Published in:
01-11-2018 | Brief Communication
Morvan’s syndrome—is a pathogen behind the curtain?
Authors:
Rohit Singh, Pritam Das, Upinder Kaur, Anamika Misra, Ashis Choudhury, Sukdev Manna, Rohit Gaude, Deepak Gautam, Indrajeet Singh Gambhir, Sankha Shubhra Chakrabarti
Published in:
Neurological Sciences
|
Issue 11/2018
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Abstract
Morvan’s syndrome is a rare syndrome of likely autoimmune etiology characterized by peripheral nerve hyperexcitability, dysautonomia, insomnia, and fluctuating delirium with prominent hallucinations. Since its first mention in 1890, less than 100 cases have been described in literature. The largest existing review includes details of 29 cases. This case series describes 4 cases (M = 4) of Morvan’s syndrome which presented between May and November 2017 to a single tertiary care referral teaching hospital in north India. All the four patients manifested behavioral abnormalities, sleep disturbances, hallucinations, autonomic dysfunction, and clinical signs of peripheral nerve hyperexcitability, mostly as myokymia. Two of the patients had Anti-CASPR2 (contactin-associated protein 2) antibodies. Three of them had electromyography features of peripheral nerve hyperexcitability and only one had elevated cerebrospinal fluid protein level. We hypothesize that Morvan’s syndrome and other less characterized autoimmune encephalitis/peripheral nervous system syndromes may have infectious triggers. A possible viral trigger may result in generation of autoantibodies which result in the typical manifestations. We base these hypotheses on the finding of four cases of an orphan disease within a short period of time in a limited geographical distribution.