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Anti-GAD Antibodies and Periodic Alternating Nystagmus
Caroline Tilikete, MD;
Alain Vighetto, MD;
Paul Trouillas, MD;
Jérome Honnorat, MD
Arch Neurol. 2005;62:1300-1303.
Background Autoantibodies directed against glutamic acid decarboxylase (GAD-Ab) have recently been described in a few patients with progressive cerebellar ataxia, suggesting an autoimmune physiopathologic mechanism.
Objective To determine the exact role of GAD-Ab and -aminobutyric acid (GABA)ergic neurotransmission in the pathogenesis of cerebellar ataxia.
Design Case report.
Setting University neurological hospital.
Patient We report the case of a patient with subacute cerebellar ataxia associated with GAD-Ab showing periodic alternating nystagmus (PAN).
Intervention Baclofen, a GABAergic medication, was given to the patient.
Main Outcome Measures Eye movement recording of spontaneous nystagmus and postrotatory vestibular responses.
Results Baclofen was effective in suppressing PAN and improving postrotatory vestibular responses but not for improving cerebellar ataxia.
Conclusion The presence of PAN and the response to baclofen provide a unique opportunity to suggest a direct role of GAD-Ab in cerebellar dysfunction in this patient.
Author Affiliations: Neuro-Ophthalmology Unit (Drs Tilikete and Vighetto) and Neurology B (Drs Trouillas and Honnorat), Université Claude Bernard Lyon-I, Hospices Civils de Lyon, and INSERM U534 (Drs Tilikete and Vighetto), Bron, France; and Institut Fédératif des Neurosciences Lyon, Hôpital Neuro-Cardiologique (Drs Tilikete, Vighetto, Trouillas, and Honnorat), Lyon, France.
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