Morvan's fibrillary chorea: A paraneoplastic manifestation of thymoma

E. K. Lee, Ricardo A Maselli, W. G. Ellis, M. A. Agius

Research output: Contribution to journalArticlepeer-review

119 Scopus citations

Abstract

Morvan's fibrillary chorea is a rare disease characterised by symptoms which include neuromyotonia, cramping, weakness, pruritis, hyperhidrosis, insomnia, and delirium. The first case of Morvan's fibrillary chorea to be associated with clinical manifestations of myasthenia gravis with thymoma, psoriasis, and atopic dermatitis is reported. Muscle histopathology disclosed chronic denervation and myopathic changes and in vitro electrophysiology demonstrated both presynaptic and postsynaptic defects in neuromuscular transmission. Serum antibodies to acetylcholine receptors, titin, N-type calcium channels, and voltage gated potassium channels were detected. Plasmapheresis, thymectomy, and long term immunosuppression induced a dramatic resolution of symptoms. The association of thymoma with other autoimmune disorders and autoantibodies, and prolonged and sustained remission with chronic immunosuppression, place Morvan's fibrillary chorea on the range of neurological diseases arising as a paraneoplastic complication of cortical thymomas.

Original languageEnglish (US)
Pages (from-to)857-862
Number of pages6
JournalJournal of Neurology Neurosurgery and Psychiatry
Volume65
Issue number6
StatePublished - 1998

Keywords

  • Morvan's fibrillary chorea
  • Neuromyotonia
  • Paraneoplastic
  • Thymoma

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Neuroscience(all)
  • Psychiatry and Mental health

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