J Neurol Neurosurg Psychiatry 1998;65:857-862 ( December )

Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma

E K Lee,^a R A Maselli,^a W G Ellis,^b M A Agius^a

^a Department of Neurology, ^b Department of Pathology, University of California, Davis Medical Center, Sacramento, CA, USA

Correspondence to: s to: Dr E K Lee, 4860 Y Street, Suite 3700, Sacramento, CA 95817, USA. Telephone 001 916 734 6280; fax 001 916 452 2739.

Received 12 March 1998 and in revised form 29 May 1998; Accepted 15 June 1998

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.

Keywords: Morvan's fibrillary chorea; neuromyotonia; thymoma; paraneoplastic

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© 1998 by Journal of Neurology, Neurosurgery, and Psychiatry
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