Results 161 to 170 of about 2,684 (177)

Pyruvate dehydrogenase deficiency in spinocerebellar degenerations

Neurology, 1979
To study the incidence of abnormalities of the pyruvate (PDH) or ketoglutarate (KGDH) dehydrogenase complexes in patients with spinocerebellar degenerations, we measured the activities of PDH and KGDH in platelet-enriched preparations from the blood of 14 patients. Low PDH was found in 6 of the 14 patients; low KGDH was found in 2 of the 6.
R A, Kark, M, Rodriguez-Budelli
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Glutamate dehydrogenase deficiency in spinocerebellar degenerations

Neurochemical Research, 1982
Glutamate dehydrogenase (GDH) activity in leukocytes and platelets in spinocerebellar degenerations (SCD) was determined. In the same subject, GDH activity was higher and more reproducible in platelets than in leukocytes. GDH was decreased significantly in olivopontocerebellar atrophy (OPCA) (Ca. 30% decrease). Pyruvate dehydrogenase (PDH) in platelets
T, Yamaguchi, K, Hayashi, H, Murakami, K, Ota, S, Maruyama   +4 more
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Spinocerebellar degeneration

Neurology, 1979
Two adult sisters had spinocerebellar degeneration. Biochemical studies revealed a very low activity of both fraction A and fraction B of the lysosomal enzyme, hexosaminidase, in serum and leukocytes. A skin biopsy showed lesions suggestive of neuronal storage disease. The disorder seems to be an adult form of GM2 gangliosidosis.
J G, Oonk, H J, van der Helm, J J, Martin   +2 more
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Visual evoked potentials in spinocerebellar degenerations

Clinical Neurology and Neurosurgery, 1983
Pattern reversal visual evoked potentials were studied in 21 patients with spinocerebellar ataxias among whom 6 had Friedreich's ataxia, 10 had hereditary spastic ataxia and 5 had spinocerebellar degeneration with slow eye movements (olivopontocerebellar degeneration).
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[Memory dysfunction of spinocerebellar degeneration].

Rinsho shinkeigaku = Clinical neurology, 1990
We evaluated Rey's auditory verbal learning test (AVLT) and Wechsler adult intelligence scale (WAIS) in 29 patients with spinocerebellar degeneration (SCD) younger than 65 years old. They showed a significant impairment in the recall subtest of AVLT. However in the recognition subtest, there was no significant impairment in the recall subtest of AVLT ...
N, Hirono, A, Yamadori, M, Kameyama, T, Mezaki, K, Abe   +4 more
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Familial Adult-Onset Spinocerebellar Degenerations

2000
The familial spinocerebellar degenerations are a heterogeneous group of disorders with onset in both childhood and adulthood. Their prevalence is estimated to be between 1.5 and 22.1 per 100,000. Although pathologic and clinical classification systems have been proposed in the past, all have had shortcomings. In the past several years, great strides in
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TRH AND SPINOCEREBELLAR DEGENERATION

The Lancet, 1982
PeterA. Lewitt, JoelR.L. Ehrenkranz
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[Spinocerebellar degeneration].

Ryoikibetsu shokogun shirizu, 1997
Y, Iwasaki, M, Kinoshita, T, Shiojima
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[Neurochemical aspects of spinocerebellar degeneration].

Rinsho shinkeigaku = Clinical neurology, 1994
Spinocerebellar degeneration (SCD) is one of the most frequent neurodegenerative disorders in Japan. In spite of this, only a little attention has been paid on the disease, especially from the biochemical point of view. In this symposium, I would like to briefly review the fruits of biochemical researches in Japan.
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[Spinocerebellar degeneration].

Nihon rinsho. Japanese journal of clinical medicine, 2002
I, Yabe, H, Sasaki, K, Tashiro
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