RT Journal Article
SR Electronic
T1 Spinocerebellar ataxia type 2 with glial cell cytoplasmic inclusions
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 503
OP 505
DO 10.1136/jnnp.2003.011825
VO 75
IS 3
A1 S Probst-Cousin
A1 T Acker
A1 J T Epplen
A1 M Bergmann
A1 K H Plate
A1 B Neundörfer
A1 D Heuss
YR 2004
UL http://jnnp.bmj.com/content/75/3/503.abstract
AB 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.