[A new case of cerebellar ataxia with anti-GAD antibodies treated with corticosteroids and initially seronegative].
BACKGROUND
Cerebellar ataxia with antiglutamic acid decarboxylase antibodies (GAD-ab) is an exceptional newly recognized autoimmune disorder. The cerebellar ataxia may occur in isolation or be associated with stiff man syndrome another rare GAD-Ab induced disorder of central nervous system.


METHODS
A 38-year-old woman with a past history of Graves disease presented with insidious cerebellar symptoms including ataxic gait, dysmetria, dysarthria, and oscillopsia. A thorough survey of markers of paraneoplastic cerebellar ataxia and collagen diseases was negative. Her serum contained high level of GAD-ab (647.2 U/ml) and MRI evidenced pure cerebellar atrophy leading to diagnosis of autoimmune cerebellar ataxia. Under corticosteroids, cerebellar symptoms partially improved, but serum GAD-ab titre dramatically decreased.


CONCLUSIONS
Testing for GAD-ab may be indicated in patients with idiopathic cerebellar ataxia, particularly mature women with organ-specific autoimmune diseases. Corticosteroids must be started to prevent irreversible cerebellar atrophy.
Birand
B
B
Service de neurologie, hôpital Pierre-Zobda-Quitmann, CHU de Fort-de-France, 97261 Fort-de-France, France.
Cabre
P
P
Bonnan
M
M
Olindo
S
S
Smadja
D
D
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use*; D000328:Adult; D001284:Atrophy; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000188:drug therapy; D002524:Cerebellar Ataxia / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D002531:Cerebellum / Q000473:pathology; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D013997:Time Factors; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D005968:Glutamate Decarboxylase