Intrathecal immune activation in three patients with progressive myoclonic ataxia.
Three patients displaying a clinical picture of progressively evolving multifocal action myoclonus and cerebellar ataxia showed a marked intrathecal immune activation, which was persistent over a 2- to 5-year time span in the two serially investigated patients. A thorough search for metabolic, toxic, infectious, or degenerative causes of myoclonus was unsuccessful. The presence of intrathecal immune activation in at least a subgroup of patients with the clinical features of progressive myoclonic ataxia suggests the possibility of immune-mediated damage within the central nervous system in this condition.
Salmaggi
A
A
Istituto Nazionale Neurologico C. Besta, Milano, Italy.
Carella
F
F
Ciano
C
C
Binelli
S
S
Giovannini
P
P
Palazzini
E
E
Girotti
F
F
D000328:Adult; D001259:Ataxia / Q000175:diagnosis / Q000276:immunology*; D002490:Central Nervous System / Q000276:immunology*; D019411:Clinical Laboratory Techniques; D006801:Humans; D008214:Lymphocytes; D008297:Male; D008875:Middle Aged; D009207:Myoclonus / Q000175:diagnosis / Q000276:immunology*