Dissociation of grey and white matter reduction in spinocerebellar ataxia type 3 and 6: A voxel-based morphometry study

Brain structural damage in spinocerebellar ataxia type 1

“…The atrophy of the GM of the rostral vermis and paramedian anterior lobes of the cerebellum and the WM loss in the peridentate region and middle cerebellar peduncles that VBM demonstrated in our SCA1 patients are partially in line with the above neuropathological descriptions and the results of previous VBM studies in SCA2 [1], SCA3 [13] and multi-system atrophy cerebellar type (MSA-C) [14,20] which are other causes of OPCA.…”

“…Interestingly this limitation of VBM would add to a similar limitation of the method to detect mesial temporal lobe atrophy in Alzheimer's disease [7]. As expected, the distribution of the cerebellar GM atrophy in our SCA1 patients, while similar to that demonstrated by VBM in SCA3 [1,13] and MSA-C [14,20] differs from that reported with the same technique in patients with SCA 6 in whom atrophy involves diffusely the hemispheric lobules and vermis [13] and in patients with SCA17 in whom atrophy was observed in the mesial and posterior cerebellar structures [12]. Interestingly, in the only available VBM study in SCA2 a relative sparing of the rostral vermis was shown [1].…”

“…Voxel-based morphometry (VBM) is an unbiased whole-brain method which assesses regional differences between groups of subjects of volume of the brain gray matter (GM) and white matter (WM) in T1-weighted images [6] and was applied to the investigation of CNS atrophy in patients with autosomic dominant ataxias including SCA2 [1], SCA3, SCA6 [13] and SCA17 [12]. So far, no VBM study evaluated patients with SCA1.…”

Quantitative Assessment of Brain Stem and Cerebellar Atrophy in Spinocerebellar Ataxia Types 3 and 6: Impact on Clinical Status

“…1,6 Brain stem atrophy in SCA3 has been assessed in various MR imaging studies, and its impact on clinical severity has been demonstrated recently. 4,5,10,11,32 We found brain stem volume to be markedly decreased in patients with SCA3. Although patients with SCA3 exhibited additional volume loss in the cerebellum, atrophy of the brain stem was the strongest MR imaging marker explaining the variance of clinical dysfunction in this genotype.…”

Classification of Cerebellar Atrophy Using Voxel-based Morphometry and SPECT with an Easy Z-score Imaging System

“…VBM is capable of assessing gray matter atrophy by differentiating gray matter from white matter (7). It has been reported that VBM is useful for the diagnosis of cerebellar atrophy (4)(5)(6)(7)(8) …”