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Biomedical subjects

G Sobue

Publications and source records attributed to G Sobue.

At least 289 records · Page 16Linked to original sources
PubMed1991

[Two cases of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) associated with Graves' disease].

We have reported two cases of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) associated with Graves' disease. Case 1: a 45-year-old woman noticed a diffuse goiter, palpitation and emaciation in 1977. Laboratory studies confirmed that she had Graves' disease, and she was treated with antithyroid drug. In 1986, when the hyperthyroidism was subsided, she showed progressive symmetrical weakness and numbness in her limbs, and she was almost in tetraplegia at 1987. Markedly slowed motor and sensory nerve conductions and elevated CSF proteins as well as clinical manifestations confirmed the diagnosis of CIDP. Following corticosteroid-pulse therapy and plasmapheresis resulted in good recovery in both motor and sensory impairment, though two-times of relapses were observed. Case 2: a 33-year-old man first noticed weakness in his legs in 1977, motor and sensory disturbances progressed for 12 years. Slowed nerve conduction, high CSF proteins and two-times of relapses in early phase indicated that the CIDP was the diagnosis. In 1989 he complained general fatigue, hyperhidrosis and body-weight loss. The serum thyroid hormone levels were high, and other laboratory studies confirmed the presence of Graves' disease. The cases with both CIDP and Graves' disease has rarely been reported. The background mechanism of this association is not well understood, but the susceptibility to CIDP and Graves' disease may be related to the HLA antigens and immunoglobulin Gm allotypes of which are the genes linked to the major histocompatibility complex and controlling immune responses. The present two cases commonly shared several HLA-DR antigens, but their significance should be confirmed by examining many cases.

Adult↗
PubMed1991

[Local effect of calcitonin gene-related peptide on human sweat gland function].

The effect of local administration of calcitonin gene-related peptide (CGRP) on sweating activity was evaluated on normal human volunteers. CGRP and methacholine chloride (MCH) was dissolved in 0.1 ml of 0.9% NaCl solution to a specified concentration, and was injected intradermally at the center of a 1.3 cm2 forearm test area. The sweat rate was recorded continuously by capacitance hygrometry in a relatively cool environment (Ta, 23 degrees C). CGRP did not elicit any sweat secretion when administrated by alone, but significantly increased the sweat rate when it was administrated with MCH. The maximum enhancement of MCH-induced sweating by CGRP was observed at a concentration of 10(-5) g/ml of CGRP. There was clear dose-dependent relationship between the dose of CGRP and its enhancement. Recently, CGRP-like immunoreactivity is demonstrated to be present in cholinergic nerve terminals around the human sweat glands. These observations have strongly suggested that CGRP enhances the cholinergic sweating activity. Although the underlying mechanism is still obscure, CGRP may enhance the sweating as a consequence of vasodilation which has been known to be a major activity of CGRP. As for the evaluation of human sweat gland function, CGRP-induced peptidergic regulation should be considered as well as cholinergic regulation.

Acetylcholine↗
PubMed1991

[A clinicopathological study of the somatic motor efferents in the pseudopolyneuritic form of amyotrophic lateral sclerosis].

Histopathology of the somatic motor efferents of three cases with pseudopolyneuritic from of amyotrophic lateral sclerosis (ALS) was analyzed, and the results were correlated with the clinical symptoms. The population of myelinated fibers in the lateral corticospinal tract at the thoracic segment, and in the fourth ventral roots, and motor neurons in the fourth ventral horns were morphometrically quantified. The patients were two males and one female ranging in age from 43 to 67 years, and showed a clinical course of 1.8 to 9.5 years. All three cases did not show any pyramidal sign nor spasticity but showed an extensive leg involvement, particularly the distally accentuated muscular weakness with depressed deep tendon reflexes. The clinical manifestations were consistent with the pseudopolyneuritic form of ALS. Fiber size profile of the myelinated fibers in the lateral corticospinal tracts at T7 segments and L4 lumbar ventral roots were estimated on the epon-embedded transverse sections as previously described (Sobue et al, 1981). Number and size distribution of anterior horn cells in the L4 segments were measured on 300-500 consecutive 10 microns-thick paraffin sections stained with Klüver-Barrera technique, using a TGZ-3 particle size analyzer (Zeiss). The location of the remained anterior horn cells was also quantitatively estimated. Three cases with common form of ALS and three control cases were analyzed in the same manner. The large myelinated fibers in the lateral corticospinal tract were extensively and predominantly depleted in all three cases. The small myelinated fibers were also depopulated but in lesser degree. The mode of these myelinated fiber loss was almost compatible with those in common form of ALS.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1990

Nerve growth factor enhances neurite arborization of adult sensory neurons; a study in single-cell culture.

Nerve growth factor (NGF) is a well-established trophic factor of sympathetic and sensory neurons during development. NGF is, however, little known to be required for the maintenance or regulation of differentiated phenotypes of matured peripheral neurons. Since trophic factors, including NGF, are currently known to be secreted by non-neuronal cells, like Schwann cells and fibroblasts, a highly pure-neuron culture is required to assess the direct action of trophic factors on neurons. We have developed a single-neuron culture from neonatal and adult rat dorsal root ganglia in serum-free conditions, and estimated the primary effect of NGF on the morphological geometry of sensory neurons. We found that NGF promoted the neurite length of neonatal sensory neurons, rather than promoting arborization (branching of neurites), while in adult matured neurons NGF significantly enhanced neurite arborizations, rather than the maximal neurite extension, distance from the cell soma to the maximum margin of the territory of neurite extension. Total neurite length, the summed length of all neurites per neuron was significantly increased by NGF in both neonatal and adult neurons. NGF also increased the size of neuronal soma independent of neuronal maturation. Neonatal sensory neurons tended to die in 1 week despite the presence of NGF. In contrast, some adult sensory neurons were alive for more than 2 weeks in the absence of NGF. These results indicate that NGF more than simply accelerates a pre-existing developmental program in the matured stage, and that the promotion of neurite arborization by NGF in adult sensory neurons suggests that NGF may have some role in peripheral nerve regeneration via promotion of axonal sprouting.

Aging↗
PubMed1990

Phosphorylated high molecular weight neurofilament protein in lower motor neurons in amyotrophic lateral sclerosis and other neurodegenerative diseases involving ventral horn cells.

Lower motor neurons of the spinal cord of patients with amyotrophic lateral sclerosis (ALS), Werdnig-Hoffmann's disease (WH), X-linked recessive bulbospinal neuronopathy (X-BSNP) and multiple system atrophy (MSA), all of which were known to involve the lower motor neurons, were immunohistochemically examined by using a monoclonal antibody (Ta-51) specific to phosphorylated epitopes of high molecular weight subunits of neurofilaments. The incidence of Ta-51-positive neurons was significantly increased in ALS, WH and MSA, but not in X-BSNP. Ta-51-positive neurons showed a wide variety of morphological appearances, including neurons with normal appearance, central chromatolysis, simple atrophy and neurons containing massive neurofilamentous accumulation. In aged-control cases, similar Ta-51-positive neurons were observed, although to a much lesser extent. In ALS, spheroids and globules, which were strongly positive for Ta-51, were also significantly increased. Ta-51-positive motor neurons, spheroids and globules appeared in proportional to the number of remaining large motor neurons in ALS.

Adolescent↗
PubMed1990

Type I familial amyloid polyneuropathy. A pathological study of the peripheral nervous system.

The neuropathological changes were examined in 2 cases of type I familial amyloid polyneuropathy (FAP), confirmed by a genetic study with human transthyretin (prealbumin) cDNA. These cases were from different foci of type I FAP in Japan, but showed a similar pathology in the peripheral nerves. Loss of dorsal root and sympathetic ganglion neurons, predominantly those of small size, was prominent, whereas ventral horn cells were well preserved. Distally accentuated axonal loss with marked axonal sprouting was the principal feature. Fibre sprouts were ubiquitous throughout the nerves and affected the fibre size distribution. Segmental demyelination and remyelination were prominent in the proximal portions of nerves, but axonal degeneration was more conspicuous in the distal portions. The centrally-directed branches of the primary sensory neurons did not show distally-accentuated axonal loss in the dorsal columns. Amyloid deposits were present universally in the endoneurial spaces of the peripheral nerves, but more prominently in the dorsal root ganglia, sympathetic ganglia and more proximal portions of the nerves, and the distribution correlated well with the occurrence of the pathology of peripheral nerves. Neurofilamentous accumulation was frequent in the proximal axons and neuronal cell bodies of the sensory and sympathetic neurons. Schwann cells and satellite cells to which amyloid deposits were attached frequently showed disappearance of basement membrane and proliferation of distorted processes. The findings in the present cases suggest that the Schwann and satellite cells may be directly affected by the amyloid deposits, but the pathogenetic mechanism of marked axonal and neuronal involvement still remains to be elucidated.

Adult↗
PubMed1990

[The role of Schwann cells in peripheral nerve degeneration and regeneration--NGF-NGF receptor system].

In a wide variety of peripheral neuropathies, Schwann cells are known to be involved. However, it is still obscure whether Schwann cells play a role in the pathogenesis of nerve degeneration or are only secondarily involved in many neuropathies. Since Schwann cell culture technique is introduced, a number of Schwann cell functions have been clarified, and most of these functions are now believed to be related more with the peripheral nerve regeneration rather than degeneration. When Schwann cells are released from axonal contact, they express NGF receptor on the surface and also secrete NGF. These NGF receptor expression and NGF secretion by Schwann cells also extensively occur in the nerves undergoing active degeneration, and subside again when nerve regeneration is completed. NGF is actually a potent modulator for increasing the neurite sprouts from adult rat DRG neuron in culture. Taking account of these evidences, I discussed the role of NGF-NGF receptor system in the peripheral neuropathies.

Animals↗
PubMed1990

[Spatial distribution of the peripheral nerve lesion in polyarteritis nodosa].

We have examined the peripheral nerves in four patients with polyarteritis nodosa. They were consisted of two males and two females with the age of 70, 49, 65 and 75 years respectively. The sciatic and posterior-tibial nerves, sural nerves, ventral and dorsal spinal nerve roots, dorsal ganglia and spinal cord were removed at autopsy. Particularly, the sciatic and posterior-tibial nerves were removed as a whole and suspended in a glass cylinder with a weight and fixed in 1.5% glutaraldehyde and 0.05 M phosphate buffer, pH 7.4 for 16 hours. The nerve fragments of every 3 to 3.5 cm along the sciatic and posterior-tibial nerve were examined on conventional paraffin-embedded and epon-embedded sections, and osmicated teased-fibers. Necrotizing angitis was commonly present in the epineurium of the sciatic and posterior-tibial nerves in all four patients. Although necrotizing angitis was diffusely distributed in the proximal to distal portions of the nerve, loss of myelinated fibers, occurred only in the distal to mid-lower portions of the sciatic nerve. There was no substantial myelinated fiber loss in the proximal part of the sciatic nerve in three of four cases. Myelinated fiber loss in the fascicle was central fascicular or multiple-focal in pattern in the proximal portions of the nerve, but was diffuse in more distal portion of the posterior tibial nerve. Segmental demyelination and myelin irregularity in the teased-fiber preparation were more prominently observed in the proximal portions, but fibers with axonal degeneration were more frequent in the distal portions of the nerve.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1990

[Autonomic dysfunction in vasculitic neuropathy--special reference to sudomotor function].

We examined autonomic functions in 14 patients with peripheral neuropathy caused by necrotizing vasculitis. These patients consisted of three allergic granulomatous angitis (Churg-Strauss syndrome, AGA), two systemic lupus erythematosus (SLE), two progressive systemic sclerosis (PSS), one mixed connective tissue disease (MCTD), one polyarteritis nodosa (PN) and five nonsystemic vasculitis. All of them were proven to have a vasculitis by sural nerve, muscle or skin biopsy. Sixteen age- and sex-matched healthy volunteers were also examined. Local sweating induced by intradermal injection of pilocarpine and nicotine (a concentration of 10(-4) g/dl, 0.1 ml) was measured with ventilated capsular method on the forearm and lower lateral leg at 23 degrees C of room temperature and 40% of relative humidity. Other autonomic functions including skin temperature at rest and after cold loading (15 degrees C, 6 minutes), variation in the R-R interval of heart beat (CV%), orthostatic hypotension and bladder dysfunction were also monitored. A decrease in sweat rate and recovery rate of skin temperature after cold-loading was seen more frequently in patients with necrotizing vasculitis than normal volunteers. Abnormality in the local sweat response against nicotine and pilocarpine was more frequently present in the involved area of somato-sensory and motor nerves as compared with those in the non-involved area. An occurrence of decrease in recovery rate of skin temperature after cold-loading also well correlated to the region of somato-sensory- and motor involvement. So far other autonomic dysfunction, only one patient had orthostatic hypotension, impotence and bladder dysfunction.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

Low uptake of [3H]2-deoxy-D-glucose by cultured rat Schwann cells.

[3H]2-Deoxy-D-glucose (2-DG) was used to investigate the glucose uptake in cultured rat Schwann cells from postnatal Sprague-Dawley rat sciatic nerves. The glucose uptake of Schwann cells slightly increased in a time- and dose-dependent manner. However, the maximal uptake level was much lower than that of ethylnitrosourea (ENU)-induced transformed rat schwannoma-like cells and fibroblasts. By autoradiography of the cultured system, we were able to visualize the accumulation of [3H]2-DG grains in the schwannoma-like cells and fibroblasts, but not in Schwann cells.

Animals↗
PubMed1989

Nerve growth factor receptor immunoreactivity in human benign peripheral nerve sheath tumor.

In situ expression of nerve growth factor (NGF) receptors in human dermal and plexiform neurofibroma, schwannoma and traumatic neuroma was examined by an immunohistochemical method using a monoclonal anti-human NGF receptor antibody. Immunoreactivity for the NGF receptor was observed on the principal cells of both neurofibroma and schwannoma. Immunostaining by the anti-S-100 beta protein antibody in adjacent sections suggested that the vast majority of NGF receptor-positive cells were also positive for S-100 beta protein. In traumatic neuroma, staining for the NGF receptor was more intense in the perineurium than in the endoneurial cells.

Adult↗
PubMed1989

X-linked recessive bulbospinal neuronopathy. A clinicopathological study.

A clinicopathological study on X-linked recessive bulbospinal neuronopathy was undertaken on 9 cases, with morphological observations on 3 autopsied cases and sural nerve biopsies from 6 patients. Both lower motor and primary sensory neurons were involved. Lower motor neurons were markedly depleted through all spinal segments and in brainstem motor nuclei except for the third, fourth and sixth cranial nerves. Primary sensory neurons were less severely affected. A quantitative study of primary sensory axons at several levels in the peripheral nervous system suggested that a distally accentuated axonopathy was the salient pathological process. Segmental demyelination and remyelination clustered on individual fibres, and g ratios (axon diameter: total fibre diameter) in the sural nerve showed an increased scatter in some cases. Evidence of regeneration was inconspicuous. Unmyelinated fibres were well preserved throughout all the nerves examined. Neurons in the Onufrowicz nuclei, in the intermediolateral columns and in Clarke's columns of the spinal cord were generally well preserved. These observations indicate that a lower motor and primary sensory neuronopathy is a major neurological manifestation in this disease.

Aged↗
PubMed1989

Nerve growth factor receptor immunoreactivity in the neuronal perikarya of human sensory and sympathetic nerve ganglia.

We examined immunohistochemically the dorsal root ganglia, sympathetic ganglia, spinal cord, ventral and dorsal roots, and sciatic nerves obtained at autopsy from adult humans, using a monoclonal antibody against the human nerve growth factor receptor. We observed labelling in a granular pattern in the neuronal perikarya of dorsal root and sympathetic nerve ganglia. Ventral horn cells and axons were not labelled.

Antibodies, Monoclonal↗
PubMed1989

Myoinositol inhibits proliferation of cultured Schwann cells: evidence for neurotoxicity of myoinositol.

To determine whether so-called uraemic toxins exhibit direct toxic effects on Schwann cells, the latter were cultured in a monolayer with the addition of uraemic toxins. Proliferation of Schwann cells was estimated with [3H]-thymidine incorporation into DNA synthesis. Myoinositol, methylguanidine, guanidinosuccinic acid, parathyroid hormone, phenol, p-cresol, 4-hydroxyphenylacetic acid, 4-hydroxybenzoic acid, and 3-carboxy-4-methyl-5-propyl-2-furanpropionic acid were added at various concentrations to the culture medium. Addition of myoinositol at concentrations between 50 and 100 micrograms/ml, values analogous to those in in a dose-dependent way. Myoinositol also inhibited axolemma-stimulated proliferation of Schwann cells. The other compounds at concentrations occurring in uraemic serum did not show any toxic effect on Schwann cells. These results suggest that myoinositol has a neurotoxic effect on Schwann cells, and uraemic polyneuropathy seemed to be caused by a defect in maintaining normal myelin due to accumulation of myoinositol.

Animals↗
PubMed1989

[Migrating multiple mononeuritis and nonsystemic angitis].

Multiple mononeuritis of migrating nature at onset occurs in a variety of disease conditions. "Migrant sensory neuritis" without a systemic underlying disorder described by Wartenberg (1958) is the most distinctive form of this type of neuropathy. Its pathomechanism is not uniform, but angiopathy has been suggested by Matthews et al. (1981). In this report, we describe five cases of sensory-dominant multiple mononeuritis with migrating nature and without systemic visceral involvement. The patients consisted of four females and one male between 26 and 71 years of age. All showed recurrent episodes of sensory involvement along the distal branches of the cutaneous nerve. The patients presented with sudden onset of numbness or pain radiating along the cutaneous branches of the involved nerves often followed by persistent sensory deficit. In mild episodes, the sensory symptom with numbness almost completely subsided. The frequency of these episodes ranged from 7 times in 6 months to 6 times in 9 years. The clinical manifestations were similar or identical to those of migrant sensory neuritis reported by Watenberg and Matthews et al. Cranial nerve involvement and less frequent episodes in the present series differed from those in the previous reports. Laboratory examinations did not disclose any underlying disorder, suggesting systemic collagen vascular disease. Sural nerve biopsy study in two patients revealed vasculitis in small or medium-sized arteries in the epineurium as well as reduced population of large myelinated fibers. Small myelinated fibers were slightly increased in number, probably to regenerating fibers. Sensory action potentials were low or not evoked in the nerves examined.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

[Expression of nerve growth factor receptor in human benign peripheral nerve sheath tumor].

We examined in vivo and in vitro expression of nerve growth factor (NGF) receptors in 13 dermal and 9 plexiform neurofibromas, 28 schwannomas, and 4 traumatic neuromas with an immunohistochemical method using a monoclonal antihuman NGF receptor antibody (ME20-4) and anti S-100 beta protein antibody. Immunoreactivity for NGF receptor and S-100 beta protein was universally observed on the principal cells of both neurofibroma and schwannoma in vivo. Moreover, we examined the NGF receptor and S-100 beta protein immunoreactivity in the culture system of the neurofibroma and schwannoma with an immunofluorescent double staining method. The principal cells of both tumors were positively stained by both ME20-4 and anti S-100 beta protein antibodies. These cells also express the ability for 125I-NGF binding, which was examined with an autoradiographic technique. These results suggest that NGF receptors are universally expressed on the S-100 beta protein positive Schwann-like cells composing of these tumors both in vivo and in vitro. In traumatic neuroma, however, a positive staining of NGF receptor was more strongly observed in the perineurium rather than in the endoneurial cells.

Adolescent↗