Results 1 to 10 of about 341,259 (167)

Spinocerebellar degenerations: An update

Current Neurology and Neuroscience Reports, 2002
Over the past decade, the spinocerebellar degenerations have gone from a diverse group of loosely defined phenotypes to a family of diseases with many identifiable genotypes and the promise of gene-specific treatments. The evaluation of the spinocerebellar ataxias has been simplified, and the counseling of patients and families has been enhanced by the
Susan Perlman
exaly   +4 more sources

Peripheral neuropathy in spinocerebellar degenerations

Muscle and Nerve, 1981
AbstractNerve conduction studies have been performed in 19 subjects with hereditary spinocerebellar degenerations other than Friedreich ataxia. Clinically, they may be classified as olivopontocerebellar atrophy or cerebello‐olivary degeneration. In 9 patients, sensory conduction was abnormal, and in the whole group there was a significant impariment of
J G, McLeod, W A, Evans
exaly   +4 more sources

Mitochondrial dysfunction and spinocerebellar degenerations

Neurochemical Pathology, 1986
A simplified classification of the spinocerebellar degenerations is proposed. Axonal ataxias include Friedreich's ataxia and other conditions involving, primarily, neurons with very long axons. Multiple system degenerations include the various olivopontocerebellar atrophies and related disorders.
Jesse M Cedarbaum, John P Blass, J P Blass   +2 more
exaly   +4 more sources

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 Pieter Kark
exaly   +4 more sources

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
Kohei Ota, Yamaguchi Tomoko
exaly   +4 more sources

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).
Dr. R. Sridharan
exaly   +4 more sources

Spinocerebellar degenerations

Expert Opinion on Pharmacotherapy, 2003
The spinocerebellar ataxias (SCA) are a large group of inherited disorders affecting the cerebellum and its afferent and efferent pathways. Their hallmark symptom is slowly progressive, symmetrical, midline, and appendicular ataxia. Some may also have associated hyperkinetic movements (chorea, dystonia, myoclonus, postural/action tremor, restless legs,
S. Perlman
semanticscholar   +5 more sources

Differentiation Between Multiple System Atrophy and Other Spinocerebellar Degenerations Using Diffusion Kurtosis Imaging

Academic Radiology, 2019
RATIONALE AND OBJECTIVE Differentiation between multiple system atrophy (MSA) and other spinocerebellar degenerations showing cerebellar ataxia is often difficult.
Kenji Itô, Kunihiro Yoshioka, Tetsuya Maeda   +2 more
exaly   +2 more sources

Spinocerebellar degenerations

Indian Journal of Pediatrics, 1962
A brief review of spinocerebellar degenerations as seen in pediatric practive with four illustrative case reports is presented.
O P Ghai, Chandra R K, Ghai O P
exaly   +3 more sources

PREVALENCE AND PATTERN OF SPINOCEREBELLAR DEGENERATIONS IN NORTHEASTERN LIBYA

Brain, 1985
Kurupath Radhakrishnan, P P Ashok, Sridharan R   +2 more
exaly   +2 more sources