Supratentorial atrophy in spinocerebellar ataxia type 2 : MRI study of 20 patients

There have been only few studies of brain magnetic resonance imaging (MRI) in spinocerebellar ataxia (SCA) type 2. We investigated 20 SCA2 patients, from 11 Sicilian families, and 20 age-matched control subjects using MRI. Our data confirm that olivopontocerebellar atrophy (OPCA) is the typical patt...

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Bibliographic Details
Published in:Journal of neurology 1999-05, Vol.246 (5), p.383-388
Main Authors: GIUFFRIDA, S, SAPONARA, R, RESTIVO, D. A, TROVATO SALINARO, A, TOMARCHIO, L, PUGLIARES, P, FABBRI, G, MACCAGNANO, C
Format: Article
Language:English
Subjects:
Adult
Aged
Atrophy
Biological and medical sciences
Cerebellum - pathology
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Humans
Magnetic Resonance Imaging
Male
Medical sciences
Middle Aged
Neurology
Olivopontocerebellar Atrophies - diagnosis
Olivopontocerebellar Atrophies - etiology
Repetitive Sequences, Nucleic Acid
Spinocerebellar Degenerations - complications
Spinocerebellar Degenerations - diagnosis
Spinocerebellar Degenerations - genetics
Spinocerebellar Degenerations - pathology
Time Factors
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Summary:There have been only few studies of brain magnetic resonance imaging (MRI) in spinocerebellar ataxia (SCA) type 2. We investigated 20 SCA2 patients, from 11 Sicilian families, and 20 age-matched control subjects using MRI. Our data confirm that olivopontocerebellar atrophy (OPCA) is the typical pattern in SCA2. We found no significant correlation between infratentorial atrophy, disease duration, or the number of CAG repeats in our SCA2 patients, but there was supratentorial atrophy in 12 patients, with a significant correlation between supratentorial atrophy and disease duration. OPCA appears to represent the "core" of the SCA2: however, central nervous system involvement is not limited to pontocerebellar structures. We therefore consider central nervous system degeneration in SCA2 as a widespread atrophy. MRI is helpful in diagnosing SCA, but it is not diagnostic in the absence of clinical and molecular studies. We suggest that serial MRI may play a role in evaluating "in vivo" the progressive steps of neurodegeneration in SCA2, for a better comprehension of the pathophysiology of this disorder.
ISSN:0340-5354
1432-1459
DOI:10.1007/s004150050368