Spinocerebellar ataxia type 2 with glial cell cytoplasmic inclusions
- 1Department of Neurology, Friedrich-Alexander University, Erlangen, Germany
- 2Department of Neuropathology, Friedrich-Alexander University, Erlangen
- 3Department of Molecular Human Genetics, Ruhr University Bochum, Bochum, Germany
- 4Institute of Clinical Neuropathology, Central Hospital Bremen-Ost, Bremen, Germany
- Correspondence to: Dr Stefan Probst-Cousin Department of Neurology, Friedrich-Alexander University, Schwabachanlage 6, D-91054 Erlangen, Germany; stefan.probst-cousinneuro.imed.uni-erlangen.de
- Received 29 January 2003
- Accepted 13 May 2003
- Revised 19 April 2003
Abstract
Glial cell cytoplasmic inclusions were identified in a case of spinocerebellar ataxia type 2. These have not been reported before. The inclusions were found in low frequency in the dentate nucleus, cerebellar white matter, pontine transverse fibres, and the inferior olivary nucleus. They were of variable size and shape and expressed ubiquitin, thus resembling glial cytoplasmic inclusions in multiple system atrophy. However, their immunohistochemical profile was different as they did not show immunoreactivity for either tau protein or α-synuclein. There was no evidence of expanded polyglutamine tracts in these inclusions, which also failed to label with silver stains. As in many other neurodegenerative diseases, in spinocerebellar ataxia type 2 there may be pathogenic contributions of glial cells other than the common astrogliotic response to neuronal damage.
- NI, neuronal intranuclear inclusion
- MSA, multiple system atrophy
- OPCA, olivo-ponto-cerebellar atrophy
- SCA2, spinocerebellar ataxia type 2
Footnotes
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Competing interests: none declared
