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1. Update on treatment options for Lambert–Eaton myasthenic syndrome: focus on use of amifampridine

Author

Lindquist S and Stangel M

Subjects
Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571, Neurology. Diseases of the nervous system, RC346-429
Abstract

Sabine Lindquist, Martin StangelSection Clinical Neuroimmunology and Neurochemistry, Department of Neurology, Hannover Medical School, Hannover, GermanyAbstract: In Lambert–Eaton myasthenic syndrome (LEMS), antibodies against presynaptic voltage-gated calcium channels reduce the quantal release of acetylcholine, causing muscle weakness and autonomic dysfunction. More than half of the affected patients have associated small cell lung cancer, and thorough screening for an underlying malignancy is crucial. The mainstay of treatment for LEMS is symptomatic but immunotherapy is needed in more severely affected patients. Symptomatic therapies aim at increasing the concentration of acetylcholine at the muscle endplate. While acetylcholinesterase inhibitors were the first drugs to be used for the amelioration of symptoms, 3,4-diaminopyridine (3,4-DAP, amifampridine) has been shown to be more effective. 3,4-DAP blocks presynaptic potassium channels, thereby prolonging the action potential and increasing presynaptic calcium concentrations. This then results in increased quantal release of acetylcholine. The efficacy of 3,4-DAP for increasing muscle strength and resting compound muscle action potentials has been demonstrated by four placebo-controlled trials. Side effects are usually mild, and the most frequently reported are paresthesias. The most common serious adverse events are epileptic seizures. 3,4-DAP is currently the treatment of choice in patients with Lambert–Eaton myasthenic syndrome.Keywords: Lambert–Eaton myasthenic syndrome, symptomatic treatment, management, 3,4-diaminopyridine, amifampridine

Published
2011

3. A rare case of neuro‐ and otosyphilis in secondary syphilis

Author

Traidl, S., primary, Angela, Y., additional, Stender, A., additional, Kulberg, A., additional, Tronnier, M., additional, Prenzler, N.K., additional, Wattjes, M.P., additional, Kapp, A., additional, Stangel, M., additional, Schacht, V., additional, and Werfel, T., additional

Published
2021
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4. S1-Leitlinie: Lumbalpunktion und Liquordiagnostik: Zusammenfassung der Leitlinie der Deutschen Gesellschaft für Neurologie

Author

Tumani, H., Petereit, H. F., Gerritzen, A., Groß, C. C., Huss, A., Isenmann, S., Jesse, S., Khalil, M., Lewerenz, J., Leypoldt, F., Melzer, N., Meuth, S. G., Otto, M., Ruprecht, K., Sindern, E., Spreer, A., Stangel, M., Strik, H., Uhr, M., Vogelgsang, J., Wandinger, K.-P., Weber, T., Wick, M., Wildemann, B., Wiltfang, J., Woitalla, D., Zerr, I., and Zimmermann, T.

Published
2019
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7. S1 guidelines “lumbar puncture and cerebrospinal fluid analysis” (abridged and translated version)

Author

Tumani, H., primary, Petereit, H. F., additional, Gerritzen, A., additional, Gross, C. C., additional, Huss, A., additional, Isenmann, S., additional, Jesse, S., additional, Khalil, M., additional, Lewczuk, P., additional, Lewerenz, J., additional, Leypoldt, F., additional, Melzer, N., additional, Meuth, S. G., additional, Otto, M., additional, Ruprecht, K., additional, Sindern, E., additional, Spreer, A., additional, Stangel, M., additional, Strik, H., additional, Uhr, M., additional, Vogelgsang, J., additional, Wandinger, K.-P., additional, Weber, T., additional, Wick, M., additional, Wildemann, B., additional, Wiltfang, J., additional, Woitalla, D., additional, Zerr, I., additional, and Zimmermann, T., additional

Published
2020
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15. Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): a randomised, double-blind, placebo-controlled, phase 3 trial

Author

van Schaik, Ivo N, primary, Bril, Vera, additional, van Geloven, Nan, additional, Hartung, Hans-Peter, additional, Lewis, Richard A, additional, Sobue, Gen, additional, Lawo, John-Philip, additional, Praus, Michaela, additional, Mielke, Orell, additional, Durn, Billie L, additional, Cornblath, David R, additional, Merkies, Ingemar S J, additional, Sabet, A., additional, George, K., additional, Roberts, L., additional, Carne, R., additional, Blum, S., additional, Henderson, R., additional, Van Damme, P., additional, Demeestere, J., additional, Larue, S., additional, D'Amour, C., additional, Bril, V., additional, Breiner, A., additional, Kunc, P., additional, Valis, M., additional, Sussova, J., additional, Kalous, T., additional, Talab, R., additional, Bednar, M., additional, Toomsoo, T., additional, Rubanovits, I., additional, Gross-Paju, K., additional, Sorro, U., additional, Saarela, M., additional, Auranen, M., additional, Pouget, J., additional, Attarian, S., additional, Le Masson, G., additional, Wielanek-Bachelet, A., additional, Desnuelle, C., additional, Delmont, E., additional, Clavelou, P., additional, Aufauvre, D., additional, Schmidt, J., additional, Zschuentssch, J., additional, Sommer, C., additional, Kramer, D., additional, Hoffmann, O., additional, Goerlitz, C., additional, Haas, J., additional, Chatzopoulos, M., additional, Yoon, R., additional, Gold, R., additional, Berlit, P., additional, Jaspert-Grehl, A., additional, Liebetanz, D., additional, Kutschenko, A., additional, Stangel, M., additional, Trebst, C., additional, Baum, P., additional, Bergh, F., additional, Klehmet, J., additional, Meisel, A., additional, Klostermann, F., additional, Oechtering, J., additional, Lehmann, H., additional, Schroeter, M., additional, Hagenacker, T., additional, Mueller, D., additional, Sperfeld, A., additional, Bethke, F., additional, Drory, V., additional, Algom, A., additional, Yarnitsky, D., additional, Murinson, B., additional, Di Muzio, A., additional, Ciccocioppo, F., additional, Sorbi, S., additional, Mata, S., additional, Schenone, A., additional, Grandis, M., additional, Lauria, G., additional, Cazzato, D., additional, Antonini, G., additional, Morino, S., additional, Cocito, D., additional, Zibetti, M., additional, Yokota, T., additional, Ohkubo, T., additional, Kanda, T., additional, Kawai, M., additional, Kaida, K., additional, Onoue, H., additional, Kuwabara, S., additional, Mori, M., additional, Iijima, M., additional, Ohyama, K., additional, Baba, M., additional, Tomiyama, M., additional, Nishiyama, K., additional, Akutsu, T., additional, Yokoyama, K., additional, Kanai, K., additional, van Schaik, I.N., additional, Eftimov, F., additional, Notermans, N.C., additional, Visser, N., additional, Faber, C., additional, Hoeijmakers, J., additional, Rejdak, K., additional, Chyrchel-Paszkiewicz, U., additional, Casanovas Pons, C., additional, Alberti Aguiló, M., additional, Gamez, J., additional, Figueras, M., additional, Marquez Infante, C., additional, Benitez Rivero, S., additional, Lunn, M., additional, Morrow, J., additional, Gosal, D., additional, Lavin, T., additional, Melamed, I., additional, Testori, A., additional, Ajroud-Driss, S., additional, Menichella, D., additional, Simpson, E., additional, Chi-Ho Lai, E., additional, Dimachkie, M., additional, Barohn, R.J., additional, Beydoun, S., additional, Johl, H., additional, Lange, D., additional, Shtilbans, A., additional, Muley, S., additional, Ladha, S., additional, Freimer, M., additional, Kissel, J., additional, Latov, N., additional, Chin, R., additional, Ubogu, E., additional, Mumfrey, S., additional, Rao, T., additional, MacDonald, P., additional, Sharma, K., additional, Gonzalez, G., additional, Allen, J., additional, Walk, D., additional, Hobson-Webb, L., additional, and Gable, K., additional

Published
2018
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17. Monitoring von Blutparametern unter verlaufsmodifizierender MS-Therapie: Substanzspezifische Relevanz und aktuelle Handlungsempfehlungen

Author

Klotz, L., Berthele, A., Brück, W., Chan, A., Flachenecker, P., Gold, R., Haghikia, A., Hellwig, K., Hemmer, B., Hohlfeld, R., Korn, T., Kümpfel, T., Lang, M., Limmroth, V., Linker, R. A., Meier, U., Meuth, S. G., Paul, F., Salmen, A., Stangel, M., Tackenberg, B., Tumani, H., Warnke, C., Weber, M. S., Ziemssen, T., Zipp, F., and Wiendl, H.

Published
2016
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18. Costs and health-related quality of life in patients with NMO spectrum disorders and MOG-antibody-associated disease: CHANCE(NMO) study

Author

Hümmert, M.W., Schöppe, L.M., Bellmann-Strobl, J., Siebert, N., Paul, F., Duchow, A., Pellkofer, H., Kümpfel, T., Havla, J., Jarius, S., Wildemann, B., Berthele, A., Bergh, F.T., Pawlitzki, M., Klotz, L., Kleiter, I., Stangel, M., Gingele, S., Weber, M.S., Faiss, J.H., Pul, R., Walter, A., Zettl, U., Senel, M., Stellmann, J.P., Häußler, V., Hellwig, K., Ayzenberg, I., Aktas, O., Ringelstein, M., Schreiber-Katz, O., and Trebst, C.

Subjects
Function and Dysfunction of the Nervous System, health care economics and organizations
Abstract

OBJECTIVE: To evaluate costs and health-related quality of life (HRQoL) of neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein-antibody associated disease (MOGAD).MethodsIn this multicenter cross-sectional study, data on consumption of medical and non-medical resources and work ability were assessed via patient questionnaires. Costs were analyzed in EUR for 2018 from the societal perspective. HRQoL was captured by the EuroQoL EQ-5D-5L questionnaire. Clinical data were retrieved from the Neuromyelitis Optica Study Group (NEMOS) database. RESULTS: Two hundred twelve patients (80% women; median age 50 [19-83] years; median disease duration 7 [0-43] years; median Expanded Disability Status Scale [EDSS] 3.5 [0-8.5]; 66% Aquaporin-4 IgG, 22% MOG IgG positive, 12% double seronegative) were analyzed. The mean total annual per capita cost of illness accounted for EUR (Euro) 59 574 (95% CI 51 225 to 68 293; USD [United States dollars] 70 297, 95% CI 60 445 to 80 586), and the mean index value of the EQ-5D-5L was 0.693 (95% CI 0.65 to 0.73). The most important cost drivers were informal care costs (28% of total costs), indirect costs (23%) and drugs (16%), especially immunotherapeutics. Costs showed a positive correlation with disease severity (ρ=0.56, 95% CI 0.45 to 0.65); in the EDSS 6.5-8.5 subgroup the mean annual costs were EUR 129 687 (95% CI 101 946 to 160 336; USD 153 031, 95% CI 120 296 to 189 196). The HRQoL revealed a negative correlation to disease severity (ρ=-0.69, 95% CI -0.76 to -0.61); in the EDSS 6.5-8.5 subgroup the EQ-5D-5L mean index value was 0.195 (95% CI 0.13 to 0.28). Neither antibody status nor disease duration influenced the total annual costs or HRQoL. CONCLUSION: These German data from the era without approved preventive immunotherapies show enormous effects of the diseases on costs and quality of life. An early and cost-effective therapy should be provided to prevent long-term disability and preserve quality of life.

Published
2022

22. Aktuelles zur Therapieumstellung bei Multipler Sklerose

Author

Kolber, P., Luessi, F., Meuth, S.G., Klotz, L., Korn, T., Trebst, C., Tackenberg, B., Kieseier, B., Kümpfel, T., Fleischer, V., Tumani, H., Wildemann, B., Lang, M., Flachenecker, P., Meier, U., Brück, W., Limmroth, V., Haghikia, A., Hartung, H.-P., Stangel, M., Hohlfeld, R., Hemmer, B., Gold, R., Wiendl, H., and Zipp, F.

Published
2015
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30. Anti-LGI1 and anti-NMDA receptor encephalitis show distinct patterns of brain glucose metabolism in FDG-PET

Author

Wegner, F, primary, Nabavi, E, additional, Wilke, F, additional, Ben Tayeb, S, additional, Boeck, AL, additional, Trebst, C, additional, Stangel, M, additional, Voss, E, additional, Schrader, C, additional, Ahrens, J, additional, Leffler, A, additional, Rodriguez-Raecke, R, additional, Dengler, R, additional, Geworski, L, additional, Bengel, F, additional, and Berding, G, additional

Published
2014
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31. Efficacy and safety of IVIG in CIDP: Combined data of the PRIMA and PATH studies

Author

Merkies, Ingemar S. J., van Schaik, Ivo N., Léger, Jean-Marc, Bril, Vera, van Geloven, Nan, Hartung, Hans-Peter, Lewis, Richard A., Sobue, Gen, Lawo, John-Philip, Durn, Billie L., Cornblath, David R., De Bleecker, Jan L., Sommer, Claudia, Robberecht, Wim, Saarela, Mika, Kamienowski, Jerzy, Stelmasiak, Zbigniew, Tackenberg, Björn, Mielke, Orell, Sabet, A., George, K., Roberts, L., Carne, R., Blum, S., Henderson, R., Van Damme, P., Demeestere, J., Larue, S., D'Amour, C., Kunc, P., Valis, M., Sussova, J., Kalous, T., Talab, R., Bednar, M., Toomsoo, T., Rubanovits, I., Gross-Paju, K., Sorro, U., Saarela, M., Auranen, M., Pouget, J., Attarian, S., Masson, G. Le, Wielanek-Bachelet, A., Desnuelle, C., Delmont, E., Clavelou, P., Aufauvre, D., Schmidt, J., Zschuentzsch, J., Sommer, C., Kramer, D., Hoffmann, O., Goerlitz, C., Haas, J., Chatzopoulos, M., Yoon, R., Gold, R., Berlit, P., Jaspert-Grehl, A., Liebetanz, D., Kutschenko, A., Stangel, M., Trebst, C., Baum, P., Bergh, F., Klehmet, J., Meisel, A., Klostermann, F., Oechtering, J., Lehmann, H., Schroeter, M., Hagenacker, T., Mueller, D., Sperfeld, A., Bethke, F., Drory, V., Algom, A., Yarnitsky, D., Murinson, B., Di Muzio, A., Ciccocioppo, F., Sorbi, S., Mata, S., Schenone, A., Grandis, M., Lauria, G., Cazzato, D., Antonini, G., Morino, S., Cocito, D., Zibetti, M., Yokota, T., Ohkubo, T., Kanda, T., Kawai, M., Kaida, K., Onoue, H., Kuwabara, S., Mori, M., Iijima, M., Ohyama, K., Baba, M., Tomiyama, M., Nishiyama, K., Akutsu, T., Yokoyama, K., Kanai, K., van Schaik, I. N., Eftimov, F., Notermans, N. C., Visser, N., Faber, C., Hoeijmakers, J., Rejdak, K., Chyrchel-Paszkiewicz, U., Casanovas Pons, C., Antonia, M., Gamez, J., Salvado, M., Infante, C. Marquez, Benitez, S., Lunn, M., Morrow, J., Gosal, D., Lavin, T., Melamed, I., Testori, A., Ajroud-Driss, S., Menichella, D., Simpson, E., Lai, E. Chi-Ho, Dimachkie, M., Barohn, R. J., Beydoun, S., Johl, H., Lange, D., Shtilbans, A., Muley, S., Ladha, S., Freimer, M., Kissel, J., Latov, N., Chin, R., Ubogu, E., Mumfrey, S., Rao, T., Macdonald, P., Sharma, K., Gonzalez, G., Allen, J., Walk, D., Hobson-Webb, L., Gable, K., De Bleecker, J. L., Robberecht, W., Franques, J., Léger, J. -M., Morales, R. Juntas, Nguento, A., Schrey, Ch., Kamienowski, J., Stelmasiak, Z., Zwolińska, G., Neurology, AII - Infectious diseases, ANS - Neuroinfection & -inflammation, CSL Behring, Neurologian yksikkö, Clinicum, Department of Food and Nutrition, and HUS Neurocenter

Subjects
Research Report, Male, Outcome Assessment, efficacy, Medizin, Chronic inflammatory demyelinating polyneuropathy, CIDP, IVIG, PATH, PRIMA, Neuroscience (all), Neurology (clinical), INFLAMMATORY DEMYELINATING POLYNEUROPATHY, 3124 Neurology and psychiatry, law.invention, Grip strength, 0302 clinical medicine, Randomized controlled trial, law, Interquartile range, hemic and lymphatic diseases, Outcome Assessment, Health Care, 80 and over, Medicine and Health Sciences, Prospective Studies, Chronic Inflammatory Demyelinating, Prospective cohort study, Aged, 80 and over, education.field_of_study, General Neuroscience, Immunoglobulins, Intravenous, Middle Aged, OPEN-LABEL, humanities, 3. Good health, PREVALENCE, Europe, 030220 oncology & carcinogenesis, Cohort, POLYRADICULONEUROPATHY, Female, Intravenous, Polyneuropathy, Life Sciences & Biomedicine, Adult, medicine.medical_specialty, Efficacy, Neuroscience(all), Population, Clinical Neurology, Immunoglobulins, MAINTENANCE TREATMENT, 03 medical and health sciences, Young Adult, Double-Blind Method, Internal medicine, medicine, Journal Article, Humans, Immunologic Factors, INTRAVENOUS IMMUNOGLOBULIN, Aged, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, education, Science & Technology, business.industry, Neurosciences, 3112 Neurosciences, Research Reports, medicine.disease, PHASE-III, Health Care, cidp, ivig, path, prima, Neurosciences & Neurology, business, 030217 neurology & neurosurgery
Abstract

PRIMA Trial Investigators and the PATH Study Group., Intravenous immunoglobulin (IVIG) is a potential therapy for chronic inflammatory demyelinating polyneuropathy (CIDP). To investigate the efficacy and safety of the IVIG IgPro10 (Privigen) for treatment of CIDP, results from Privigen Impact on Mobility and Autonomy (PRIMA), a prospective, open‐label, single‐arm study of IVIG in immunoglobulin (Ig)‐naïve or IVIG pre‐treated subjects (NCT01184846, n = 28) and Polyneuropathy And Treatment with Hizentra (PATH), a double‐blind, randomized study including an open‐label, single‐arm IVIG phase in IVIG pre‐treated subjects (NCT01545076, IVIG restabilization phase n = 207) were analyzed separately and together (n = 235). Efficacy assessments included change in adjusted inflammatory neuropathy cause and treatment (INCAT) score, grip strength and Medical Research Council (MRC) sum score. Adverse drug reactions (ADRs) and ADRs/infusion were recorded. Adjusted INCAT response rate was 60.7% in all PRIMA subjects at Week 25 (76.9% in IVIG pre‐treated subjects) and 72.9% in PATH. In the pooled cohort (n = 235), INCAT response rate was 71.5%; median time to INCAT improvement was 4.3 weeks. No clear demographic differences were noticed between early (responding before Week 7, n = 148) and late responders (n = 21). In the pooled cohort, median change from baseline to last observation was −1.0 (interquartile range −2.0; 0.0) point for INCAT score; +8.0 (0.0; 20.0) kPa for maximum grip strength; +3.0 (1.0; 7.0) points for MRC sum score. In the pooled cohort, 271 ADRs were reported in 105 subjects (44.7%), a rate of 0.144 ADRs per infusion. This analysis confirms the efficacy and safety of IgPro10, a recently FDA‐approved IVIG for CIDP, in a population of mainly pre‐treated subjects with CIDP [Correction added on 14 March 2019 after first online publication: the INCAT response rate has been corrected.].

Published
2019

36. Cerebrospinal fluid parameters of B cell-related activity in patients with active disease during natalizumab therapy

Author

Harrer, A, primary, Tumani, H, additional, Niendorf, S, additional, Lauda, F, additional, Geis, C, additional, Weishaupt, A, additional, Kleinschnitz, C, additional, Rauer, S, additional, Kuhle, J, additional, Stangel, M, additional, Weber, F, additional, Uhr, M, additional, Linnebank, M, additional, Wildemann, B, additional, Jarius, S, additional, Guger, M, additional, Ayzenberg, I, additional, Chan, A, additional, Zettl, U, additional, Wiendl, H, additional, Pilz, G, additional, Hitzl, W, additional, Weber, JR, additional, and Kraus, J, additional

Published
2012
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37. The CSF JCV antibody index for diagnosis of natalizumab-associated PML: OS2124

Author

Warnke, C., von Geldern, G., Markwerth, P., Dehmel, T., Hoepner, R., Gold, R., Pawlita, M., Kümpfel, T., Mäurer, M., Stangel, M., Wegner, F., Hohlfeld, R., Straeten, V., Limmroth, V., Weber, T., Hermsen, D., Kleinschnitz, C., Hartung, H.-P., Wattjes, M. P., Svenningson, A., Major, E., Olsson, T., Kieseier, B. C., and Adams, O.

Published
2014

40. Sjögren Syndrom ist häufige Ursache von schweren Polyneuropathien mit Paresen

Author

Seeliger, T, Prenzler, N, Gingele, S, Seeliger, B, Körner, S, Thiele, T, Bönig, L, Sühs, KW, Witte, T, Stangel, M, and Skripuletz, T

Subjects
ddc: 610, 610 Medical sciences, Medicine
Abstract

Einleitung: Das Sjögren Syndrom ist eine autoimmune entzündliche Erkrankung bei der insbesondere Speicheldrüsen und Tränendrüsen angegriffen werden. Es können aber auch innere Organe und das periphere Nervensystem von der Entzündung betroffen sein. Das Ziel der vorliegenden[zum vollständigen Text gelangen Sie über die oben angegebene URL], 47. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 33. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 29. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

Published
2019
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