Opsoclonus-myoclonus-ataxia syndrome with autoantibodies to glutamic acid decarboxylase.
Opsoclonus-myoclonus-ataxia syndrome (OMS) is a rare neurological disorder of probably autoimmune origin. Most cases are associated with a remote neoplasm or a viral infection; however in some instances no underlying aetiology can be demonstrated. We report the presence of anti-glutamic acid decarboxylase antibodies (anti-GAD Abs) in the serum and CSF of a patient with idiopathic OMS. Treatment with intravenous immunoglobulin led to a remarkable clinical improvement with parallel reduction of anti-GAD titers. Anti-GAD Abs have been associated with several neurological syndromes. They could also be responsible for the clinical triad of OMS, by impairing GABAergic transmission in specific brainstem and cerebellar circuits. We propose that testing for anti-GAD Abs should be performed in OMS, especially when no other aetiological association can be demonstrated.
Markakis
Ioannis
I
Department of Neurology, Piraeus General State Hospital, Nikaia, Greece. jmarkak2@otenet.gr <jmarkak2@otenet.gr>
Alexiou
Eleni
E
Xifaras
Michael
M
Gekas
Georgios
G
Rombos
Antonios
A
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D001323:Autoantibodies / Q000032:analysis* / Q000097:blood / Q000134:cerebrospinal fluid; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology*; D005260:Female; D020234:Gait Ataxia / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology*; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D008775:Methylprednisolone / Q000627:therapeutic use; D009207:Myoclonus / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology*; D015835:Ocular Motility Disorders / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology*; D011863:Radioimmunoassay; D013577:Syndrome; D014635:Valproic Acid / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D000927:Anticonvulsants; D001323:Autoantibodies; D014635:Valproic Acid; D005968:Glutamate Decarboxylase; D008775:Methylprednisolone