Results 151 to 160 of about 2,684 (177)
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Adrenoleukomyelo‐neuropathy presenting as spinocerebellar degeneration
Neurology, 1982A spinocerebellar syndrome has been described rarely in adrenoleukodystrophy. We report a family containing three cases of typical adrenoleukodystrophy, and one patient with a slowly progressive spinocerebellar syndrome. A high index of suspicion and the ACTH stimulation test are required when dealing with multisystem neurologic disease of unknown ...
C D, Marsden, J A, Obeso, A E, Lang
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Journal of Computer Assisted Tomography, 1988
Magnetic resonance imaging at 1.5 T was performed in 27 patients with either the cerebellar or spinocerebellar form of spinocerebellar degeneration and in 10 control subjects. Neither T1- nor T2-weighted images (T1WIs and T2WIs) of the patients showed any abnormal intensity areas within the cerebellum or in any other structures of the brain.
H, Nabatame +5 more
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Magnetic resonance imaging at 1.5 T was performed in 27 patients with either the cerebellar or spinocerebellar form of spinocerebellar degeneration and in 10 control subjects. Neither T1- nor T2-weighted images (T1WIs and T2WIs) of the patients showed any abnormal intensity areas within the cerebellum or in any other structures of the brain.
H, Nabatame +5 more
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Spinocerebellar degenerations: An update
Current Neurology and Neuroscience Reports, 2002Over 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
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Journal of the Medical Association of Thailand = Chotmaihet thangphaet, 1991
Forty cases with several forms of spinocerebellar degenerations were studied. The diagnosis was based on an appropriate clinical picture, radiological investigation and family history. There are multiple variants of spinocerebellar degeneration and classification of these diseases remains unsatisfactory and therefore controversial.
K, Phanthumchinda, A, Srikiatkachorn
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Forty cases with several forms of spinocerebellar degenerations were studied. The diagnosis was based on an appropriate clinical picture, radiological investigation and family history. There are multiple variants of spinocerebellar degeneration and classification of these diseases remains unsatisfactory and therefore controversial.
K, Phanthumchinda, A, Srikiatkachorn
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Familial spinocerebellar degeneration with corneal dystrophy
American Journal of Medical Genetics, 1985AbstractWe report on two sisters born to normal but consanguineous parents, with the unusual combination of spinocerebellar degeneration and corneal dystrophy. Their manifestations include mental subnormality, bilateral corneal opacification starting in the second year of life and leading to severe visual impairment, and slowly progressive cerebellar ...
Deeb, Mary E. +9 more
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Mitochondrial hydroxyapatite deposits in spinocerebellar degeneration
Annals of Neurology, 1987AbstractWe report the presence of crystalline deposits of calcium hydroxyapatite in the mitochondria of 2 children with sporadic spinocerebellar degeneration. The deposits, identified by electron microscopy, were found in the mitochondria of neurons and smooth muscle cells in one patient and in only smooth muscle cells in the second child, but not in ...
D G, Munoz, E S, Emery, R A, Highland
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[Treatment for Spinocerebellar Degeneration].
Brain and nerve = Shinkei kenkyu no shinpo, 2023No disease-modifying therapy has been established for spinocerebellar degeneration and multiple system atrophy, and only symptomatic therapy is currently available. Taltirelin and protirelin are drugs covered by health insurance for cerebellar ataxia symptoms, and are expected to suppress the progression of symptoms.
Masaaki, Matsushima, Ichiro, Yabe
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Nystagmus-sensation Dissociation in Spinocerebellar Degeneration
Acta Oto-Laryngologica, 2003To study nystagmus-sensation dissociation (NSD), i.e. caloric nystagmus without the sensation of vertigo, in patients with spinocerebellar degeneration (SCD).The neuro-otological and neuro-radiological records of 179 patients clinically diagnosed as having SCD (91 males, 88 females; age range 20-89 years) and 48 patients diagnosed as having peripheral ...
Yasuko, Ishibashi +4 more
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Ataxia - spinocerebellar degeneration
Neurologia Croatica. Supplement, 2013Izlaganje je posvećeno klasifikaciji, kliničkoj prezentaciji i molekularnoj patogenezi najučestalijih ataksija uz uvid u terapijske pristupe.
Miletić, Vladimir, Relja, Maja
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Tapetoretinal Degenerations in Spinocerebellar Degenerations (Heredoataxias)
Acta geneticae medicae et gemellologiae, 1974The association of hereditary spino-ponto-cerebellar degenerations with tapetoretinal degenerations of varying types, and particularly with Stargardt's macular degeneration, is not rare, as at the present time more than 200 cases are known. Therefore the one-gene hypothesis seems to be the most plausible.
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