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Biomedical subjects

G Sobue

Publications and source records attributed to G Sobue.

At least 361 records · Page 20Linked to original sources

Spinal and cranial motor nerve roots in amyotrophic lateral sclerosis and X-linked recessive bulbospinal muscular atrophy: morphometric and teased-fiber study.

Amyotrophic lateral sclerosis (ALS) and adult onset X-linked recessive bulbospinal muscular atrophy (SPMA), constituting the category of adult onset form of motor neuron disease, were analyzed on motor nerve roots. The results of morphometric analysis on ventral spinal roots (VSR) of all spinal segments from ALS and SPMA revealed the following three findings: (1) the large-myelinated alpha-motoneuron fibers were markedly decreased in number throughout all segments; (2) thin-myelinated autonomic preganglionic fibers were almost completely preserved; (3) small-intermediate-myelinated fibers which are considered to correspond to gamma-motoneuron fibers were generally well preserved in ALS, but decreased by one-half to one-third in SPMA. However, all the components of the nerve roots of the oculomotor, trochlear, and abducent nerves were completely preserved in both ALS and SPMA. Moreover, the teased-fiber study showed that the regenerating-sprouting process rarely occurred in the VSR of ALS and SPMA. The present study suggested that the site of the primary lesion seems to be in the alpha-motoneuron fibers in motor neuron diseases, such as ALS or SPMA. However, the marked discrepancy in the pathologic change in the alpha-motoneuron fibers in the VSR and the nerve roots innervating the external ocular muscles was noteworthy.

Amyotrophic Lateral Sclerosis↗

Pathology of myelinated fibers in cervical and lumbar ventral spinal roots in amyotrophic lateral sclerosis.

Pathological alterations were evaluated by morphometry and by a teased-fiber study on the 6th cervical (C6) and the 4th lumbar (L4) ventral spinal roots of cases of amyotrophic lateral sclerosis (ALS). The large-diameter fibers were severely affected in both spinal segments. However, small-diameter myelinated fibers were numerically well preserved. The number of large fibers in C6 and L4 ventral roots was strongly correlated to the strength of muscles innervated by C6 or L4 segments. There was no correlation of the number of small fibers with muscle strength. Teased fiber studies revealed a marked increase in the incidence of fibers showing axonal degeneration. Fibers considered to be regenerative were rarely observed. These observations suggest that large myelinated fibers, which correspond to alpha-motoneuron fibers, are selectively affected, and that small myelinated fibers, which are considered to correspond to gamma-motoneuron fibers, are preserved to some extent in the C6 and L4 ventral spinal roots in ALS.

Amyotrophic Lateral Sclerosis↗

Peripheral neuropathy in Marinesco-Sjögren syndrome.

A case of Marinesco-Sjögren syndrome manifesting cataract, short stature, psychomotor retardation, hypogonadism, and progressive ataxia was described. The result of electrophysiological study reflected the presence of peripheral nerve involvement, which was clearly confirmed by sural nerve biopsy. The conspicuous abnormality observed in the peripheral nerve was the presence of segmental demyelination. The process of axonal degeneration was not remarkable. Results confirmed that Marinesco-Sjögren syndrome is a disorder which may involve both central and peripheral nervous system.

Adolescent↗

Alterations of lipid and protein composition in myelin of acute experimental allergic neuritis.

Lipid and protein components of the myelin isolated from the spinal roots of rabbits at the acute stage of experimental allergic neuritis (EAN) and of control rabbits were analyzed. The results obtained from the EAN myelin in comparison with the control myelin were as follows: (1) The content of cholesterol increased and that of cerebroside significantly decreased, while the content of plasmalogen and phospholipid was not changed. The level of cholesterol ester was negligible both in EAN and control myelin. (2) The sum of saturated fatty acids increased while the sum of unsaturated fatty acids decreased. (3) The decrease of the basic proteins P1 and P2 was more noticeable than that of the PO + Y protein. Concomitant to these observations, with 1,6-diphenyl 1,3,5-hexatriene as a fluorescent probe, the microviscosity of the isolated myelin was measured. The results indicated that myelin obtained from EAN was less fluid. Diminished myelin fluidity in EAN was consistent with the results of the lipid and fatty acid analyses.

Acute Disease↗

Asialoglycoproteinemia in a case of primary hepatic cancer.

This article reports on a case with the most unusual finding of asialoglycoproteinemia. The patient was a man aged 40 years suffering from primary hepatic cancer. Among the various serum glycoproteins tested, the concentration of the desialylated form of alpha 2-HSglycoprotein, transferrin and alpha 2-antitrypsin was markedly elevated. The asialoprotein/sialoprotein ratio varied with each glycoprotein. In the case of transferrin, the ratio changed considerably in the course of the disease, and was found to be clearly related to the change in size of the hepatic tumor.

Asialoglycoproteins↗

Regulation of Schwann cell nerve growth factor receptor by cyclic adenosine 3',5'-monophosphate.

Previous studies indicated that Schwann cells in immature nerves express nerve growth factor (NGF) receptors, and that this expression is down regulated during development but re-induced by Wallerian degeneration. It was also shown that immature Schwann cells are induced to express galactocerebroside and other molecules characteristic of mature Schwann cells by either contact with an axon or treatment with the cyclic adenosine 3',5'-monophosphate (cAMP) analogues dibutyryl cAMP (dbcAMP) and 8-bromo cAMP or the adenylate cyclase activator forskolin. In the present study, NGF receptors on the surface of cultured Schwann cells were demonstrated by binding of an anti-rat NGF receptor monoclonal antibody or of radioiodinated NGF. Treatment of cultured Schwann cells with cAMP analogues or forskolin resulted in a progressive decrease in both immunoreactive NGF receptors and radioiodinated NGF binding. The cultured Schwann cells contained a polyadenylated RNA species homologous with human melanoma NGF receptor mRNA in sequence and size. The amount of this NGF mRNA was lower in cAMP analogue-treated than in untreated Schwann cells.

8-Bromo Cyclic Adenosine Monophosphate↗

Geometric response to nerve growth factor is preserved in aged rat sensory neurons: a single-neuron culture study.

We previously established a single-neuron culture system to analyze the primary effects of neurotrophic factors and reported that NGF promoted neurite extension in young adult (4- to 6-month-old) rat dorsal root ganglion (DRG) neurons by promoting neurite arborization. In this study, we demonstrated that the effects of NGF on neurite regeneration in DRG neurons was well preserved in aged rats (20- to 24-month-old and 33-month-old). NGF did not increase the percent process-bearing neurons in aged rats, which indicated that neuronal survival was not promoted by NGF, but it significantly enhanced the number of branching points, total neurite length, and soma size in aged neurons. These effects of NGF on neurite geometry tended to be reduced to some extent in aged neurons and the initiation of neurite-outgrowth in aged neurons was also delayed as compared with young adult neurons. NGF-responsive subpopulation of neurons, found in the entire range of neuronal size, were preserved in aged rats. These findings indicate that NGF could play an important role in regeneration of injured DRG neurons of aged animals.

Aging↗

Epidemiology of acute childhood encephalitis. Aichi Prefecture, Japan, 1984-90.

The case records for 256 patients with acute encephalitis, including meningoencephalitis, acute encephalopathy and Reye syndrome, were obtained from 36 departments of pediatrics in large hospitals through questionnaires which were sent in 1990-92. The incidence rate per 100,000 child years was 3.3, and was more frequent in children aged 0-4 years (rate 6.6) than 5-15 years (rate 2.0), with statistical significance. The disease in the 105 etiologically diagnosed patients was due to measles virus (24), herpes simplex (21), rubella (24), and other agents (36), including two cases of human herpesvirus 6. A third of the infants with measles encephalitis were first seen at less than 18 months of age. Eighteen cases of rubella encephalitis occurred during an epidemic of rubella in Aichi Prefecture, 1987-88. The short-term outcome (mean length of follow-up: 2 years 3 months) of encephalitis was death in 20 cases (7.8%) and sequelae in 58 (24%). Eleven patients died within 7 days and five by the 8-14th day from onset. Thirty-six children had multiple residual impairments, including twelve who were severely handicapped. The prognosis for both life and sequelae was significantly poor for herpes simplex encephalitis in etiologically diagnosed patients. We stress the importance of an increase in the vaccination rate and the establishment of an early diagnostic system.

Adolescent↗