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Opsoclonus myoclonus
Jonathan R Scarff1, Bushra Iftikhar, Aniket Tatugade
1University of Louisville School of Medicine, Department of Psychiatry and Behavioral Sciences, Louisville, Kentucky, USA.
Abstract:
Opsoclonus myoclonus is a rare autoimmune condition characterized by cerebellar degeneration. It occurs most often as a paraneoplastic syndrome when a cancer remote to the brain induces cerebellar dysfunction that is unrelated to metastases. Half of all cases occur in children with a neuroblastoma. Most adults with opsoclonus myoclonus have neoplastic, infectious, metabolic, or idiopathic etiologies. Signs of cerebellar dysfunction noted at presentation include opsoclonus, myoclonus and ataxia, hence the name "dancing eyes, dancing feet syndrome." Opsoclonus is characterized by rapid, involuntary eye movements that are dysrhythmic and uncoordinated.Neuronal damage is induced by antibodies usually related to the primary pathology. Treatment targets the etiology and also employs steroids, plasmapheresis, immunosuppressive agents, or other anti-inflammatory therapies. Children with opsoclonus myoclonus resulting from a neuroblastoma often retain neurological sequelae. Adult cases of opsoclonus myoclonus with idiopathic or infectious etiologies have a more favorable prognosis than those with neoplastic origins.
Insights
Opsoclonus myoclonus, a rare autoimmune disorder causing cerebellar degeneration, is often linked to cancer. Treatment involves addressing the cause and using anti-inflammatory therapies.
Area of Science:
- Neurology
- Immunology
- Oncology
Background:
- Opsoclonus myoclonus is a rare autoimmune disorder.
- It is characterized by cerebellar degeneration and often presents as a paraneoplastic syndrome.
- Commonly known as "dancing eyes, dancing feet syndrome," it involves rapid, involuntary eye movements and ataxia.
Purpose of the Study:
- To summarize the key features of opsoclonus myoclonus.
- To discuss its various etiologies, including paraneoplastic, infectious, metabolic, and idiopathic causes.
- To outline current treatment strategies and prognostic factors.
Main Methods:
- Literature review of opsoclonus myoclonus cases.
- Analysis of clinical presentation, diagnostic criteria, and treatment outcomes.
- Focus on pediatric (neuroblastoma-associated) and adult cases.
Main Results:
- Half of opsoclonus myoclonus cases occur in children with neuroblastoma.
- Adult etiologies are diverse, including neoplastic, infectious, metabolic, or idiopathic.
- Antibody-mediated neuronal damage is a key pathogenic mechanism.
Conclusions:
- Treatment requires addressing the underlying etiology and implementing anti-inflammatory therapies.
- Children with neuroblastoma-associated opsoclonus myoclonus may have long-term neurological deficits.
- Adults with idiopathic or infectious causes generally have a better prognosis than those with neoplastic origins.
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