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

Salvatore Giuffrida, Riccardo Saponara, Domenico A. Restivo, Angela Trovato Salinaro, Letizia Tomarchio, Pietro Pugliares, Giuseppe Fabbri, Carmelo Maccagnano

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)383-388
Number of pages6
JournalJournal of Neurology
Volume246
Issue number5
DOIs
Publication statusPublished - 1999

Keywords

  • Cerebral atrophy
  • Disease duration
  • Magnetic resonance imaging
  • Spinocerebellar ataxia type 2

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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