Management/Treatment of Lambert-Eaton Myasthenic Syndrome

Purpose of review This article reviews the current treatment strategies for patients with Lambert-Eaton myasthenic syndrome (LEMS) including potassium channel blockers, immunosuppressant therapies, and management of dysautonomia. Recent findings Since the first report in 1983, 3,4-diaminopyridine (a...
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Autor*in:	Harada, Yohei [verfasserIn] Guptill, Jeffrey T.

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2021

Schlagwörter:	Lambert-Eaton myasthenic syndrome 3,4-DAP Treatment Amifampridine Amifampridine phosphate

Anmerkung:	© The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021

Übergeordnetes Werk:	Enthalten in: Current treatment options in neurology - Philadelphia, Pa. : Current Science Inc., 1999, 23(2021), 10 vom: 30. Sept.
Übergeordnetes Werk:	volume:23 ; year:2021 ; number:10 ; day:30 ; month:09

Links:	Volltext

DOI / URN:	10.1007/s11940-021-00690-4

Katalog-ID:	SPR045194793

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allfields	10.1007/s11940-021-00690-4 doi (DE-627)SPR045194793 (SPR)s11940-021-00690-4-e DE-627 ger DE-627 rakwb eng Harada, Yohei verfasserin aut Management/Treatment of Lambert-Eaton Myasthenic Syndrome 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021 Purpose of review This article reviews the current treatment strategies for patients with Lambert-Eaton myasthenic syndrome (LEMS) including potassium channel blockers, immunosuppressant therapies, and management of dysautonomia. Recent findings Since the first report in 1983, 3,4-diaminopyridine (amifampridine) has been used as a symptomatic treatment for LEMS. The biggest recent development in LEMS treatment is the approval of two amifampridine drugs in 2018 by the Food and Drug Administration (FDA). Amifampridine phosphate (Firdapse®) is a salt form that was approved for adult patients with LEMS. Amifampridine (Ruzurgi®) was approved for children aged 6 to 17 years old. Summary The primary treatment option for LEMS remains amifampridine, and the efficacy of both FDA-approved formulations has been demonstrated in high-quality clinical trials. Other immunosuppressant treatments, such as corticosteroids, azathioprine, mycophenolate mofetil, and rituximab, can be used when amifampridine is unavailable, are contraindicated, or do not fully control patient symptoms. For the management of acute exacerbations or severe disease, IVIg or plasma exchange can be considered. High-quality evidence supporting the use of immunosuppressive and immunomodulatory treatments in LEMS patients is generally lacking. Treatment response can be monitored by bedside exams and the Triple Timed Up-and-Go test. Lambert-Eaton myasthenic syndrome (dpeaa)DE-He213 3,4-DAP (dpeaa)DE-He213 Treatment (dpeaa)DE-He213 Amifampridine (dpeaa)DE-He213 Amifampridine phosphate (dpeaa)DE-He213 Guptill, Jeffrey T. aut Enthalten in Current treatment options in neurology Philadelphia, Pa. : Current Science Inc., 1999 23(2021), 10 vom: 30. Sept. (DE-627)345859839 (DE-600)2076603-8 1534-3138 nnns volume:23 year:2021 number:10 day:30 month:09 https://dx.doi.org/10.1007/s11940-021-00690-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 23 2021 10 30 09
spelling	10.1007/s11940-021-00690-4 doi (DE-627)SPR045194793 (SPR)s11940-021-00690-4-e DE-627 ger DE-627 rakwb eng Harada, Yohei verfasserin aut Management/Treatment of Lambert-Eaton Myasthenic Syndrome 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021 Purpose of review This article reviews the current treatment strategies for patients with Lambert-Eaton myasthenic syndrome (LEMS) including potassium channel blockers, immunosuppressant therapies, and management of dysautonomia. Recent findings Since the first report in 1983, 3,4-diaminopyridine (amifampridine) has been used as a symptomatic treatment for LEMS. The biggest recent development in LEMS treatment is the approval of two amifampridine drugs in 2018 by the Food and Drug Administration (FDA). Amifampridine phosphate (Firdapse®) is a salt form that was approved for adult patients with LEMS. Amifampridine (Ruzurgi®) was approved for children aged 6 to 17 years old. Summary The primary treatment option for LEMS remains amifampridine, and the efficacy of both FDA-approved formulations has been demonstrated in high-quality clinical trials. Other immunosuppressant treatments, such as corticosteroids, azathioprine, mycophenolate mofetil, and rituximab, can be used when amifampridine is unavailable, are contraindicated, or do not fully control patient symptoms. For the management of acute exacerbations or severe disease, IVIg or plasma exchange can be considered. High-quality evidence supporting the use of immunosuppressive and immunomodulatory treatments in LEMS patients is generally lacking. Treatment response can be monitored by bedside exams and the Triple Timed Up-and-Go test. Lambert-Eaton myasthenic syndrome (dpeaa)DE-He213 3,4-DAP (dpeaa)DE-He213 Treatment (dpeaa)DE-He213 Amifampridine (dpeaa)DE-He213 Amifampridine phosphate (dpeaa)DE-He213 Guptill, Jeffrey T. aut Enthalten in Current treatment options in neurology Philadelphia, Pa. : Current Science Inc., 1999 23(2021), 10 vom: 30. Sept. (DE-627)345859839 (DE-600)2076603-8 1534-3138 nnns volume:23 year:2021 number:10 day:30 month:09 https://dx.doi.org/10.1007/s11940-021-00690-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 23 2021 10 30 09
allfields_unstemmed	10.1007/s11940-021-00690-4 doi (DE-627)SPR045194793 (SPR)s11940-021-00690-4-e DE-627 ger DE-627 rakwb eng Harada, Yohei verfasserin aut Management/Treatment of Lambert-Eaton Myasthenic Syndrome 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021 Purpose of review This article reviews the current treatment strategies for patients with Lambert-Eaton myasthenic syndrome (LEMS) including potassium channel blockers, immunosuppressant therapies, and management of dysautonomia. Recent findings Since the first report in 1983, 3,4-diaminopyridine (amifampridine) has been used as a symptomatic treatment for LEMS. The biggest recent development in LEMS treatment is the approval of two amifampridine drugs in 2018 by the Food and Drug Administration (FDA). Amifampridine phosphate (Firdapse®) is a salt form that was approved for adult patients with LEMS. Amifampridine (Ruzurgi®) was approved for children aged 6 to 17 years old. Summary The primary treatment option for LEMS remains amifampridine, and the efficacy of both FDA-approved formulations has been demonstrated in high-quality clinical trials. Other immunosuppressant treatments, such as corticosteroids, azathioprine, mycophenolate mofetil, and rituximab, can be used when amifampridine is unavailable, are contraindicated, or do not fully control patient symptoms. For the management of acute exacerbations or severe disease, IVIg or plasma exchange can be considered. High-quality evidence supporting the use of immunosuppressive and immunomodulatory treatments in LEMS patients is generally lacking. Treatment response can be monitored by bedside exams and the Triple Timed Up-and-Go test. Lambert-Eaton myasthenic syndrome (dpeaa)DE-He213 3,4-DAP (dpeaa)DE-He213 Treatment (dpeaa)DE-He213 Amifampridine (dpeaa)DE-He213 Amifampridine phosphate (dpeaa)DE-He213 Guptill, Jeffrey T. aut Enthalten in Current treatment options in neurology Philadelphia, Pa. : Current Science Inc., 1999 23(2021), 10 vom: 30. Sept. (DE-627)345859839 (DE-600)2076603-8 1534-3138 nnns volume:23 year:2021 number:10 day:30 month:09 https://dx.doi.org/10.1007/s11940-021-00690-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 23 2021 10 30 09
allfieldsGer	10.1007/s11940-021-00690-4 doi (DE-627)SPR045194793 (SPR)s11940-021-00690-4-e DE-627 ger DE-627 rakwb eng Harada, Yohei verfasserin aut Management/Treatment of Lambert-Eaton Myasthenic Syndrome 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021 Purpose of review This article reviews the current treatment strategies for patients with Lambert-Eaton myasthenic syndrome (LEMS) including potassium channel blockers, immunosuppressant therapies, and management of dysautonomia. Recent findings Since the first report in 1983, 3,4-diaminopyridine (amifampridine) has been used as a symptomatic treatment for LEMS. The biggest recent development in LEMS treatment is the approval of two amifampridine drugs in 2018 by the Food and Drug Administration (FDA). Amifampridine phosphate (Firdapse®) is a salt form that was approved for adult patients with LEMS. Amifampridine (Ruzurgi®) was approved for children aged 6 to 17 years old. Summary The primary treatment option for LEMS remains amifampridine, and the efficacy of both FDA-approved formulations has been demonstrated in high-quality clinical trials. Other immunosuppressant treatments, such as corticosteroids, azathioprine, mycophenolate mofetil, and rituximab, can be used when amifampridine is unavailable, are contraindicated, or do not fully control patient symptoms. For the management of acute exacerbations or severe disease, IVIg or plasma exchange can be considered. High-quality evidence supporting the use of immunosuppressive and immunomodulatory treatments in LEMS patients is generally lacking. Treatment response can be monitored by bedside exams and the Triple Timed Up-and-Go test. Lambert-Eaton myasthenic syndrome (dpeaa)DE-He213 3,4-DAP (dpeaa)DE-He213 Treatment (dpeaa)DE-He213 Amifampridine (dpeaa)DE-He213 Amifampridine phosphate (dpeaa)DE-He213 Guptill, Jeffrey T. aut Enthalten in Current treatment options in neurology Philadelphia, Pa. : Current Science Inc., 1999 23(2021), 10 vom: 30. Sept. (DE-627)345859839 (DE-600)2076603-8 1534-3138 nnns volume:23 year:2021 number:10 day:30 month:09 https://dx.doi.org/10.1007/s11940-021-00690-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 23 2021 10 30 09
allfieldsSound	10.1007/s11940-021-00690-4 doi (DE-627)SPR045194793 (SPR)s11940-021-00690-4-e DE-627 ger DE-627 rakwb eng Harada, Yohei verfasserin aut Management/Treatment of Lambert-Eaton Myasthenic Syndrome 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021 Purpose of review This article reviews the current treatment strategies for patients with Lambert-Eaton myasthenic syndrome (LEMS) including potassium channel blockers, immunosuppressant therapies, and management of dysautonomia. Recent findings Since the first report in 1983, 3,4-diaminopyridine (amifampridine) has been used as a symptomatic treatment for LEMS. The biggest recent development in LEMS treatment is the approval of two amifampridine drugs in 2018 by the Food and Drug Administration (FDA). Amifampridine phosphate (Firdapse®) is a salt form that was approved for adult patients with LEMS. Amifampridine (Ruzurgi®) was approved for children aged 6 to 17 years old. Summary The primary treatment option for LEMS remains amifampridine, and the efficacy of both FDA-approved formulations has been demonstrated in high-quality clinical trials. Other immunosuppressant treatments, such as corticosteroids, azathioprine, mycophenolate mofetil, and rituximab, can be used when amifampridine is unavailable, are contraindicated, or do not fully control patient symptoms. For the management of acute exacerbations or severe disease, IVIg or plasma exchange can be considered. High-quality evidence supporting the use of immunosuppressive and immunomodulatory treatments in LEMS patients is generally lacking. Treatment response can be monitored by bedside exams and the Triple Timed Up-and-Go test. Lambert-Eaton myasthenic syndrome (dpeaa)DE-He213 3,4-DAP (dpeaa)DE-He213 Treatment (dpeaa)DE-He213 Amifampridine (dpeaa)DE-He213 Amifampridine phosphate (dpeaa)DE-He213 Guptill, Jeffrey T. aut Enthalten in Current treatment options in neurology Philadelphia, Pa. : Current Science Inc., 1999 23(2021), 10 vom: 30. Sept. (DE-627)345859839 (DE-600)2076603-8 1534-3138 nnns volume:23 year:2021 number:10 day:30 month:09 https://dx.doi.org/10.1007/s11940-021-00690-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 23 2021 10 30 09
language	English
source	Enthalten in Current treatment options in neurology 23(2021), 10 vom: 30. Sept. volume:23 year:2021 number:10 day:30 month:09
sourceStr	Enthalten in Current treatment options in neurology 23(2021), 10 vom: 30. Sept. volume:23 year:2021 number:10 day:30 month:09
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Lambert-Eaton myasthenic syndrome 3,4-DAP Treatment Amifampridine Amifampridine phosphate
isfreeaccess_bool	false
container_title	Current treatment options in neurology
authorswithroles_txt_mv	Harada, Yohei @@aut@@ Guptill, Jeffrey T. @@aut@@
publishDateDaySort_date	2021-09-30T00:00:00Z
hierarchy_top_id	345859839
id	SPR045194793
language_de	englisch
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author	Harada, Yohei
spellingShingle	Harada, Yohei misc Lambert-Eaton myasthenic syndrome misc 3,4-DAP misc Treatment misc Amifampridine misc Amifampridine phosphate Management/Treatment of Lambert-Eaton Myasthenic Syndrome
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topic_title	Management/Treatment of Lambert-Eaton Myasthenic Syndrome Lambert-Eaton myasthenic syndrome (dpeaa)DE-He213 3,4-DAP (dpeaa)DE-He213 Treatment (dpeaa)DE-He213 Amifampridine (dpeaa)DE-He213 Amifampridine phosphate (dpeaa)DE-He213
topic	misc Lambert-Eaton myasthenic syndrome misc 3,4-DAP misc Treatment misc Amifampridine misc Amifampridine phosphate
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title	Management/Treatment of Lambert-Eaton Myasthenic Syndrome
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title_full	Management/Treatment of Lambert-Eaton Myasthenic Syndrome
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author_browse	Harada, Yohei Guptill, Jeffrey T.
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title_sort	management/treatment of lambert-eaton myasthenic syndrome
title_auth	Management/Treatment of Lambert-Eaton Myasthenic Syndrome
abstract	Purpose of review This article reviews the current treatment strategies for patients with Lambert-Eaton myasthenic syndrome (LEMS) including potassium channel blockers, immunosuppressant therapies, and management of dysautonomia. Recent findings Since the first report in 1983, 3,4-diaminopyridine (amifampridine) has been used as a symptomatic treatment for LEMS. The biggest recent development in LEMS treatment is the approval of two amifampridine drugs in 2018 by the Food and Drug Administration (FDA). Amifampridine phosphate (Firdapse®) is a salt form that was approved for adult patients with LEMS. Amifampridine (Ruzurgi®) was approved for children aged 6 to 17 years old. Summary The primary treatment option for LEMS remains amifampridine, and the efficacy of both FDA-approved formulations has been demonstrated in high-quality clinical trials. Other immunosuppressant treatments, such as corticosteroids, azathioprine, mycophenolate mofetil, and rituximab, can be used when amifampridine is unavailable, are contraindicated, or do not fully control patient symptoms. For the management of acute exacerbations or severe disease, IVIg or plasma exchange can be considered. High-quality evidence supporting the use of immunosuppressive and immunomodulatory treatments in LEMS patients is generally lacking. Treatment response can be monitored by bedside exams and the Triple Timed Up-and-Go test. © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021
abstractGer	Purpose of review This article reviews the current treatment strategies for patients with Lambert-Eaton myasthenic syndrome (LEMS) including potassium channel blockers, immunosuppressant therapies, and management of dysautonomia. Recent findings Since the first report in 1983, 3,4-diaminopyridine (amifampridine) has been used as a symptomatic treatment for LEMS. The biggest recent development in LEMS treatment is the approval of two amifampridine drugs in 2018 by the Food and Drug Administration (FDA). Amifampridine phosphate (Firdapse®) is a salt form that was approved for adult patients with LEMS. Amifampridine (Ruzurgi®) was approved for children aged 6 to 17 years old. Summary The primary treatment option for LEMS remains amifampridine, and the efficacy of both FDA-approved formulations has been demonstrated in high-quality clinical trials. Other immunosuppressant treatments, such as corticosteroids, azathioprine, mycophenolate mofetil, and rituximab, can be used when amifampridine is unavailable, are contraindicated, or do not fully control patient symptoms. For the management of acute exacerbations or severe disease, IVIg or plasma exchange can be considered. High-quality evidence supporting the use of immunosuppressive and immunomodulatory treatments in LEMS patients is generally lacking. Treatment response can be monitored by bedside exams and the Triple Timed Up-and-Go test. © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021
abstract_unstemmed	Purpose of review This article reviews the current treatment strategies for patients with Lambert-Eaton myasthenic syndrome (LEMS) including potassium channel blockers, immunosuppressant therapies, and management of dysautonomia. Recent findings Since the first report in 1983, 3,4-diaminopyridine (amifampridine) has been used as a symptomatic treatment for LEMS. The biggest recent development in LEMS treatment is the approval of two amifampridine drugs in 2018 by the Food and Drug Administration (FDA). Amifampridine phosphate (Firdapse®) is a salt form that was approved for adult patients with LEMS. Amifampridine (Ruzurgi®) was approved for children aged 6 to 17 years old. Summary The primary treatment option for LEMS remains amifampridine, and the efficacy of both FDA-approved formulations has been demonstrated in high-quality clinical trials. Other immunosuppressant treatments, such as corticosteroids, azathioprine, mycophenolate mofetil, and rituximab, can be used when amifampridine is unavailable, are contraindicated, or do not fully control patient symptoms. For the management of acute exacerbations or severe disease, IVIg or plasma exchange can be considered. High-quality evidence supporting the use of immunosuppressive and immunomodulatory treatments in LEMS patients is generally lacking. Treatment response can be monitored by bedside exams and the Triple Timed Up-and-Go test. © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021
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title_short	Management/Treatment of Lambert-Eaton Myasthenic Syndrome
url	https://dx.doi.org/10.1007/s11940-021-00690-4
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up_date	2024-07-03T14:23:28.577Z
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fullrecord_marcxml	<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">SPR045194793</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20230519201220.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">211005s2021 xx \|\|\|\|\|o 00\| \|\|eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1007/s11940-021-00690-4</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)SPR045194793</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(SPR)s11940-021-00690-4-e</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="100" ind1="1" ind2=" "><subfield code="a">Harada, Yohei</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Management/Treatment of Lambert-Eaton Myasthenic Syndrome</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2021</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">© The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Purpose of review This article reviews the current treatment strategies for patients with Lambert-Eaton myasthenic syndrome (LEMS) including potassium channel blockers, immunosuppressant therapies, and management of dysautonomia. Recent findings Since the first report in 1983, 3,4-diaminopyridine (amifampridine) has been used as a symptomatic treatment for LEMS. The biggest recent development in LEMS treatment is the approval of two amifampridine drugs in 2018 by the Food and Drug Administration (FDA). Amifampridine phosphate (Firdapse®) is a salt form that was approved for adult patients with LEMS. Amifampridine (Ruzurgi®) was approved for children aged 6 to 17 years old. Summary The primary treatment option for LEMS remains amifampridine, and the efficacy of both FDA-approved formulations has been demonstrated in high-quality clinical trials. Other immunosuppressant treatments, such as corticosteroids, azathioprine, mycophenolate mofetil, and rituximab, can be used when amifampridine is unavailable, are contraindicated, or do not fully control patient symptoms. For the management of acute exacerbations or severe disease, IVIg or plasma exchange can be considered. 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