paraneoplastic opsoclonus myoclonus ataxia; Kinsbourne syndrome; myoclonic encephalopathy of infants; dancing eyes-dancing feet syndrome
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Etiology
- mediator: anti-Ri
- associated neoplasms:
- neuroblastoma
- bronchial carcinoma
- small cell lung cancer
- breast cancer
- fallopian cancer
- viral infections
Epidemiology
- rare
- most often seen in children with tumors
Pathology
- degeneration of the dentate nuclei
- loss of inhibitory control of motor neurons in spinal cord & brainstem
- IgG autoantibody ANNA-2 (anti-Ri)
Clinical manifestations
- evolution over weeks
- dancing eyes & feet
- cerebellar ataxia
- encephalopathy in adults
- myoclonus
- opsoclonus
- variable features
Laboratory
Management
- corticosteroids or ACTH
- treatment of tumor
- prognosis
- with treatment of the underlying cause, there may be an improvement of symptoms
- symptoms sometimes recur without warning
- generally the disorder is not fatal
More general terms
Additional terms
References
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 624
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 16 American College of Physicians, Philadelphia 2012
- ↑ NINDS Opsoclonus Myoclonus Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Opsoclonus-myoclonus-Information-Page