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Morvan syndrome

MedGen UID:
1632829
•Concept ID:
C3854373
•
Disease or Syndrome
Synonym: Morvan's fibrillary chorea
SNOMED CT: Morvan syndrome (763803004); Limbic encephalitis, neuromyotonia, hyperhidrosis, polyneuropathy syndrome (763803004); Morvan fibrillary chorea (763803004)
 
Monarch Initiative: MONDO:0008718
OMIM®: 201300
Orphanet: ORPHA83467

Definition

A rare life threatening acquired neurologic disease with characteristics of neuromyotonia, dysautonomia and encephalopathy with severe insomnia. Signs involving central (e.g. hallucinations, confusion, amnesia, myoclonus), autonomic (e.g. variations in blood pressure, hyperhidrosis) and peripheral (e.g. painful cramps, myokymia) hyperactivity, as well as systemic manifestations (such as weight loss, pruritus, fever), are reported. Thymoma is present in some cases. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMorvan syndrome

Professional guidelines

PubMed

Updates in the Diagnosis and Treatment of Paraneoplastic Neurologic Syndromes.
Grativvol RS, Cavalcante WCP, Castro LHM, Nitrini R, Simabukuro MM
Curr Oncol Rep 2018 Nov 10;20(11):92. doi: 10.1007/s11912-018-0721-y. PMID: 30415318
Management of voltage-gated potassium channel antibody disorders.
Merchut MP
Neurol Clin 2010 Nov;28(4):941-59. doi: 10.1016/j.ncl.2010.03.024. PMID: 20816272

Recent clinical studies

Etiology

Motor symptoms in nonparaneoplastic CNS disorders associated with neural antibodies.
Gaig C, Graus F
Handb Clin Neurol 2023;196:277-294. doi: 10.1016/B978-0-323-98817-9.00004-1. PMID: 37620074
Autoimmune neuromyotonia.
Comperat L, Pegat A, Honnorat J, Joubert B
Curr Opin Neurol 2022 Oct 1;35(5):597-603. Epub 2022 Aug 19 doi: 10.1097/WCO.0000000000001104. PMID: 35989569
CASPR2 autoimmunity in children expanding to mild encephalopathy with hypertension.
Syrbe S, Stettner GM, Bally J, Borggraefe I, Bien CI, Ferfoglia RI, Huppke P, Kern J, Polster T, Probst-Müller E, Schmid S, Steinfeld R, Strozzi S, Weichselbaum A, Weitz M, Ziegler A, Wandinger KP, Leypoldt F, Bien CG
Neurology 2020 Jun 2;94(22):e2290-e2301. Epub 2020 May 18 doi: 10.1212/WNL.0000000000009523. PMID: 32424051
Admission diagnoses of patients later diagnosed with autoimmune encephalitis.
Baumgartner A, Rauer S, Hottenrott T, Leypoldt F, Ufer F, Hegen H, Prüss H, Lewerenz J, Deisenhammer F, Stich O
J Neurol 2019 Jan;266(1):124-132. Epub 2018 Nov 12 doi: 10.1007/s00415-018-9105-3. PMID: 30421340
Updates in the Diagnosis and Treatment of Paraneoplastic Neurologic Syndromes.
Grativvol RS, Cavalcante WCP, Castro LHM, Nitrini R, Simabukuro MM
Curr Oncol Rep 2018 Nov 10;20(11):92. doi: 10.1007/s11912-018-0721-y. PMID: 30415318

Diagnosis

Autoimmune neuromyotonia.
Comperat L, Pegat A, Honnorat J, Joubert B
Curr Opin Neurol 2022 Oct 1;35(5):597-603. Epub 2022 Aug 19 doi: 10.1097/WCO.0000000000001104. PMID: 35989569
Sleep Disturbances Associated with Neurological Autoimmunity.
Devine MF, St Louis EK
Neurotherapeutics 2021 Jan;18(1):181-201. Epub 2021 Mar 30 doi: 10.1007/s13311-021-01020-x. PMID: 33786802Free PMC Article
Caspr2 autoantibody-associated Morvan syndrome predating thymoma relapse by 30 months.
Masrori P, Vaesen Bentein H, Raskin J, Montagna M, De Picker L, De Volder I, Van Schil PE, Janssens A, Mercelis R
Lung Cancer 2021 Mar;153:117-119. Epub 2021 Jan 13 doi: 10.1016/j.lungcan.2021.01.012. PMID: 33485137
Updates in the Diagnosis and Treatment of Paraneoplastic Neurologic Syndromes.
Grativvol RS, Cavalcante WCP, Castro LHM, Nitrini R, Simabukuro MM
Curr Oncol Rep 2018 Nov 10;20(11):92. doi: 10.1007/s11912-018-0721-y. PMID: 30415318
Peripheral Nerve Hyperexcitability Syndromes.
Sawlani K, Katirji B
Continuum (Minneap Minn) 2017 Oct;23(5, Peripheral Nerve and Motor Neuron Disorders):1437-1450. doi: 10.1212/CON.0000000000000520. PMID: 28968370

Therapy

Michel Jouvet as a clinical neurophysiologist and neurologist.
Bastuji H
Sleep Med 2018 Sep;49:73-77. Epub 2018 Aug 2 doi: 10.1016/j.sleep.2018.07.010. PMID: 30145123
Immunotherapy of oneiric stupor in Morvan syndrome: Efficacy documented by actigraphy.
Baiardi S, Provini F, Avoni P, Pasquinelli M, Liguori R
Neurology 2015 Jun 16;84(24):2457-9. Epub 2015 May 15 doi: 10.1212/WNL.0000000000001694. PMID: 25979695
Management of voltage-gated potassium channel antibody disorders.
Merchut MP
Neurol Clin 2010 Nov;28(4):941-59. doi: 10.1016/j.ncl.2010.03.024. PMID: 20816272
Morvan syndrome following B-cell lymphoma.
El-Bitar MK, Muwakkit SA, Abboud MR, Sawaya RA, Boustany RM
J Child Neurol 2010 Aug;25(8):1038-41. Epub 2010 Jan 28 doi: 10.1177/0883073809356108. PMID: 20110220
Immunotherapy-reversed compulsive, monoaminergic, circadian rhythm disorder in Morvan syndrome.
Spinazzi M, Argentiero V, Zuliani L, Palmieri A, Tavolato B, Vincent A
Neurology 2008 Dec 9;71(24):2008-10. doi: 10.1212/01.wnl.0000336972.62597.41. PMID: 19064883

Prognosis

Distinct phenotypes in a cohort of anti-CASPR2 associated neurological syndromes.
Moura J, Samões R, Cardoso M, Sousa AP, Damásio J, Marinho A, Carneiro P, Neves E, Silva AM, Santos E
Clin Neurol Neurosurg 2023 Nov;234:107994. Epub 2023 Sep 30 doi: 10.1016/j.clineuro.2023.107994. PMID: 37797365
Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study.
Qin X, Yang H, Zhu F, Wang Q, Shan W
Front Immunol 2021;12:652864. Epub 2021 May 13 doi: 10.3389/fimmu.2021.652864. PMID: 34054814Free PMC Article
Clinical characteristics of patients double positive for CASPR2 and LGI1-antibodies.
Jia Y, Shi H, Ye J, Wang Y
Clin Neurol Neurosurg 2020 Oct;197:106187. Epub 2020 Aug 26 doi: 10.1016/j.clineuro.2020.106187. PMID: 32911250
The clinical spectrum of Caspr2 antibody-associated disease.
van Sonderen A, Ariño H, Petit-Pedrol M, Leypoldt F, Körtvélyessy P, Wandinger KP, Lancaster E, Wirtz PW, Schreurs MW, Sillevis Smitt PA, Graus F, Dalmau J, Titulaer MJ
Neurology 2016 Aug 2;87(5):521-8. Epub 2016 Jul 1 doi: 10.1212/WNL.0000000000002917. PMID: 27371488Free PMC Article
Morvan syndrome: clinical and serological observations in 29 cases.
Irani SR, Pettingill P, Kleopa KA, Schiza N, Waters P, Mazia C, Zuliani L, Watanabe O, Lang B, Buckley C, Vincent A
Ann Neurol 2012 Aug;72(2):241-55. Epub 2012 Apr 4 doi: 10.1002/ana.23577. PMID: 22473710

Clinical prediction guides

Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study.
Qin X, Yang H, Zhu F, Wang Q, Shan W
Front Immunol 2021;12:652864. Epub 2021 May 13 doi: 10.3389/fimmu.2021.652864. PMID: 34054814Free PMC Article
Anesthetic Management of Robotic Thymectomy in a Patient With Morvan Syndrome: A Case Report.
Royston SE, Hartigan PM
A A Pract 2021 Jan 14;15(1):e01383. doi: 10.1213/XAA.0000000000001383. PMID: 33512906
Morvan syndrome: clinical and serological observations in 29 cases.
Irani SR, Pettingill P, Kleopa KA, Schiza N, Waters P, Mazia C, Zuliani L, Watanabe O, Lang B, Buckley C, Vincent A
Ann Neurol 2012 Aug;72(2):241-55. Epub 2012 Apr 4 doi: 10.1002/ana.23577. PMID: 22473710
Agrypnia excitata.
Lugaresi E, Provini F, Cortelli P
Sleep Med 2011 Dec;12 Suppl 2:S3-10. doi: 10.1016/j.sleep.2011.10.004. PMID: 22136896
Neurophysiologic studies in Morvan syndrome.
Josephs KA, Silber MH, Fealey RD, Nippoldt TB, Auger RG, Vernino S
J Clin Neurophysiol 2004 Nov-Dec;21(6):440-5. doi: 10.1097/00004691-200411000-00008. PMID: 15622131

Recent systematic reviews

Systematic review of the clinical spectrum of CASPR2 antibody syndrome.
Boyko M, Au KLK, Casault C, de Robles P, Pfeffer G
J Neurol 2020 Apr;267(4):1137-1146. Epub 2020 Jan 7 doi: 10.1007/s00415-019-09686-2. PMID: 31912210
LGI1 and CASPR2 autoimmunity in children: Systematic literature review and report of a young girl with Morvan syndrome.
Nosadini M, Toldo I, Tascini B, Bien CG, Parmeggiani L, De Gaspari P, Zuliani L, Sartori S
J Neuroimmunol 2019 Oct 15;335:577008. Epub 2019 Jul 18 doi: 10.1016/j.jneuroim.2019.577008. PMID: 31352183

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