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paraneoplastic opsoclonus myoclonus ataxia; Kinsbourne syndrome; myoclonic encephalopathy of infants; dancing eyes-dancing feet syndrome

Etiology: 1) mediator: anti-Ri 2) associated neoplasms: a) neuroblastoma b) bronchial carcinoma c) small cell lung cancer c) breast cancer d) fallopian cancer 3) viral infections Epidemiology: 1) rare 2) most often seen in children with tumors Pathology: 1) degeneration of the dentate nuclei 2) loss of inhibitory control of motor neurons in spinal cord & brainstem 3) IgG autoantibody ANNA-2 (anti-Ri) Clinical manifestations: 1) evolution over weeks 2) dancing eyes & feet - involuntary eye movements 3) cerebellar ataxia 4) encephalopathy in adults 5) myoclonus 6) opsoclonus 7) variable features a) dysarthria b) hypotonia c) lethargy d) irritability e) malaise Laboratory: - ANNA-2 antibody (anti-Ri) Management: 1) corticosteroids or ACTH 2) treatment of tumor 3) prognosis a) with treatment of the underlying cause, there may be an improvement of symptoms b) symptoms sometimes recur without warning c) generally the disorder is not fatal

Interactions

disease interactions

Related

anti-Ri antibody (type-2 ANNA)

General

neuromuscular disease; myoneural disease paraneoplastic neurologic syndrome

References

  1. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 624
  2. Medical Knowledge Self Assessment Program (MKSAP) 16 American College of Physicians, Philadelphia 2012
  3. NINDS Opsoclonus Myoclonus Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Opsoclonus-myoclonus-Information-Page