[Stiff-person syndrome and other neurological disorders associated with anti-GAD antibodies].
Autoantibodies to glutamic acid decarboxylase (GAD), originally identified in the stiff-person syndrome, are also associated with rare cases of therapy-resistant epilepsy and sporadic cerebellar ataxia. The association of GAD antibodies with these three syndromes and other auto-immune diseases, particularly type 1 diabetes mellitus, argues for their auto-immune origin. Anti-GAD antibodies inhibit GABAergic circuits inducing a neuronal hyper-excitability that seems to be responsible for these three syndromes. However, an additional mechanism seems to be involved in the degenerative component of the cerebellar ataxia associated with anti-GAD antibodies. A more accurate diagnosis and the study of neuropathological cases are necessary to document the different mechanisms implicated in these neurological disorders.
Hijazi
J
J
Service de neurologie, CHU de Rouen, Rouen, France. hijazi_jihad@yahoo.fr
Bedat-Millet
A-L
AL
Hannequin
D
D
D001323:Autoantibodies / Q000276:immunology*; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D009422:Nervous System Diseases / Q000276:immunology*; D016750:Stiff-Person Syndrome / Q000276:immunology*
D001323:Autoantibodies; D005968:Glutamate Decarboxylase