Journal of Neurology

, Volume 246, Issue 5, pp 383–388

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
Original communication
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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.

Key words Spinocerebellar ataxia type 2 Magnetic resonance imaging Cerebral atrophy Disease duration 

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Copyright information

© Steinkopff Verlag 1999

Authors and Affiliations

  • Salvatore Giuffrida
    • 1
  • Riccardo Saponara
    • 1
  • Domenico A. Restivo
    • 1
  • Angela Trovato Salinaro
    • 2
  • Letizia Tomarchio
    • 3
  • Pietro Pugliares
    • 1
  • Giuseppe Fabbri
    • 4
  • Carmelo Maccagnano
    • 4
  1. 1.Istituto di Scienze Neurologiche, Università di Catania, ItalyIT
  2. 2.Istituto di Biochimica, Università di Catania, ItalyIT
  3. 3.Istituto di Radiologia, Università di Catania, ItalyIT
  4. 4.Servizio di Radiologia, Ospedale Garibaldi, Catania, ItalyIT
  5. 5.Istituto di Scienze Neurologiche Università di Catania, Viale A. Doria 6, I-95125 Catania, Italy Fax: +39-095-330943 Tel.: +39-095-256770IT

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