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Abstract Details

Adult-Onset Idiopathic Opsoclonus-Myoclonus-Ataxia Syndrome
Autoimmune Neurology
P3 - Poster Session 3 (12:00 PM-1:00 PM)
064

To describe the first case of an adult-onset idiopathic opsoclonus-myoclonus-ataxia syndrome (OMAS) in Central America.

OMAS is a rare immunological central nervous system disease that mostly affects children, with an incidence of 0.2 per 1 million per year, and it is extremely uncommon in adults. It usually presents idiopathically, as a parainfectious condition, or as a paraneoplastic syndrome. No case of adult-onset OMAS has ever been reported in Central America.
NA
A 39-year-old female patient presented with a 1-week history of rapid-onset and progressive dizziness, nausea, and vomiting, associated with a 2-day history of gait instability, memory loss, and sleep disturbances. Past medical history was notable for psoriatic arthritis controlled with methotrexate. Neurologic examination revealed involuntary, rapid, and multidirectional eye saccades compatible with opsoclonus, fast-twitching and jerking movements of the head and bilateral upper extremities compatible with myoclonus, and wide-based gait with instability compatible with ataxia, which suggested the diagnosis of OMAS. There were no motor or sensory deficits, seizures, fever, or symptoms suggestive of infections. Brain magnetic resonance imaging, computed tomography scan of the head, neck, thorax, abdomen, and pelvis with and without contrast showed no abnormalities. Breast, abdomen, and gynecologic ultrasound, esophagogastroduodenoscopy, and colonoscopy showed no lesions suggestive of underlying neoplasia. Cerebrospinal fluid (CSF) analysis showed mild hyperproteinorrhachia and lymphocytic pleocytosis, along with oligoclonal bands. Viral, bacterial, and autoimmune encephalitis panels were negative. CSF bacterial, mycobacterial, and fungi cultures were negative. Serum viral serologies, tumor markers, and antineuronal antibodies were negative. The patient received treatment with plasmapheresis, intravenous immunoglobulin, methylprednisolone, and dexamethasone, with significant but partial improvement of her symptoms.
Although the exact pathophysiology of OMAS remains uncertain, evidence suggests it has an autoimmune mechanism. The diagnosis should be focused on ruling out underlying neoplasia and infections, and the mainstay of management is immunosuppressive therapy.
Authors/Disclosures
Anthony Hong, MD
PRESENTER 		Dr. Hong has nothing to disclose.
Ana Santos, MD Ms. Santos has nothing to disclose.
Jose D. Villegas, MD (CCSS) Dr. Villegas has nothing to disclose.