Exploring the Symptoms of and Insights Into Idiopathic Opsoclonus-Myoclonus-Ataxia Syndrome in Adults.
| Autor: | Santos AB; College of Medicine, University of Costa Rica, San José, CRI., Hong A; College of Medicine, University of Costa Rica, San José, CRI., Hong I; College of Medicine, University of Costa Rica, San José, CRI., Villegas JD; Neurology, Hospital San Juan de Dios, San José, CRI. |
|---|---|
| Jazyk: | angličtina |
| Zdroj: | Cureus [Cureus] 2024 Oct 15; Vol. 16 (10), pp. e71568. Date of Electronic Publication: 2024 Oct 15 (Print Publication: 2024). |
| DOI: | 10.7759/cureus.71568 |
| Abstrakt: | Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare immunological central nervous system disorder that mostly affects children, and it is extremely uncommon in adults. It usually presents idiopathically, as a parainfectious condition, or as a paraneoplastic syndrome. We present a case of a patient who developed adult-onset opsoclonus-myoclonus-ataxia syndrome (OMAS) without any associated infectious or neoplastic disease, a condition that is considered very rare in Central America. This study aimed to document a rare case of adult-onset opsoclonus-myoclonus-ataxia syndrome in a 39-year-old female, highlighting its atypical presentation and the diagnostic challenges involved. The patient presented with a one-week history of rapid-onset and progressive dizziness, nausea, and vomiting, associated with a two-day history of gait instability, memory loss, and sleep disturbances. Past medical history was only notable for psoriatic arthritis controlled with methotrexate. The neurologic examination revealed involuntary, rapid, multidirectional eye saccades consistent with opsoclonus, fast-twitching and jerking movements of the head and bilateral upper extremities consistent with myoclonus, and a wide-based gait with instability indicative of ataxia, suggesting a diagnosis of OMAS. There were no motor or sensory deficits, seizures, fever, or symptoms suggestive of infections. Brain magnetic resonance imaging and 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, and methylprednisolone, with significant but partial improvement of her symptoms. Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work. (Copyright © 2024, Santos et al.) |
| Databáze: | MEDLINE |
| Externí odkaz: |
|