Research & Literature

Paraneoplastic nerve hyperexcitability.

Liebenthal JA, Rezania K, Nicholas MK, Lukas RV

Neurological research 2015; (37(6)):553-9 doi:10.1179/1743132815Y.0000000006.

Peripheral nerve hyperexcitability syndromes.

Küçükali CI, Kürtüncü M, Akçay Hİ, et al.

Reviews in the neurosciences 2015; (26(2)):239-51.

Isaacs syndrome: A review.

Ahmed A, Simmons Z

Muscle & nerve 2015; (52(1)):5-12 doi:10.1002/mus.24632.

The relevance of VGKC positivity in the absence of LGI1 and Caspr2 antibodies.

van Sonderen A, Schreurs MW, de Bruijn MA, et al.

Neurology 2016; (86(18)):1692-9 doi:10.1212/WNL.0000000000002637.

Voltage-gated potassium channel-complex autoimmunity and associated clinical syndromes.

Irani SR, Vincent A

Handbook of clinical neurology 2016; (133()):185-97.

Therapeutic Implications of Peripheral Nerve Hyperexcitability in Muscle Cramping: A Retrospective Review.

Hurst RL, Hobson-Webb LD

Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society 2016; (33(6)):560-563 doi:10.1097/WNP.0000000000000291.

Fatal Morvan Syndrome Associated With Myasthenia Gravis.

Nagappa M, Mahadevan A, Sinha S, et al.

The neurologist 2017; (22(1)):29-33 doi:10.1097/NRL.0000000000000097.

Intracellular and non-neuronal targets of voltage-gated potassium channel complex antibodies.

Lang B, Makuch M, Moloney T, et al.

Journal of neurology, neurosurgery, and psychiatry 2017; (88(4)):353-361 doi:10.1136/jnnp-2016-314758.

Osteopathic Manipulative Treatment in the Management of Isaacs Syndrome.

Shanahan LK, Raines SG, Coggins RL, et al.

The Journal of the American Osteopathic Association 2017; (117(3)):194-198 doi:10.7556/jaoa.2017.035.

Autoimmune encephalitis with anti-leucine-rich glioma-inactivated 1 or anti-contactin-associated protein-like 2 antibodies (formerly called voltage-gated potassium channel-complex antibodies).

Bastiaansen AEM, van Sonderen A, Titulaer MJ

Current opinion in neurology 2017; (30(3)):302-309 doi:10.1097/WCO.0000000000000444.

Netrin-1 receptor antibodies in thymoma-associated neuromyotonia with myasthenia gravis.

Torres-Vega E, Mancheño N, Cebrián-Silla A, et al.

Neurology 2017; (88(13)):1235-1242 doi:10.1212/WNL.0000000000003778.

Fasciculation in amyotrophic lateral sclerosis: origin and pathophysiological relevance.

de Carvalho M, Kiernan MC, Swash M

Journal of neurology, neurosurgery, and psychiatry 2017; (88(9)):773-779 doi:10.1136/jnnp-2017-315574.

LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes.

Binks SNM, Klein CJ, Waters P, et al.

Journal of neurology, neurosurgery, and psychiatry 2018; (89(5)):526-534 doi:10.1136/jnnp-2017-315720.

Paraneoplastic autoimmune movement disorders.

Lim TT

Parkinsonism & related disorders 2017; (44()):106-109 doi:10.1016/j.parkreldis.2017.08.017.

Mechanisms of Caspr2 antibodies in autoimmune encephalitis and neuromyotonia.

Patterson KR, Dalmau J, Lancaster E

Annals of neurology 2018; (83(1)):40-51 doi:10.1002/ana.25120.

Morvan's syndrome-is a pathogen behind the curtain?

Singh R, Das P, Kaur U, et al.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2018; (39(11)):1965-1969 doi:10.1007/s10072-018-3515-y.

Acquired neuromyotonia in thymoma-associated myasthenia gravis: a clinical and serological study.

Gastaldi M, De Rosa A, Maestri M, et al.

European journal of neurology 2019; (26(7)):992-999 doi:10.1111/ene.13922.

Peripheral nerve hyperexcitability.

Katirji B

Handbook of clinical neurology 2019; (161()):281-290 doi:10.1016/B978-0-444-64142-7.00054-0.

Waves in Extremities: A Rare Report of Isolated Isaacs' Syndrome.

Bin Waqar SH

Cureus 2019; (11(5)):e4687 doi:10.7759/cureus.4687.

Systematic review of the clinical spectrum of CASPR2 antibody syndrome.

Boyko M, Au KLK, Casault C, et al.

Journal of neurology 2020; (267(4)):1137-1146 doi:10.1007/s00415-019-09686-2.

Painful myokymia after surgery in a patient with Isaacs' syndrome: a case report.

Okutani H, Okano Y, Hirose M

JA clinical reports 2020; (6(1)):14 doi:10.1186/s40981-020-00321-y.

Clinical features of nine cases of leucine-rich glioma inactivated 1 protein antibody-associated encephalitis.

Li Y, Song F, Liu W, Wang Y

Acta neurologica Belgica 2021; (121(4)):889-897 doi:10.1007/s13760-020-01336-z.

Stop testing for autoantibodies to the VGKC-complex: only request LGI1 and CASPR2.

Michael S, Waters P, Irani SR

Practical neurology 2020; (20(5)):377-384 doi:10.1136/practneurol-2019-002494.

Anti-CASPR2 clinical phenotypes correlate with HLA and immunological features.

Muñiz-Castrillo S, Joubert B, Elsensohn MH, et al.

Journal of neurology, neurosurgery, and psychiatry 2020; (91(10)):1076-1084 doi:10.1136/jnnp-2020-323226.

Tacrolimus as a therapeutic option in patients with acquired neuromyotonia.

Liu C, Ji S, Bi Z, et al.

Journal of neuroimmunology 2021; (355()):577569 doi:10.1016/j.jneuroim.2021.577569.

Demystifying the spontaneous phenomena of motor hyperexcitability.

Bashford J, Chan WK, Coutinho E, et al.

Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology 2021; (132(8)):1830-1844 doi:10.1016/j.clinph.2021.03.053.

CASPR2-Related Morvan Syndrome: Autonomic, Polysomnographic, and Neuropsychological Observations.

Swayang PS, Nalini A, Preethish-Kumar V, et al.

Neurology. Clinical practice 2021; (11(3)):e267-e276 doi:10.1212/CPJ.0000000000000978.

Morvan Syndrome Converted from Isaacs' Syndrome after Thymectomy with Positivity for Both Anti-LGI1 and Anti-CASPR2 Antibodies.

Suzuki D, Suzuki Y, Sato D, et al.

Internal medicine (Tokyo, Japan) 2022; (61(9)):1443-1445 doi:10.2169/internalmedicine.8145-21.

Isaacs' syndrome as the initial presentation of malignant thymoma and associated with double-positive voltage-gated potassium channel complex antibodies, a case report.

Li KC, Liao MF, Wu YR, Lyu RK

BMC neurology 2022; (22(1)):74 doi:10.1186/s12883-022-02584-7.

Autoimmune neuromyotonia.

Comperat L, Pegat A, Honnorat J, Joubert B

Current opinion in neurology 2022; (35(5)):597-603 doi:10.1097/WCO.0000000000001104.

Rituximab Was Effective in Relieving Symptoms of Isaacs Syndrome: A Case Report.

Horiuchi K, Kudo A, Inoue T, et al.

Cureus 2022; (14(10)):e30100 doi:10.7759/cureus.30100.

A Rare Phenomenon of Isaacs Syndrome: A Case Report.

Patel AN, Patel PK, Desai J, et al.

Cureus 2023; (15(1)):e34150 doi:10.7759/cureus.34150.

Effectiveness of therapeutic plasma exchange in case of rare neurological disorder Isaacs syndrome.

Tripathi PP, Kumari S, Prabhat N, et al.

Asian journal of transfusion science 2023; (17(1)):117-120 doi:10.4103/ajts.ajts_88_21.

Acquired hyperexcitable peripheral nerve disorders: Clinical and laboratory features, therapeutic responses, and long-term follow-up.

Khadilkar SV, Pandya DC, Dhonde P, et al.

Muscle & nerve 2024; (69(1)):48-54 doi:10.1002/mus.27999.

Systematic Review of the Clinical Characteristics and Management of Isaac Syndrome.

Al-Chalabi M, Hegde P, Moore SR, et al.

Journal of clinical neuromuscular disease 2023; (25(2)):94-106 doi:10.1097/CND.0000000000000460.

Paraneoplastic autoimmune neurologic disorders associated with thymoma.

Iorio R, Lennon VA

Handbook of clinical neurology 2024; (200()):385-396 doi:10.1016/B978-0-12-823912-4.00008-6.

Neuromyotonia.

Zandi MS

Handbook of clinical neurology 2024; (203()):205-210 doi:10.1016/B978-0-323-90820-7.00014-8.

Agrypnia excitata: a human model to explore the derailment of sleep-wake cycle integrated control.

Mutti C, Baldelli L, Cortelli P, et al.

Journal of sleep research 2025; (34(2)):e14324 doi:10.1111/jsr.14324.

The neurobiology and immunology of CASPR2-associated neurological disorders.

Joubert B

Revue neurologique 2024; (180(9)):950-956 doi:10.1016/j.neurol.2024.09.005.

Isaacs' syndrome: Clinical and paraclinical perspectives in a series of cases.

Sarraf P, Shafie M, Farahmand G, et al.

Current journal of neurology 2024; (23(1)):1-14 doi:10.18502/cjn.v23i1.16428.

Muscle ultrasound as a key in assisting the diagnosis of neuromyotonia.

Roy U, Srivastava AK, Cartwright MS, et al.

Neuromuscular disorders : NMD 2025; (46()):105243 doi:10.1016/j.nmd.2024.105243.

Prevalence, clinical profiles, and prognosis of Isaacs syndrome: A nationwide survey study in Japan.

Matsui N, Tanaka K, Kokubun N, et al.

Journal of the neurological sciences 2025; (472()):123442 doi:10.1016/j.jns.2025.123442.

A case report of Isaacs' syndrome with treatment-resistant pain responsive to rituximab.

Inan B, Duzgun U, Kilic SM, et al.

Ideggyogyaszati szemle 2025; (78(5-6)):214-216 doi:10.18071/isz.78.0214.

Thymomas and Thymic Carcinomas, Version 2.2025, NCCN Clinical Practice Guidelines In Oncology.

Riely GJ, Wood DE, Loo BW, et al.

Journal of the National Comprehensive Cancer Network : JNCCN 2025; (23(6)):255-269 doi:10.6004/jnccn.2025.0027.

Video-Polysomnography in Peripheral Nerve Hyperexcitability: Clues to Morvan Syndrome in Two Patients and Literature Review.

Cakar MM, Maharramov E, Gul AC, et al.

Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society 2026; (43(2)):185-188 doi:10.1097/WNP.0000000000001222.

Muscle Stiffness due to Neuromuscular Hyperexcitability.

Katirji B

Muscle & nerve 2026; doi:10.1002/mus.70271.

A case of low-frequency myokymia visualized by simultaneous EMG-ultrasound recording.

Sugimoto T, Naito H, Tachiyama K, et al.

Clinical neurophysiology practice 2026; (11()):348-351 doi:10.1016/j.cnp.2026.04.007.

Survival and relapse of Danish patients with thymic epithelial tumors.

Østergaard T, Bjerke CVA, Santoni-Rugiu E, et al.

Acta oncologica (Stockholm, Sweden) 2026; (65()):460-467 doi:10.2340/1651-226X.2026.45407.

Seronegative Morvan Syndrome Presenting With Agrypnia Excitata and Peripheral Nerve Hyperexcitability: A Phenotype-Supported Diagnostic and Therapeutic Approach in a Resource-Limited Setting.

Taha HM, Taha KH

Clinical case reports 2026; (14(7)):e73196 doi:10.1002/ccr3.73196.

Cardiovascular autonomic dysfunction in Morvan syndrome: A comparative study of heart rate variability and cardiovascular autonomic reflex tests.

Nandan C, Inbaraj G, Arjun K, et al.

Revue neurologique 2026; (182(8)):749-761 doi:10.1016/j.neurol.2026.07.003.

Beyond Nerve Hyperexcitability: Potential Implications of Cerebrospinal Fluid CASPR2 Antibodies for Central Pain Processing in Isaacs Syndrome: A Case-Based Systematic Review.

Theologou R, Zis P

Pain and therapy 2026; doi:10.1007/s40122-026-00893-0.