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 journalArticle

117 Citations (Scopus)

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

Fingerprint

Myokymia
Thymoma
Immunosuppression
Isaacs Syndrome
N-Type Calcium Channels
Connectin
Hyperhidrosis
Voltage-Gated Potassium Channels
Thymectomy
Plasmapheresis
Delirium
Electrophysiology
Myasthenia Gravis
Sleep Initiation and Maintenance Disorders
Cholinergic Receptors
Denervation
Atopic Dermatitis
Pruritus
Rare Diseases
Psoriasis

Keywords

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

ASJC Scopus subject areas

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

Cite this

Morvan's fibrillary chorea : A paraneoplastic manifestation of thymoma. / Lee, E. K.; Maselli, Ricardo A; Ellis, W. G.; Agius, M. A.

In: Journal of Neurology Neurosurgery and Psychiatry, Vol. 65, No. 6, 1998, p. 857-862.

Research output: Contribution to journalArticle

@article{282d364dd3504553a69fa2e5687e44e6,
title = "Morvan's fibrillary chorea: A paraneoplastic manifestation of thymoma",
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.",
keywords = "Morvan's fibrillary chorea, Neuromyotonia, Paraneoplastic, Thymoma",
author = "Lee, {E. K.} and Maselli, {Ricardo A} and Ellis, {W. G.} and Agius, {M. A.}",
year = "1998",
language = "English (US)",
volume = "65",
pages = "857--862",
journal = "Journal of Neurology, Neurosurgery and Psychiatry",
issn = "0022-3050",
publisher = "BMJ Publishing Group",
number = "6",

}

TY - JOUR

T1 - Morvan's fibrillary chorea

T2 - A paraneoplastic manifestation of thymoma

AU - Lee, E. K.

AU - Maselli, Ricardo A

AU - Ellis, W. G.

AU - Agius, M. A.

PY - 1998

Y1 - 1998

N2 - 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.

AB - 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.

KW - Morvan's fibrillary chorea

KW - Neuromyotonia

KW - Paraneoplastic

KW - Thymoma

UR - http://www.scopus.com/inward/record.url?scp=0032253325&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0032253325&partnerID=8YFLogxK

M3 - Article

C2 - 9854961

AN - SCOPUS:0032253325

VL - 65

SP - 857

EP - 862

JO - Journal of Neurology, Neurosurgery and Psychiatry

JF - Journal of Neurology, Neurosurgery and Psychiatry

SN - 0022-3050

IS - 6

ER -