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Biomedical subjects

I Sobue

Publications and source records attributed to I Sobue.

At least 109 records · Page 6Linked to original sources
PubMed1981

Ophthalmoplegia-plus. Its occurrence with periventricular diffuse low density on computed tomography scan.

Familial cases of progressive external ophthalmoplegia, deafness, generalized weakness, and hypogonadism were studied. A muscle biopsy specimen showed increased amounts of glycogen particles, lipid droplets, and mitochondria that frequently contained paracrystalline inclusion bodies between intramitochondrial and extramitochondrial membranes. Involvement of the CNS was suspected from a computed tomography scan that revealed diffuse, low-density deep cerebral white matter. Therapy with corticosteroids (prednisolone) was effective for recovery of muscular strength in the extremities. Possible involvement of the CNS in ophthalmoplegia-plus might be related to an abnormal metabolism of mitochondria.

Adolescent↗
PubMed1981

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↗
PubMed1981

Experimental ethambutol neuropathy in rats. Morphometric and teased-fiber studies.

Eight rats were given 500 mg/kg body weight of the antituberculous drug ethambutol orally every day for 3 months (group A), and 7 took 150 mg/kg of the drug for 9 months (group B). The sciatic nerves at midthigh level and the posterior tibial nerves at ankle level were studied with morphometric examination and teased-fiber method. The number of myelinated fiber/mm(2) of fascicular area was significantly reduced in both sciatic and posterior tibial nerves of group B. The pattern of frequency distribution of myelinated nerve fiber diameters of both groups was not different from that of the controls. Teased-fiber study revealed that many fibers were undergoing axonal degeneration in both groups A and B. In addition, regenerating fibers after axonal degeneration were observed in group B. These results showed that the predominant pathologic change in ethambutol neuropathy was axonal degeneration and that regeneration was already occurring in the animals taking a small dose of the drug for a long period. The fact that the degree of pathologic change was rather more severe in the sciatic nerve than in the posterior tibial nerve indicated that this was not a "dying-back" neuropathy.

Animals↗
PubMed1981

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↗
PubMed1980

Genetic and clinical patterns of heritable cerebellar ataxias in adults. I. Genetic analyses.

Family data on 299 probands with spinocerebellar types, 163 with late cortical cerebellar atrophies (LCCA), and 180 with olivopontocerebellar atrophies (OPCA) were analysed. Spinocerebellar types included cases with cerebellar ataxia combined with spasticity. Their family history was not compatible with sex linked inheritance. There were few recessive cases occurring before 24 years of age. The majority of cases were products of non-consanguineous marriages in which one partner was affected. The genetic ratio was 0.5 for each decade at onset between 20 and 59, suggesting that these cases were dominant. The proportion of these familial cases, however, decreased with increasing age at onset, and there were quite a few sporadic cases among the elderly. LCCA and OPCA were considered to represent extremes of a correlated disease spectrum. Although they were weakly familial, single gene heredity was rejected. Sib recurrence rate was 7.5% for LCCA and 9.4% for OPCA. When calculated for different age groups at onset, the rates decreased with increasing age in the two diseases. The rates increased to 25% and 20%, respectively, given two sibs already affected, and to 37.5% and 22.2% when three were affected. These patterns were incompatible with single gene mechanisms and were similar to those in multifactorial diseases with a threshold effect.

Adolescent↗