Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma.
Morvan's fibrillary chorea is a rare disease characterised by symptoms which include neuromyotonia, cramping, weakness, pruritus, 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.
Lee
E K
EK
Department of Neurology, University of California, Davis Medical Center, Sacramento, USA.
Maselli
R A
RA
Ellis
W G
WG
Agius
M A
MA
D001706:Biopsy; D002819:Chorea / Q000150:complications* / Q000175:diagnosis; D018450:Disease Progression; D004576:Electromyography / Q000379:methods; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008875:Middle Aged; D009128:Muscle Spasticity / Q000150:complications; D018482:Muscle, Skeletal / Q000294:innervation / Q000473:pathology; D010257:Paraneoplastic Syndromes; D006255:Rhinitis, Allergic, Seasonal / Q000150:complications; D013945:Thymoma / Q000150:complications*; D013953:Thymus Neoplasms / Q000150:complications*
D007074:Immunoglobulin G