Brain, Vol. 124, No. 12, 2417-2426,
December 2001
© 2001 Oxford University Press
Morvan's syndrome: peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels
1 Institute of Neurology, University of Bologna, Italy, 2 Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK and 3 Division of Neuropathology, Case Western Reserve University, Cleveland, Ohio, USA
Correspondence to:
Dr Rocco Liguori, Istituto di Clinica Neurologica, Via Ugo Foscolo 7, 40123 Bologna, Italy E-mail: liguori{at}neuro.unibo.it
Morvan's ‘fibrillary chorea’ or Morvan's syndrome is characterized by neuromyotonia (NMT), pain, hyperhydrosis, weight loss, severe insomnia and hallucinations. We describe a man aged 76 years with NMT, dysautonomia, cardiac arrhythmia, lack of slow-wave sleep and abnormal rapid eye movement sleep. He had raised serum antibodies to voltage-gated K+ channels (VGKC), oligoclonal bands in his CSF, markedly increased serum norepinephrine, increased serum cortisol and reduced levels and absent circadian rhythms of prolactin and melatonin. The neurohormonal findings and many of the clinical features were very similar to those in fatal familial insomnia, a hereditary prion disease that is associated with thalamic degenerative changes. Strikingly, however, all symptoms in our MFC patient improved with plasma exchange. The patient died unexpectedly 11 months later. At autopsy, there was a pulmonary adenocarcinoma, but brain pathology showed only a microinfarct in the hippocampus and no thalamic changes. The NMT and some of the autonomic features are likely to be directly related to the VGKC antibodies acting in the periphery. The central symptoms might also be due to the direct effects of VGKC antibodies, or perhaps of other autoantibodies still to be defined, on the limbic system with secondary effects on neurohormone levels. Alternatively, changes in secretion of neurohormones in the periphery might contribute to the central disturbance. The relationship between VGKC antibodies, neurohormonal levels, autonomic, limbic and sleep disorders requires further study.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  R. Hazin, J. Y. Abuzetun, P. Giglio, and F. Khan Agrypnia Excitata: Current Concepts and Future Prospects in Management J Neuropsychiatry Clin Neurosci, May 1, 2009; 21(2): 126 - 131. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Badruddin, R. S. Menon, and A. T. Reder 4-AMINOPYRIDINE TOXICITY MIMICS AUTOIMMUNE-MEDIATED LIMBIC ENCEPHALITIS Neurology, March 24, 2009; 72(12): 1100 - 1101. [Full Text] [PDF]
|
|
|
|
 |
|
 C. Antozzi, S. Binelli, C. Frassoni, C. Ciano, A. Vincent, F. Andreetta, F. Panzica, S. Franceschetti, P. Confalonieri, and R. Mantegazza Immunotherapy responsive startle with antibodies to voltage gated potassium channels BMJ Case Reports, February 2, 2009; 2009(jan27_1): bcr0920080988 - bcr0920080988. [Abstract] [Full Text]
|
|
|
|
|
|
A. Vincent, C. Buckley, B. Lang, S. Irani, S. J. Pittock, and K. Meng Tan CLINICAL SPECTRUM OF VOLTAGE-GATED POTASSIUM CHANNEL AUTOIMMUNITY Neurology, January 6, 2009; 72(1): 99 - 100. [Full Text] [PDF]
|
|
|
|
|
|
M. Spinazzi, V. Argentiero, L. Zuliani, A. Palmieri, B. Tavolato, and A. Vincent IMMUNOTHERAPY-REVERSED COMPULSIVE, MONOAMINERGIC, CIRCADIAN RHYTHM DISORDER IN MORVAN SYNDROME Neurology, December 9, 2008; 71(24): 2008 - 2010. [Full Text] [PDF]
|
|
|
|
|
|
K. M. Tan, V. A. Lennon, C. J. Klein, B. F. Boeve, and S. J. Pittock Clinical spectrum of voltage-gated potassium channel autoimmunity Neurology, May 13, 2008; 70(20): 1883 - 1890. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S Jacob, S R Irani, Y A Rajabally, A Grubneac, R J Walters, M Yazaki, L Clover, and A Vincent Hypothermia in VGKC antibody-associated limbic encephalitis J. Neurol. Neurosurg. Psychiatry, February 1, 2008; 79(2): 202 - 204. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. V. Wuttke, K. Jurkat-Rott, W. Paulus, M. Garncarek, F. Lehmann-Horn, and H. Lerche Peripheral nerve hyperexcitability due to dominant-negative KCNQ2 mutations Neurology, November 27, 2007; 69(22): 2045 - 2053. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Antozzi, S. Binelli, C. Frassoni, C. Ciano, A. Vincent, F. Andreetta, F. Panzica, S. Franceschetti, P. Confalonieri, and R. Mantegazza Immunotherapy responsive startle with antibodies to voltage gated potassium channels J. Neurol. Neurosurg. Psychiatry, November 1, 2007; 78(11): 1281 - 1290. [Full Text] [PDF]
|
|
|
|
 |
|
 A. McKeon, M. Marnane, M. O'Connell, J. P. Stack, P. J. Kelly, and T. Lynch Potassium Channel Antibody Associated Encephalopathy Presenting With a Frontotemporal Dementia like Syndrome Arch Neurol, October 1, 2007; 64(10): 1528 - 1530. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S R Samarasekera, A Vincent, J L Welch, M Jackson, P Nichols, and T D Griffiths Course and outcome of acute limbic encephalitis with negative voltage-gated potassium channel antibodies J. Neurol. Neurosurg. Psychiatry, April 1, 2007; 78(4): 391 - 394. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Hiraga, S. Kuwabara, S. Hayakawa, S. Ito, K. Arimura, K. Kanai, T. Yonezu, and T. Hattori Voltage-gated potassium channel antibody-associated encephalitis with basal ganglia lesions Neurology, June 13, 2006; 66(11): 1780 - 1781. [Full Text] [PDF]
|
|
|
|
 |
|
 K. A. Kleopa, L. B. Elman, B. Lang, A. Vincent, and S. S. Scherer Neuromyotonia and limbic encephalitis sera target mature Shaker-type K+ channels: subunit specificity correlates with clinical manifestations Brain, June 1, 2006; 129(6): 1570 - 1584. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. J. Gomez-Choco, J. Valls-Sole, J. M. Grau, and F. Graus Episodic hyperhidrosis as the only clinical manifestation of neuromyotonia Neurology, October 25, 2005; 65(8): 1331 - 1332. [Full Text] [PDF]
|
|
|
|
|
|
B. M. Ances, R. Vitaliani, R. A. Taylor, D. S. Liebeskind, A. Voloschin, D. J. Houghton, S. L. Galetta, M. Dichter, A. Alavi, M. R. Rosenfeld, et al. Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates Brain, August 1, 2005; 128(8): 1764 - 1777. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Antozzi, C. Frassoni, A. Vincent, M. C. Regondi, F. Andreetta, P. Bernasconi, C. Ciano, T. Chang, F. Cornelio, R. Spreafico, et al. Sequential antibodies to potassium channels and glutamic acid decarboxylase in neuromyotonia Neurology, April 12, 2005; 64(7): 1290 - 1293. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T D Graves and M G Hanna Neurological channelopathies Postgrad. Med. J., January 1, 2005; 81(951): 20 - 32. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D A Cottrell, K J Blackmore, P R W Fawcett, D Birchall, A Vincent, S Barnard, and T J Walls Sub-acute presentation of Morvan's syndrome after thymectomy J. Neurol. Neurosurg. Psychiatry, October 1, 2004; 75(10): 1504 - 1505. [Full Text] [PDF]
|
|
|
|
|
|
M. J. Thieben, V. A. Lennon, B. F. Boeve, A. J. Aksamit, M. Keegan, and S. Vernino Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody Neurology, April 13, 2004; 62(7): 1177 - 1182. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Vincent, C. Buckley, J. M. Schott, I. Baker, B.-K. Dewar, N. Detert, L. Clover, A. Parkinson, C. G. Bien, S. Omer, et al. Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis Brain, March 1, 2004; 127(3): 701 - 712. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. K. Hart, P. Maddison, J. Newsom-Davis, A. Vincent, and K. R. Mills Phenotypic variants of autoimmune peripheral nerve hyperexcitability Brain, August 1, 2002; 125(8): 1887 - 1895. [Abstract] [Full Text] [PDF]
|
|