also coronal slices of MR image to estimate the extension of AICA territory infarct.

Adult↗
PubMed1996

[Central facial weakness due to medullary pyramidal infarction; a case report].

We reported a hypertensive 40-year-old man who developed sudden right hemiparesis, deep sensory disturbance, left hypoglossal nerve palsy, and mild right central facial weakness. MRI of the brain showed an infarct located in the left upper medullary pyramid. Course and connection of the corticobulbar pathway are not well known in human. Recently Cavazos (1996) described a hypothesis that some facial corticobulbar fibers descend caudally ipsilaterally as low as the upper medulla, making a loop before decussating and ascending to the contralateral facial nucleus Contralateral central facial weakness may result from interruption of these descending fibers.

Adult↗
PubMed1996

[Pathophysiology of sensory ataxic neuropathy].

The main lesions of sensory ataxic neuropathy such as chronic idiopathic sensory ataxic neuropathy, (ISAN), carcinomatous neuropathy, Sjögren syndrome-associated neuropathy and acute autonomic and sensory neuropathy (AASN) are the large-diameter sensory neurons and dosal column of the spinal cord and the large myelinated fibers in the peripheral nerve trunks. In addition, afferent fibers to the Clarke's nuclei are also severely involved, suggesting Ia fibers being involved in these neuropathies. In NT-3 knockout mouse, an animal model of sensory ataxia, large-sized la neurons as well as muscle spindle and Golgi tendon organs are depleted, and are causative for sensory ataxia. Thus, the proprioceptive Ia neurons would play a role in pathogenesis of sensory ataxia in human sensory ataxic neuropathies, but the significance of dorsal column involvement in human sensory ataxia is still needed to evaluate.

Animals↗
PubMed1995

Primary sensory neurons in X-linked recessive bulbospinal neuropathy: histopathology and androgen receptor gene expression.

Pathology of the primary sensory neurons was examined in 7 autopsied patients and 6 biopsied sural nerves from the patients with X-linked recessive bulbospinal neuronopathy (SBMA). Large myelinated fibers in the central rami (L-4 posterior root, L-4, T-7, and C-6 segment of the fasciculus gracilis), and in the peripheral rami (sural nerve) were diminished in a distally accentuated manner, while small myelinated and unmyelinate fibers were well preserved in number. Demylinating process and axonal atrophy was ubiquitous. The diameter frequency histograms of the dorsal root ganglion (DRG) neurons showed a decrease in the number of large diameter neurons and an increase in the number of small diameter neurons without substantial loss of whole number of neurons, which suggested that neuronal size was atrophied. These data suggested central and peripheral distal axonopathy with neuronal atrophy was the process of sensory neuron involvement. Expression of mutant androgen receptor mRNA with elongated CAG repeat in the DRG and sural nerve supported the view that sensory nerve involvement is the primary process in SBMA.

Aged↗
PubMed1995

X-linked recessive bulbospinal neuronopathy: clinical phenotypes and CAG repeat size in androgen receptor gene.

Clinical phenotypes and the CAG repeat size of the androgen receptor gene were assessed in 95 Japanese patients with X-linked recessive bulbospinal neuronopathy. There was an age- and duration-dependent deterioration of muscle strength, ADL scores, and plasma creatine kinase levels. However, there was no correlation between the presence of absence of gynecomastia or diabetes mellitus and the age at onset or duration of illness. Correlations were present between the CAG repeat size and the age at onset (P < 0.0001) as well as the presence or absence of gynecomastea (P < 0.05). Muscular weakness and ADL scores were also correlated with the CAG repeat size only when they were adjusted by the age at examination not by the duration of illness. These findings suggest that CAG repeat size is one of the determinant factors of disease progression. However, extensive variation in phenotypic severity in patients with the same size of CAG repeat was present even among the siblings, suggesting that other factors than CAG repeat size influence the phenotypic manifestation. The average gain of CAG repeat size expansion was a 1.4 repeat in paternal transmission which was more unstable than that in maternal transmission, but the magnitude of the expansion in paternal transmission was much smaller than is presently known for other diseases in which CAG repeat expansion is the responsible gene defect.

Adolescent↗
PubMed1995

Clinico-pathophysiological features of acute autonomic and sensory neuropathy: a long-term follow-up study.

We evaluated the clinico-pathophysiological features of three patients with acute autonomic and sensory neuropathy (AASN) who were followed for over 3 years. Signs of an autonomic disturbance including vomiting, anhidrosis, urinary disturbances, orthostatic hypotension and reduced coefficient of variation of the R-R interval on electrocardiography gradually improved about 1 year after onset. However, all three exhibited severe generalized sensory impairment for all modalities with the development of persistent sensory ataxia. No sensory nerve action potentials could be elicited and no somatosensory evoked potentials could be obtained. Sural nerve biopsy revealed severe axonopathy. In two patients, a high-intensity area was observed in the posterior column of the spinal cord on T2*-weighted axial magnetic resonance images. The level of neuron-specific enolase in cerebrospinal fluid was markedly elevated in two patients, indicating spinal nerve root or sensory neuron damage. Motor nerve function was well preserved in all patients. Our findings suggests that the major lesion in patients with AASN, particularly those with a sensory deficit, is present in the dorsal root ganglion neurons, that is there is a ganglioneuronopathy.

Action Potentials↗
PubMed1995

Magnetic resonance imaging of the corticospinal tracts in amyotrophic lateral sclerosis.

In 13 patients with amyotrophic lateral sclerosis (ALS), corticospinal tract lesions on spinal cord, brain and brain stem were examined by MR imaging. In 9 patients, areas of high signal intensity located in the dorsolateral columns coinciding with the lateral corticospinal tracts, were detected on axial T2*-weighted MR imaging of the cervical spinal cord using a gradient echo technique. In two patients, these spinal cord MR abnormalities corresponded well to the postmortem pathological findings of lateral corticospinal tract degeneration. T2-weighted abnormal MR signals along the corticospinal tract at the brain and brain stem were detected in 4 patients, all of whom also showed abnormal signals on cervical cord MR imaging. Four of 13 patients did not show any abnormal signals on brain and brain stem or spinal cord MR imaging. Spinal cord MR imaging provides a useful information regarding upper motor neuron lesions in ALS.

Adult↗
PubMed1995

Molecular features of the CAG repeats and clinical manifestation of Machado-Joseph disease.

Machado--Joseph disease (MJD) is an autosomal dominant spinocerebellar degeneration mapped to chromosome 14q32.1. The CAG expansions of the MJD1 gene was identified as the cause of the disease. We have analyzed 90 MJD individuals from 62 independent MJD families and found that the MJD1 repeat length is inversely correlated with the age of onset (r = -0.87). The MJD chromosomes contained 61-84 repeat units, whereas normal chromosomes displayed 14-34 repeats. In the normal chromosomes, 14 repeat units were the most common and the shortest. In association with the clinical anticipation of the disease, a parent--child analysis showed the unidirectional expansion of CAG repeats and no case of diminution in the affected family. The differences in CAG repeat length between parent and child and between siblings are greater in paternal transmission than in maternal transmission. Detailed analysis revealed that a large degree of expansion was associated with a shorter length of MJD1 gene in paternal transmission. On the other hand, the increments of increase were similar for shorter and longer expansion in maternal transmission. Among the three clinical subtypes, type I of MJD, with dystonia, showed a larger degree of expansion in CAG repeats of the gene and younger ages of onset than the other types.

Adult↗
PubMed1995

Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy; different beneficial effects and their correlation to the clinical features.

We report 7 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who showed significant clinical improvement by plasma exchange (PE). Their clinical features were extensively varied but fulfilled the diagnostic criteria for CIDP. The patients could be divided into 2 subgroups based on the mode of effectiveness of PE; one group consisted of those who once achieved complete remission, but required highly frequent PEs for long-term maintenance of remission and the others were those who showed persistent and complete remission with several sessions of PE alone. These findings raise issues about the long-term therapeutic goals to be achieved by PE for CIDP patients, particularly on supportive therapy other than PE, as well as the optimization of frequency and duration of PE.

Adult↗
PubMed1995

Distribution of thyrotropin releasing hormone receptor in rats: an immunohistochemical study.

Thyrotropin releasing hormone (TRH) receptor was identified immunohistochemically in the rat tissues using anti-peptide antiserum. Anti-TRH receptor antiserum was raised in New Zealand white rabbits immunized with a conjugate of synthetic TRH-receptor peptide (15-28) to bovine serum albumin. Immunohistochemical analysis was performed by the avidin-biotin complex method. TRH-receptor immunoreactivity was visualized in the central nervous system and anterior pituitary thus supporting previous investigations of TRH receptor distribution using in vitro autoradiographic ligand binding. Significant stain was detected in neural perikarya, axons and dendrites as well as in many cells of the retina, adrenal medulla, stomach mucosa, Auerbach's nervous branch and Mysner's nervous branch of the stomach, small intestine and colon. When using antiserum preincubated with synthetic TRH-receptor peptide (15-28) or rat anterior pituitary homogenate which contains TRH-receptor peptide, no significant stain of the anterior pituitary cells or neurons in the hypothalamus was detected. These findings suggest that TRH-receptor is widely distributed and that this method is valuable in studying the distribution of TRH-receptor in rats.

Journal Article↗
PubMed1995

Distribution of somatostatin receptor type 1 in the rat. An immunohistochemical study.

Somatostatin receptor type 1 (SSTR-1) was identified immunohistochemically in the rat tissues using anti-peptide antiserum. Anti-somatostatin receptor antiserum was raised in New Zealand white rabbits immunized with a conjugate of synthetic SSTR-1 peptide (41-52) with bovine serum albumin. Immunohistochemical analysis was performed by the ABC method. SSTR-1 immunoreactivity was visualized in the central nervous system, anterior pituitary, mucosa of stomach and duodenum, Auerbach's nervous branch and Mysner's nervous branch of gastrointestinal tract and pancreas. Significant staining was detected in neural perikarya, axon and dendrite. When using antiserum preincubated with synthetic SSTR-1 peptide (41-52) or rat anterior pituitary homogenated which contains SSTR-1, no significantly stained anterior pituitary cells or neurons in the hypothalamus were detected. These findings suggest that SSTR-1 is widely distributed and that this method is valuable in studying the distribution of somatostatin receptor in rats.

Journal Article↗
PubMed1995

[Abnormality of PMP-22 gene in Japanese patients with Charcot-Marie-Tooth disease--comparison between Southern blot and polymerase chain reaction analysis in the detection of PMP-22 gene duplication].

We investigated the duplication of the PMP-22 gene in 23 Japanese patients with Charcot-Marie-Tooth disease (CMT) by Southern blot and PCR analysis. To detect duplication of the PMP-22 gene region, PMP-22 cDNA and a polymorphic marker, VAW409R3, located in the region flanking the PMP-22 gene, were used as probes for Southern blot analysis. A marker, 6G1, also located in the PMP-22 flanking region, was amplified using the quantitative PCR method. The signal intensity of the 2.8-kb and 2.7-kb bands of MspI digests probed with VAW409R3 was different in patients with duplication. The ratio of these two bands, measured by densitometry, ranged from 1.75 to 2.13 in the patients with duplication and from 1.01 to 1.15 in those without duplication and in normal controls. The signal ratio of PMP-22 to the reference marker SF85 of BamHI digests ranged from 1.15 to 1.33 in the patients with duplication and from 0.96 to 1.04 in those without duplication, when compared with normal controls assigned a value of 1.0. The ranges of the intensity in these two groups were narrow, but did not overlap. The signal ratio of the PCR products of 6G1 to reference marker D1S80 on quantitative PCR analysis was also measured. The ratio ranged between 1.67 and 2.23 with duplication and between 1.29 and 1.74 without duplication according to the results of Southern blot analysis. However, some patients exhibited overlapping signal intensity in the duplication and non-duplication ranges. Thus, these three methods each has its own advantages and disadvantages in regard to detecting duplication.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗