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 language | English |
|---|---|
| Pages (from-to) | 383-388 |
| Number of pages | 6 |
| Journal | Journal of Neurology |
| Volume | 246 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - 1999 |
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Keywords
- Cerebral atrophy
- Disease duration
- Magnetic resonance imaging
- Spinocerebellar ataxia type 2
ASJC Scopus subject areas
- Clinical Neurology
- Neurology
Cite this
Supratentorial atrophy in spinocerebellar ataxia type 2 : MRI study of 20 patients. / Giuffrida, Salvatore; Saponara, Riccardo; Restivo, Domenico A.; Salinaro, Angela Trovato; Tomarchio, Letizia; Pugliares, Pietro; Fabbri, Giuseppe; Maccagnano, Carmelo.
In: Journal of Neurology, Vol. 246, No. 5, 1999, p. 383-388.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Supratentorial atrophy in spinocerebellar ataxia type 2
T2 - MRI study of 20 patients
AU - Giuffrida, Salvatore
AU - Saponara, Riccardo
AU - Restivo, Domenico A.
AU - Salinaro, Angela Trovato
AU - Tomarchio, Letizia
AU - Pugliares, Pietro
AU - Fabbri, Giuseppe
AU - Maccagnano, Carmelo
PY - 1999
Y1 - 1999
N2 - 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.
AB - 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.
KW - Cerebral atrophy
KW - Disease duration
KW - Magnetic resonance imaging
KW - Spinocerebellar ataxia type 2
UR - http://www.scopus.com/inward/record.url?scp=0242526280&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0242526280&partnerID=8YFLogxK
U2 - 10.1007/s004150050368
DO - 10.1007/s004150050368
M3 - Article
C2 - 10399871
AN - SCOPUS:0242526280
VL - 246
SP - 383
EP - 388
JO - Journal of Neurology
JF - Journal of Neurology
SN - 0340-5354
IS - 5
ER -