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.
References
Walusinski O, Honnorat J (2013) Augustin Morvan (1819–1897), a little-known rural physician and neurologist. Rev Neurol 169(1):2–8
Lancaster E (2016) The diagnosis and treatment of autoimmune encephalitis. J Clin Neurol 12(1):1–3
Irani SR, Alexander S, Waters P, Kleopa KA, Pettingill P, Zuliani L, Peles E, Buckley C, Lang B, Vincent A (2010) Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan’s syndrome and acquired neuromyotonia. Brain 133(9):2734–2748
Irani SR, Pettingill P, Kleopa KA, Schiza N, Waters P, Mazia C, Zuliani L, Watanabe O, Lang B, Buckley C, Vincent A (2012) Morvan syndrome: clinical and serological observations in 29 cases. Ann Neurol 72:241–255
Kannoth S, Nambiar V, Gopinath S, Anandakuttan A, Mathai A, Rajan PK (2018) Expanding spectrum of contactin-associated protein 2 (CASPR2) autoimmunity—syndrome of parkinsonism and ataxia. Neurol Sci 39:455–460
Sousa S, Guerreiro R, Carmona C, Gouveia LO, Pita F (2017) Caspr2 antibodies in a patient with prostate cancer: a cognitive deterioration with recurrent and paroxysmal gait ataxia and aphasia. Neurol Sci 38:2217–2219
Liguori R, Vincent A, Clover L, Avoni P, Plazzi G, Cortelli P et al (2001) Morvan's syndrome: peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels. Brain 124(12):2417–2426
Evoli A, Lancaster E (2014) Paraneoplastic disorders in thymoma patients. J Thorac Oncol 9(9):S143–S147
Maskery M, Chhetri SK, Dayanandan R, Gall C, Emsley HC (2016) Morvan syndrome: a case report with patient narrative and video. Neurohospitalist 6(1):32–35
Misawa T, Mizusawa H (2010) Anti-VGKC antibody-associated limbic encephalitis/Morvan syndrome. Brain Nerve 62(4):339–345
Kennedy PG (2004) Viral encephalitis: causes, differential diagnosis, and management. J Neurol Neurosurg Psychiatry 75:i10–i15
Venkatesan A, Geocadin RG (2014) Diagnosis and management of acute encephalitis: a practical approach. Neurol Clin Pract 4(3):206–215
Ginsburg KS, Kundsin RB, Walter CW, Schur PH (1992) Ureaplasma urealyticum and mycoplasma hominis in women with systemic lupus erythematosus. Arthritis Rheum 35(4):429–433
Ercolini AM, Miller SD (2009) The role of infections in autoimmune disease. Clin Exp Immunol 155(1):1–5
Karunarathne S, Weerasinghe S, Govindapala D, Fernando H, Jayaratne B (2012) Cold autoimmune haemolytic anaemia secondary to Epstein Barr virus infection presenting with peripheral gangrene; case report. Thromb J 10(1):4
Chaudhuri A, Kennedy P (2002) Diagnosis and treatment of viral encephalitis. Postgrad Med J 78(924):575–583
Peel MC, Finlayson BL, McMahon TA (2007) Updated world map of the Köppen-Geiger climate classification. Hydrol Earth Syst Sci 11:1633–1644
Narain JP, Dhariwal AC, MacIntyre CR (2017) Acute encephalitis in India: an unfolding tragedy. Indian J Med Res 145:584–587
Monticelli J, Geremia N, D’Agaro P, Petix V, Luzzati R (2018) Aseptic central nervous system infections in adults: what predictor for unknown etiological diagnosis? Neurol Sci 39:863–870
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Additional information
Highlights
1. Morvan’s syndrome is a rare autoimmune disorder of peripheral nerve hyperexcitability and central nervous system manifestations.
2. Morvan’s syndrome may have an infectious trigger.
3. A viral pathogen may induce autoimmunity which is responsible for the diverse clinical manifestations of Morvan’s syndrome.
Rights and permissions
About this article
Cite this article
Singh, R., Das, P., Kaur, U. et al. Morvan’s syndrome—is a pathogen behind the curtain?. Neurol Sci 39, 1965–1969 (2018). https://doi.org/10.1007/s10072-018-3515-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10072-018-3515-y