Isaacs Syndrome in A Patient with Positive CASPR2 And LGI1 Antibodies: A Case Report and Literature Review
Abstract
Isaacs Syndrome (IS) is an autoimmune disease characterized by fasciculations, dysautonomia, and hyperactivity of muscle fibers due to hyperexcitability of the peripheral nerve system. Patients with IS often express voltage-gated potassium channels (VGKCs), contactin-associated protein 2 (CASPR2), and leucine-rich glioma- inactivated protein (LGI1) antibodies. Slower rates of grouped fasciculation, known as myokymia, are a common presentation in IS patients. Recently, carbamazepine has been considered as the first-line treatment to alleviate the symptoms of IS patients. In this report, the authors present a case of a female patient with ramps and unintended movements in the abdomen and both lower limbs. She was diagnosed with IS after the detection of myokymia in the needle electromyography (EMG) and a positive paraneoplastic panel for CASPR2 and LGI antibodies. The patient is now symptom-free due to the administration of Carbamazepine, Gabapentin, and Baclofen. Additionally, due to her potential risk for solid tumors, she is under regular follow-up.
[1] Bashford J, Chan WK, Coutinho E, Norwood F, Mills K, Shaw CE. Demystifying the spontaneous phenomena of motor hyperexcitability. Clin Neurophysiol. 2021;132(8):1830-44. https://doi.org/10.1016/j.clinph.2021.03.053
[2] Vincent A, Pettingill P, Pettingill R, Lang B, Birch R, Waters P, et al. Association of Leucine-Rich Glioma Inactivated Protein 1, Contactin-Associated Protein 2, and Contactin 2 Antibodies With Clinical Features and Patient-Reported Pain in Acquired Neuromyotonia. JAMA Neurol. 2018;75(12):1519-27. https:// doi.org/10.1001/jamaneurol.2018.2681
[3] Li KC, Liao MF, Wu YR, Lyu RK. Isaacs’ syndrome as the initial presentation of malignant thymoma and associated with double-positive voltage-gated potassium channel complex antibodies, a case report. BMC Neurol. 2022;22(1):74. https:// doi.org/10.1186/s12883-022-02584-7
[4] Sawlani K, Katirji B. Peripheral Nerve Hyperexcitability Syndromes. Continuum (Minneap Minn). 2017;23(5, Peripheral Nerve and Motor Neuron Disorders):1437-50. https://doi.org/10.1212/CON.0000000000000520
[5] Viallard JF, Vincent A, Moreau JF, Parrens M, Pellegrin JL, Ellie E. Thymoma-associated neuromyotonia with antibodies against voltage-gated potassium channels presenting as chronic intestinal pseudo-obstruction. Eur Neurol. 2005;53(2):60-3. https://doi.org/10.1159/000084300
[6] Gonzalez Primomo SN, Blas L, Bertotti AC, Ameri C. Urinary manifestations in Isaacs’s syndrome. Our experience in 8 cases. Neurourol Urodyn. 2018;37(1):496-500. https://doi. org/10.1002/nau.23336
[7] Mantero V, Rigamonti A, Basso F, Stanzani L, Scaioli V, Salmaggi A. When it rains it pours: amyotrophic lateral sclerosis concealed with Isaac’s syndrome. Neurol Sci. 2016;37(7):1181- 3. https://doi.org/10.1007/s10072-016-2528-7
[8] Zambelis T, Licomanos D, Leonardos A, Potagas C. Neuromyotonia in idiopathic hypoparathyroidism. Neurol Sci. 2009;30(6):495-7. https://doi.org/10.1007/s10072-009-0140-9
[9] Hart IK, Maddison P, Newsom-Davis J, Vincent A, Mills KR. Phenotypic variants of autoimmune peripheral nerve hyperexcitability. Brain. 2002;125(Pt 8):1887-95. https://doi. org/10.1093/brain/awf178
[10] Park SB, Thurbon R, Kiernan MC. Isaacs syndrome: the frontier of neurology, psychiatry, immunology and cancer. J Neurol Neurosurg Psychiatry. 2020;91(12):1243-4. https://doi. org/10.1136/jnnp-2020-324675
[11] Nakazato T, Tsuji Y, Kanai K, Noto YI, Hoshino Y, Yamashiro K, et al. Isaacs syndrome: A slow potassium channelopathy caused by autoantibodies? Clin Neurophysiol. 2018;129(5):956-8. https://doi.org/10.1016/j.clinph.2018.01.069
[12.] Epure DA, Ioghen MR, Roza E, Teleanu RI. A case report of Morvan syndrome, the unique clinical pattern of a rare disease. Ro J Neurol. 2020;19:54-6. https://doi.org/10.37897/ RJN.2020.1.8
[13] Patel AN, Patel PK, Desai J, Margam SS, Oakley K, Reddy PJ. A Rare Phenomenon of Isaacs Syndrome: A Case Report. Cureus. 2023;15(1):e34150. https://doi.org/10.7759/cureus.34150
[14] Ahmed A, Simmons Z. Isaacs syndrome: A review. Muscle Nerve. 2015;52(1):5-12. https://doi.org/10.1002/mus.24632
[15] Caress JB, Walker FO. The spectrum of ectopic motor nerve behavior: from fasciculations to neuromyotonia. Neurologist. 2002;8(1):41-6. https://doi.org/10.1097/00127893-2002010 00-00005
[16] Jamieson PW, Katirji MB. Idiopathic generalized myokymia. Muscle Nerve. 1994;17(1):42-51.
[17] Dhand UK. Isaacs’ syndrome: clinical and electrophysiological response to gabapentin. Muscle Nerve. 2006;34(5):646-50. https://doi.org/10.1002/mus.20591
[18] Pan HL, Eisenach JC, Chen SR. Gabapentin suppresses ectopic nerve discharges and reverses allodynia in neuropathic rats. J Pharmacol Exp Ther. 1999;288(3):1026-30.
[19] Magnuson R, Abrams B, Reiss J. Continuous muscle fiber activity: Isaacs-Mertens’ syndrome. Arch Phys Med Rehabil. 1972;53(6):282-5.
[20] Hart IK, Maddison P, Newsom-Davis J, Vincent A, Mills KR. Phenotypic variants of autoimmune peripheral nerve hyperexcitability. Brain. 2002;125(8):1887-95. https://doi. org/10.1093/brain/awf178
[21] Win-Shwe TT, Fujimaki H. Neurotoxicity of toluene. Toxicol Lett. 2010;198(2):93-9. https://doi.org/10.1016/j. toxlet.2010.06.022 [22] Reeback J, Benton S, Swash M, Schwartz MS. Penicillamine- induced neuromyotonia. Br Med J. 1979;1(6176):1464 https:// doi.org/10.1136/bmj.1.6176.1464
[22] Reeback J, Benton S, Swash M, Schwartz MS. Penicillamine- induced neuromyotonia. Br Med J. 1979;1(6176):1464 https:// doi.org/10.1136/bmj.1.6176.1464
[23] Chaurasia R, Abbas A, Shukla R. Toxin induced continuous muscle fiber activity syndrome. Ann Neurosci. 2010;15(4):118- 9. https://doi.org/10.5214/ans.0972.7531.2008.150405
[24] Rana SS, Ramanathan RS, Small G, Adamovich B. Paraneoplastic Isaacs’ syndrome: a case series and review of the literature. J Clin Neuromuscul Dis. 2012;13(4):228-33. https://doi. org/10.1097/CND.0b013e318246197d
[25] Liebenthal JA, Rezania K, Nicholas MK, Lukas RV. Paraneoplastic nerve hyperexcitability. Neurol Res. 2015;37(6):553-9. https:// doi.org/10.1179/1743132815Y.0000000006
[26] Saint-Martin M, Joubert B, Pellier-Monnin V, Pascual O, Noraz N, Honnorat J. Contactin-associated protein-like 2, a protein of the neurexin family involved in several human diseases. Eur J Neurosci. 2018;48(3):1906-23. https://doi.org/10.1111/ ejn.14081
[27] van Sonderen A, Ariño H, Petit-Pedrol M, Leypoldt F, Körtvélyessy P, Wandinger KP, et al. The clinical spectrum of Caspr2 antibody- associated disease. Neurology. 2016;87(5):521-8. https://doi. org/10.1212/WNL.0000000000002917
[28] Klein CJ, Lennon VA, Aston PA, McKeon A, O’Toole O, Quek A, et al. Insights from LGI1 and CASPR2 potassium channel complex autoantibody subtyping. JAMA Neurol. 2013;70(2):229-34. https://doi.org/10.1001/jamaneurol.2013.592
[29] Boyko M, Au KLK, Casault C, de Robles P, Pfeffer G. Systematic review of the clinical spectrum of CASPR2 antibody syndrome. J Neurol. 2020;267(4):1137-46. https://doi.org/10.1007/s00415- 019-09686-2
[30] López-Chiriboga AS, Klein C, Zekeridou A, McKeon A, Dubey D, Flanagan EP, et al. LGI1 and CASPR2 neurological autoimmunity in children. Ann Neurol. 2018;84(3):473-80. https://doi. org/10.1002/ana.25310
[31] Rebello A, Asif M, Dhanusha K, Haritha B, Narmada N, Poudel RC. When muscle quivers and undulates. Clin Case Rep. 2022;10(7):e6086. https://doi.org/10.1002/ccr3.6086
[32] Surana S, Kumar R, Pitt M, Hafner P, McLellan A, Davidson J, et al. Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies. Dev Med Child Neurol. 2019;61(11):1344-7. https:// doi.org/10.1111/dmcn.14179
[33] Samogalskyi V, Alcalay Y, Gadoth A, Eilam A, Gilad R. Case report: Isolated muscle neuromyotonia, as presenting feature of Isaacs’ syndrome. J Neuroimmunol. 2021;353:577491. https://doi. org/10.1016/j.jneuroim.2021.577491
[34] Fleisher J, Richie M, Price R, Scherer S, Dalmau J, Lancaster E. Acquired neuromyotonia heralding recurrent thymoma in myasthenia gravis. JAMA Neurol. 2013;70(10):1311-4. https:// doi.org/10.1001/jamaneurol.2013.2863
[35] Vernino S, Adamski J, Kryzer TJ, Fealey RD, Lennon VA. Neuronal nicotinic ACh receptor antibody in subacute autonomic neuropathy and cancer-related syndromes. Neurology. 1998;50(6):1806-13. https://doi.org/10.1212/WNL.50.6.1806
[36] Vincent A, Irani SR. Caspr2 antibodies in patients with thymomas. J Thorac Oncol. 2010;5(10):S277-S80. https://doi. org/10.1097/JTO.0b013e3181f23f04
[37] Bernard C, Frih H, Pasquet F, Kerever S, Jamilloux Y, Tronc F, et al. Thymoma associated with autoimmune diseases: 85 cases and literature review. Autoimmun Rev. 2016;15(1):82-92. https:// doi.org/10.1016/j.autrev.2015.09.005
[38] Walsh JC. Neuromyotonia: an unusual presentation of intrathoracic malignancy. J Neurol Neurosurg Psychiatry. 1976;39(11):1086-91. https://doi.org/10.1136/jnnp.39.11.1086
[39] Partanen V, Soininen H, Saksa M, Riekkinen P. Electrom- yographic and nerve conduction findings in a patient with neuromyotonia, normocalcemic tetany and small-cell lung cancer. Acta Neurol Scand. 1980;61(4):216-26. https://doi. org/10.1111/j.1600-0404.1980.tb01486.x
[40] Garcia-Merino A, Cabello A, Mora J, Liano H. Continuous muscle fiber activity, peripheral neuropathy, and thymoma. Ann Neurol. 1991;29(2):215-8. https://doi.org/10.1002/ana.410290218
[41] Perini M, Ghezzi A, Basso P, Montanini R. Association of neuromyotonia with peripheral neuropathy, myasthenia gravis and thymoma: a case report. Ital J Neurol Sci. 1994;15:307-10. https://doi.org/10.1007/BF02339242
[42] Tsivgoulis G, Mikroulis D, Katsanos AH, Vadikolias K, Heliopoulos I, Stamboulis E, et al. Paraneoplastic Isaac’s syndrome associated with thymoma and anti-neuronal nuclear antibodies 1. J Neurol Sci. 2014;343(1-2):245-6. https://doi.org/10.1016/j. jns.2014.06.004
[43] Paul BS, Singh G, Bansal RK, Singla M. Isaac’s syndrome associated with myasthenia gravis and thymoma. Indian J Med Sci. 2010;64(7):320-4. https://doi.org/10.4103/0019- 5359.99866
[44] Caress JB, Abend WK, Preston DC, Logigian EL. A case of Hodgkin’s lymphoma producing neuromyotonia. Neurology. 1997;49(1):258-9. https://doi.org/10.1212/WNL.49.1.258
[45] Zifko U, Drlicek M, Machacek E, Jellinger K, Grisold W. Syndrome of continuous muscle fiber activity and plasmacytoma with IgM paraproteinemia. Neurology. 1994;44(3 Part 1):560-. https:// doi.org/10.1212/WNL.44.3_Part_1.560
[46] Issa SS, Herskovitz S, Lipton RB. Acquired neuromyotonia as a paraneoplastic manifestation of ovarian cancer. Neurology. 2011;76(1):100-1. https://doi.org/10.1212/ WNL.0b013e318203e91a
[47] Forte F, Pretegiani E, Battisti C, Sicurelli F, Federico A. Neuromyotonia as paraneoplastic manifestation of bladder carcinoma. J Neurol Sci. 2009;280(1-2):111-2. https://doi. org/10.1016/j.jns.2009.01.022
[48] Rana SS, Ramanathan RS, Small G, Adamovich B. Paraneoplastic Isaacs’ syndrome: a case series and review of the literature. J Clin Neuromuscul Dis. 2012;13(4):228-33. https://doi.org/10.1097/ CND.0b013e318246197d
[49] Zhang L, Liu J, Wang H, Zhao C, Lu J, Xue J, et al. Double filtration plasmapheresis benefits myasthenia gravis patients through an immunomodulatory action. J Clin Neurosci. 2014;21(9):1570-4. https://doi.org/10.1016/j.jocn.2013.11.046
[50] Song J, Jing S, Quan C, Lu J, Qiao X, Qiao K, et al. Isaacs syndrome with CASPR2 antibody: A series of three cases. J Clin Neurosci. 2017;41:63-6. https://doi.org/10.1016/j.jocn.2017.02.063
[51] Ye Z, Jin Y, Xu H, Qiao X, Yu H, Xu S, et al. Effectiveness of immu- notherapy in a CASPR2 and LGI1 antibody-positive elderly patient with Isaacs’ syndrome: a case study. Acta Neurol Belg. 2021;121(2):577-9. https://doi.org/10.1007/s13760-020- 01446-8
[52] Horiuchi K, Kudo A, Inoue T, Fujii S, Oshima Y. Rituximab Was Effective in Relieving Symptoms of Isaacs Syndrome: A Case Report. Cureus. 2022;14(10):e30100. https://doi.org/10.7759/ cureus.30100
[2] Vincent A, Pettingill P, Pettingill R, Lang B, Birch R, Waters P, et al. Association of Leucine-Rich Glioma Inactivated Protein 1, Contactin-Associated Protein 2, and Contactin 2 Antibodies With Clinical Features and Patient-Reported Pain in Acquired Neuromyotonia. JAMA Neurol. 2018;75(12):1519-27. https:// doi.org/10.1001/jamaneurol.2018.2681
[3] Li KC, Liao MF, Wu YR, Lyu RK. Isaacs’ syndrome as the initial presentation of malignant thymoma and associated with double-positive voltage-gated potassium channel complex antibodies, a case report. BMC Neurol. 2022;22(1):74. https:// doi.org/10.1186/s12883-022-02584-7
[4] Sawlani K, Katirji B. Peripheral Nerve Hyperexcitability Syndromes. Continuum (Minneap Minn). 2017;23(5, Peripheral Nerve and Motor Neuron Disorders):1437-50. https://doi.org/10.1212/CON.0000000000000520
[5] Viallard JF, Vincent A, Moreau JF, Parrens M, Pellegrin JL, Ellie E. Thymoma-associated neuromyotonia with antibodies against voltage-gated potassium channels presenting as chronic intestinal pseudo-obstruction. Eur Neurol. 2005;53(2):60-3. https://doi.org/10.1159/000084300
[6] Gonzalez Primomo SN, Blas L, Bertotti AC, Ameri C. Urinary manifestations in Isaacs’s syndrome. Our experience in 8 cases. Neurourol Urodyn. 2018;37(1):496-500. https://doi. org/10.1002/nau.23336
[7] Mantero V, Rigamonti A, Basso F, Stanzani L, Scaioli V, Salmaggi A. When it rains it pours: amyotrophic lateral sclerosis concealed with Isaac’s syndrome. Neurol Sci. 2016;37(7):1181- 3. https://doi.org/10.1007/s10072-016-2528-7
[8] Zambelis T, Licomanos D, Leonardos A, Potagas C. Neuromyotonia in idiopathic hypoparathyroidism. Neurol Sci. 2009;30(6):495-7. https://doi.org/10.1007/s10072-009-0140-9
[9] Hart IK, Maddison P, Newsom-Davis J, Vincent A, Mills KR. Phenotypic variants of autoimmune peripheral nerve hyperexcitability. Brain. 2002;125(Pt 8):1887-95. https://doi. org/10.1093/brain/awf178
[10] Park SB, Thurbon R, Kiernan MC. Isaacs syndrome: the frontier of neurology, psychiatry, immunology and cancer. J Neurol Neurosurg Psychiatry. 2020;91(12):1243-4. https://doi. org/10.1136/jnnp-2020-324675
[11] Nakazato T, Tsuji Y, Kanai K, Noto YI, Hoshino Y, Yamashiro K, et al. Isaacs syndrome: A slow potassium channelopathy caused by autoantibodies? Clin Neurophysiol. 2018;129(5):956-8. https://doi.org/10.1016/j.clinph.2018.01.069
[12.] Epure DA, Ioghen MR, Roza E, Teleanu RI. A case report of Morvan syndrome, the unique clinical pattern of a rare disease. Ro J Neurol. 2020;19:54-6. https://doi.org/10.37897/ RJN.2020.1.8
[13] Patel AN, Patel PK, Desai J, Margam SS, Oakley K, Reddy PJ. A Rare Phenomenon of Isaacs Syndrome: A Case Report. Cureus. 2023;15(1):e34150. https://doi.org/10.7759/cureus.34150
[14] Ahmed A, Simmons Z. Isaacs syndrome: A review. Muscle Nerve. 2015;52(1):5-12. https://doi.org/10.1002/mus.24632
[15] Caress JB, Walker FO. The spectrum of ectopic motor nerve behavior: from fasciculations to neuromyotonia. Neurologist. 2002;8(1):41-6. https://doi.org/10.1097/00127893-2002010 00-00005
[16] Jamieson PW, Katirji MB. Idiopathic generalized myokymia. Muscle Nerve. 1994;17(1):42-51.
[17] Dhand UK. Isaacs’ syndrome: clinical and electrophysiological response to gabapentin. Muscle Nerve. 2006;34(5):646-50. https://doi.org/10.1002/mus.20591
[18] Pan HL, Eisenach JC, Chen SR. Gabapentin suppresses ectopic nerve discharges and reverses allodynia in neuropathic rats. J Pharmacol Exp Ther. 1999;288(3):1026-30.
[19] Magnuson R, Abrams B, Reiss J. Continuous muscle fiber activity: Isaacs-Mertens’ syndrome. Arch Phys Med Rehabil. 1972;53(6):282-5.
[20] Hart IK, Maddison P, Newsom-Davis J, Vincent A, Mills KR. Phenotypic variants of autoimmune peripheral nerve hyperexcitability. Brain. 2002;125(8):1887-95. https://doi. org/10.1093/brain/awf178
[21] Win-Shwe TT, Fujimaki H. Neurotoxicity of toluene. Toxicol Lett. 2010;198(2):93-9. https://doi.org/10.1016/j. toxlet.2010.06.022 [22] Reeback J, Benton S, Swash M, Schwartz MS. Penicillamine- induced neuromyotonia. Br Med J. 1979;1(6176):1464 https:// doi.org/10.1136/bmj.1.6176.1464
[22] Reeback J, Benton S, Swash M, Schwartz MS. Penicillamine- induced neuromyotonia. Br Med J. 1979;1(6176):1464 https:// doi.org/10.1136/bmj.1.6176.1464
[23] Chaurasia R, Abbas A, Shukla R. Toxin induced continuous muscle fiber activity syndrome. Ann Neurosci. 2010;15(4):118- 9. https://doi.org/10.5214/ans.0972.7531.2008.150405
[24] Rana SS, Ramanathan RS, Small G, Adamovich B. Paraneoplastic Isaacs’ syndrome: a case series and review of the literature. J Clin Neuromuscul Dis. 2012;13(4):228-33. https://doi. org/10.1097/CND.0b013e318246197d
[25] Liebenthal JA, Rezania K, Nicholas MK, Lukas RV. Paraneoplastic nerve hyperexcitability. Neurol Res. 2015;37(6):553-9. https:// doi.org/10.1179/1743132815Y.0000000006
[26] Saint-Martin M, Joubert B, Pellier-Monnin V, Pascual O, Noraz N, Honnorat J. Contactin-associated protein-like 2, a protein of the neurexin family involved in several human diseases. Eur J Neurosci. 2018;48(3):1906-23. https://doi.org/10.1111/ ejn.14081
[27] van Sonderen A, Ariño H, Petit-Pedrol M, Leypoldt F, Körtvélyessy P, Wandinger KP, et al. The clinical spectrum of Caspr2 antibody- associated disease. Neurology. 2016;87(5):521-8. https://doi. org/10.1212/WNL.0000000000002917
[28] Klein CJ, Lennon VA, Aston PA, McKeon A, O’Toole O, Quek A, et al. Insights from LGI1 and CASPR2 potassium channel complex autoantibody subtyping. JAMA Neurol. 2013;70(2):229-34. https://doi.org/10.1001/jamaneurol.2013.592
[29] Boyko M, Au KLK, Casault C, de Robles P, Pfeffer G. Systematic review of the clinical spectrum of CASPR2 antibody syndrome. J Neurol. 2020;267(4):1137-46. https://doi.org/10.1007/s00415- 019-09686-2
[30] López-Chiriboga AS, Klein C, Zekeridou A, McKeon A, Dubey D, Flanagan EP, et al. LGI1 and CASPR2 neurological autoimmunity in children. Ann Neurol. 2018;84(3):473-80. https://doi. org/10.1002/ana.25310
[31] Rebello A, Asif M, Dhanusha K, Haritha B, Narmada N, Poudel RC. When muscle quivers and undulates. Clin Case Rep. 2022;10(7):e6086. https://doi.org/10.1002/ccr3.6086
[32] Surana S, Kumar R, Pitt M, Hafner P, McLellan A, Davidson J, et al. Acquired neuromyotonia in children with CASPR2 and LGI1 antibodies. Dev Med Child Neurol. 2019;61(11):1344-7. https:// doi.org/10.1111/dmcn.14179
[33] Samogalskyi V, Alcalay Y, Gadoth A, Eilam A, Gilad R. Case report: Isolated muscle neuromyotonia, as presenting feature of Isaacs’ syndrome. J Neuroimmunol. 2021;353:577491. https://doi. org/10.1016/j.jneuroim.2021.577491
[34] Fleisher J, Richie M, Price R, Scherer S, Dalmau J, Lancaster E. Acquired neuromyotonia heralding recurrent thymoma in myasthenia gravis. JAMA Neurol. 2013;70(10):1311-4. https:// doi.org/10.1001/jamaneurol.2013.2863
[35] Vernino S, Adamski J, Kryzer TJ, Fealey RD, Lennon VA. Neuronal nicotinic ACh receptor antibody in subacute autonomic neuropathy and cancer-related syndromes. Neurology. 1998;50(6):1806-13. https://doi.org/10.1212/WNL.50.6.1806
[36] Vincent A, Irani SR. Caspr2 antibodies in patients with thymomas. J Thorac Oncol. 2010;5(10):S277-S80. https://doi. org/10.1097/JTO.0b013e3181f23f04
[37] Bernard C, Frih H, Pasquet F, Kerever S, Jamilloux Y, Tronc F, et al. Thymoma associated with autoimmune diseases: 85 cases and literature review. Autoimmun Rev. 2016;15(1):82-92. https:// doi.org/10.1016/j.autrev.2015.09.005
[38] Walsh JC. Neuromyotonia: an unusual presentation of intrathoracic malignancy. J Neurol Neurosurg Psychiatry. 1976;39(11):1086-91. https://doi.org/10.1136/jnnp.39.11.1086
[39] Partanen V, Soininen H, Saksa M, Riekkinen P. Electrom- yographic and nerve conduction findings in a patient with neuromyotonia, normocalcemic tetany and small-cell lung cancer. Acta Neurol Scand. 1980;61(4):216-26. https://doi. org/10.1111/j.1600-0404.1980.tb01486.x
[40] Garcia-Merino A, Cabello A, Mora J, Liano H. Continuous muscle fiber activity, peripheral neuropathy, and thymoma. Ann Neurol. 1991;29(2):215-8. https://doi.org/10.1002/ana.410290218
[41] Perini M, Ghezzi A, Basso P, Montanini R. Association of neuromyotonia with peripheral neuropathy, myasthenia gravis and thymoma: a case report. Ital J Neurol Sci. 1994;15:307-10. https://doi.org/10.1007/BF02339242
[42] Tsivgoulis G, Mikroulis D, Katsanos AH, Vadikolias K, Heliopoulos I, Stamboulis E, et al. Paraneoplastic Isaac’s syndrome associated with thymoma and anti-neuronal nuclear antibodies 1. J Neurol Sci. 2014;343(1-2):245-6. https://doi.org/10.1016/j. jns.2014.06.004
[43] Paul BS, Singh G, Bansal RK, Singla M. Isaac’s syndrome associated with myasthenia gravis and thymoma. Indian J Med Sci. 2010;64(7):320-4. https://doi.org/10.4103/0019- 5359.99866
[44] Caress JB, Abend WK, Preston DC, Logigian EL. A case of Hodgkin’s lymphoma producing neuromyotonia. Neurology. 1997;49(1):258-9. https://doi.org/10.1212/WNL.49.1.258
[45] Zifko U, Drlicek M, Machacek E, Jellinger K, Grisold W. Syndrome of continuous muscle fiber activity and plasmacytoma with IgM paraproteinemia. Neurology. 1994;44(3 Part 1):560-. https:// doi.org/10.1212/WNL.44.3_Part_1.560
[46] Issa SS, Herskovitz S, Lipton RB. Acquired neuromyotonia as a paraneoplastic manifestation of ovarian cancer. Neurology. 2011;76(1):100-1. https://doi.org/10.1212/ WNL.0b013e318203e91a
[47] Forte F, Pretegiani E, Battisti C, Sicurelli F, Federico A. Neuromyotonia as paraneoplastic manifestation of bladder carcinoma. J Neurol Sci. 2009;280(1-2):111-2. https://doi. org/10.1016/j.jns.2009.01.022
[48] Rana SS, Ramanathan RS, Small G, Adamovich B. Paraneoplastic Isaacs’ syndrome: a case series and review of the literature. J Clin Neuromuscul Dis. 2012;13(4):228-33. https://doi.org/10.1097/ CND.0b013e318246197d
[49] Zhang L, Liu J, Wang H, Zhao C, Lu J, Xue J, et al. Double filtration plasmapheresis benefits myasthenia gravis patients through an immunomodulatory action. J Clin Neurosci. 2014;21(9):1570-4. https://doi.org/10.1016/j.jocn.2013.11.046
[50] Song J, Jing S, Quan C, Lu J, Qiao X, Qiao K, et al. Isaacs syndrome with CASPR2 antibody: A series of three cases. J Clin Neurosci. 2017;41:63-6. https://doi.org/10.1016/j.jocn.2017.02.063
[51] Ye Z, Jin Y, Xu H, Qiao X, Yu H, Xu S, et al. Effectiveness of immu- notherapy in a CASPR2 and LGI1 antibody-positive elderly patient with Isaacs’ syndrome: a case study. Acta Neurol Belg. 2021;121(2):577-9. https://doi.org/10.1007/s13760-020- 01446-8
[52] Horiuchi K, Kudo A, Inoue T, Fujii S, Oshima Y. Rituximab Was Effective in Relieving Symptoms of Isaacs Syndrome: A Case Report. Cureus. 2022;14(10):e30100. https://doi.org/10.7759/ cureus.30100
| Files | ||
| Issue | Vol 8 No 5 (2023): September-October | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/crcp.v8i5.15263 | |
| Keywords | ||
| Isaac syndrome; Autoimmune Myokymia Hyperexcitability CASPR2 Paraneoplastic syndromes | ||
| Rights and permissions | |
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Zafari R, Ahmadi M. Isaacs Syndrome in A Patient with Positive CASPR2 And LGI1 Antibodies: A Case Report and Literature Review. CRCP. 2024;8(5):217-222.
