Morvan's syndrome

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Morvan's syndrome
ICD-10 G60.8
ICD-9 336.0
DiseasesDB 12769
MeSH D013595

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Morvan's syndrome (or fibrillary chorea) is a rare disease named after nineteenth century French physician Augustin Marie Morvan (1819-1897).[1]

Symptoms

Morvan's syndrome is characterised by status epilepticus & tonic-clonic epilepsy (grand mal) plus neuromyotonia (involuntary fibrillary contraction of muscles at rest), autonomic nervous system dysfunction, central nervous system dysfunction and endocrine system problems. Major central nervous system problems are sleep disorder, confusion, amnesia, hallucinations and delusions.

One of the most striking symptoms is a decreased need for sleep. In 1974 a neurobiologist Michel Jouvet reported a case of a 27-year-old man with Morvan's syndrome who required no sleep and rarely felt tired for periods of many months yet would experience daily hallucinations between 9 P.M. and 11 P.M. as well as pain and blood vessel constriction.[2]

Causes

Some cases with this syndrome have been suggested to be caused by an autoimmune disease linked to VGKC antibodies and antibodies to potassium channels, others are caused by paraneoplastic syndromes or are side effects of procedures such as a thymectomy.[3]

Treatment

Treatment is variable and many cases remit spontaneously. Still others require plasmapheresis, immunosuppression, thymectomy and immunomodulating treatments. Obviously, if the cause is an underlying malignancy, the disease may remit once the malignancy itself is treated or surgically removed.

Comorbid conditions

Some conditions such as myasthenia gravis are often also present during the condition as are tumors particularly thymus tumors

References

  1. Morvan's disease at Who Named It
  2. Scientific American Mind, April/May 2006, pg. 86
  3. Cottrell D, Blackmore K, Fawcett P, Birchall D, Vincent A, Barnard S, Walls T (2004). "Sub-acute presentation of Morvan's syndrome after thymectomy". J Neurol Neurosurg Psychiatry. 75 (10): 1504–5. PMID 15377711.




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