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Open Access 29-02-2024 | Lambert-Eaton Myasthenic Syndrome | Original Communication

Burden of disease in Lambert-Eaton myasthenic syndrome: taking the patient’s perspective

Authors: Sophie Lehnerer, Meret Herdick, Regina Stegherr, Lea Gerischer, Frauke Stascheit, Maike Stein, Philipp Mergenthaler, Sarah Hoffmann, Andreas Meisel

Published in: Journal of Neurology | Issue 5/2024

Abstract

Background

Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune-mediated neuromuscular disorder leading to muscle weakness, autonomic dysregulation and hyporeflexia. Psychosocial well-being is affected. Previously, we assessed burden of disease for Myasthenia gravis (MG). Here, we aim to elucidate burden of disease by comparing health-related quality of life (HRQoL) of patients with LEMS to the general population (genP) as well as MG patients.

Methods

A questionnaire-based survey included sociodemographic and clinical data along with standardized questionnaires, e.g. the Short Form Health (SF-36). HRQoL was evaluated through matched-pairs analyses. Participants from a general health survey served as control group.

Results

46 LEMS patients matched by age and gender were compared to 92 controls from the genP and a matched cohort of 92 MG patients. LEMS participants showed lower levels of physical functioning (SF-36 mean 34.2 SD 28.6) compared to genP (mean 78.6 SD 21.1) and MG patients (mean 61.3 SD 31.8). LEMS patients showed lower mental health sub-scores compared to genP (SF-36 mean 62.7 SD 20.2, vs. 75.7 SD 15.1) and MG patients (SF-36 mean 62.7 SD 20.2, vs. 66.0 SD 18.). Depression, anxiety and fatigue were prevalent. Female gender, low income, lower activities of daily living, symptoms of depression, anxiety and fatigue were associated with a lower HRQoL in LEMS.

Discussion

HRQoL is lower in patients with LEMS compared to genP and MG in a matched pair-analysis. The burden of LEMS includes economic and social aspects as well as emotional well-being.

Trial Registration Information

drks.de: DRKS00024527, submitted: February 02, 2021, https://drks.de/search/en/trial/DRKS00024527.

Available only for authorised users

Abenroth DC, Smith AG, Greenlee JE, Austin SD, Clardy SL (2017) Lambert-Eaton myasthenic syndrome: epidemiology and therapeutic response in the national veterans affairs population. Muscle Nerve 56:421–426CrossRefPubMed

Basta IZ, Pekmezovic TD, Peric SZ, Kisic-Tepavcevic DB, Rakocevic-Stojanovic VM, Stevic ZD, Lavrnic DV (2012) Assessment of health-related quality of life in patients with myasthenia gravis in Belgrade (Serbia). Neurol Sci 33:1375–1381CrossRefPubMed

Bjelland I, Dahl AA, Haug TT, Neckelmann D (2002) The validity of the hospital anxiety and depression scale. An updated literature review. J Psychosom Res 52:69–77CrossRefPubMed

Burns TM, Conaway MR, Cutter GR, Sanders DB, Muscle Study G (2008) Less is more, or almost as much: a 15-item quality-of-life instrument for myasthenia gravis. Musc Nerve 38:957–963CrossRef

Chalder T, Berelowitz G, Pawlikowska T, Watts L, Wessely S, Wright D, Wallace EP (1993) Development of a fatigue scale. J Psychosom Res 37:147–153CrossRefPubMed

Harms L, Sieb JP, Williams AE, Graham R, Shlaen R, Claus V, Pfiffner C (2012) Long-term disease history, clinical symptoms, health status, and healthcare utilization in patients suffering from Lambert Eaton myasthenic syndrome: results of a patient interview survey in Germany. J Med Econ 15:521–530CrossRefPubMed

Herrmann-Lingen C, Buss U, Snaith RP (2010) Hospital anxiety and depression scale - deutsche version. Deutsche adaptation der hospital anxiety and depression scale (HADS) von R. P. Snaith und A. S. Zigmond

ENRICHD Investigators (2000) Enhancing recovery in coronary heart disease patients (ENRICHD): study design and methods. The ENRICHD investigators. Am Heart J 139:1–9CrossRef

Jackson C (2015) The chalder fatigue scale (CFQ 11). Occup Med (Lond) 65:86CrossRefPubMed

10.

Janssens AC, van Doorn PA, de Boer JB, Kalkers NF, van der Meche FG, Passchier J, Hintzen RQ (2003) Anxiety and depression influence the relation between disability status and quality of life in multiple sclerosis. Mult Scler 9:397–403CrossRefPubMed

11.

Jeong A, Min JH, Kang YK, Kim J, Choi M, Seok JM, Kim BJ (2018) Factors associated with quality of life of people with Myasthenia Gravis. PLoS One 13:e0206754CrossRefPubMedPubMedCentral

12.

Kamtsiuris P, Lange M, Hoffmann R, Schaffrath Rosario A, Dahm S, Kuhnert R, Kurth BM (2013) The first wave of the German Health Interview and Examination Survey for Adults (DEGS1): sample design, response, weighting and representativeness. Bundesgesundheitsblatt Gesundheitsforschung Gesundheitsschutz 56:620–630CrossRefPubMed

13.

Kendel F, Spaderna H, Sieverding M, Dunkel A, Lehmkuhl E, Hetzer R, Regitz-Zagrosek V (2011) Eine deutsche adaptation des ENRICHD social support inventory (ESSI). Diagnostica 57:99–106CrossRef

14.

Lampert T, Kroll L, Muters S, Stolzenberg H (2013) Measurement of socioeconomic status in the German Health Interview and Examination Survey for Adults (DEGS1). Bundesgesundheitsblatt Gesundheitsforschung Gesundheitsschutz 56:631–636CrossRefPubMed

15.

Lechert Y, Schroedter JPL (2006) Die Umsetzung der Bildungsklassifikation CASMIN für die Volkszählung 1970, die Mikrozensus- Zusatzerhebung 1971 und die Mikrozensen 1976–2004. ZUMA-Methodenbericht 2006/12.

16.

Lehnerer S, Jacobi J, Schilling R, Grittner U, Marbin D, Gerischer L, Stascheit F, Krause M, Hoffmann S, Meisel A (2022) Burden of disease in myasthenia gravis: taking the patient’s perspective. J Neurol 269:3050–3063CrossRefPubMed

17.

Lennon VA, Kryzer TJ, Griesmann GE, O’Suilleabhain PE, Windebank AJ, Woppmann A, Miljanich GP, Lambert EH (1995) Calcium-channel antibodies in the Lambert-Eaton syndrome and other paraneoplastic syndromes. N Engl J Med 332:1467–1474CrossRefPubMed

18.

Lipka AF, Boldingh MI, van Zwet EW, Schreurs MWJ, Kuks JBM, Tallaksen CM, Titulaer MJ, Verschuuren J (2020) Long-term follow-up, quality of life, and survival of patients with Lambert-Eaton myasthenic syndrome. Neurology 94:e511–e520CrossRefPubMedPubMedCentral

19.

Liu Y, Xi J, Zhou L, Wu H, Yue D, Zhu W, Lin J, Lu J, Zhao C, Qiao K (2019) Clinical characteristics and long term follow-up of Lambert-Eaton myasthenia syndrome in patients with and without small cell lung cancer. J Clin Neurosci 65:41–45CrossRefPubMed

20.

Maddison P, Gozzard P, Grainge MJ, Lang B (2017) Long-term survival in paraneoplastic Lambert-Eaton myasthenic syndrome. Neurology 88:1334–1339CrossRefPubMed

21.

Maddison P, Newsom-Davis J, Mills KR, Souhami RL (1999) Favourable prognosis in Lambert-Eaton myasthenic syndrome and small-cell lung carcinoma. Lancet 353:117–118CrossRefPubMed

22.

Mantegazza R, Meisel A, Sieb JP, Le Masson G, Desnuelle C, Essing M (2015) The European LEMS registry: baseline demographics and treatment approaches. Neurol Ther 4:105–124CrossRefPubMedPubMedCentral

23.

Martin A, Staufenbiel T, Gaab J, Rief W, Brähler E (2010) Messung chronischer erschöpfung—teststatistische prüfung der fatigue skala (FS). Z Klin Psychol Psychother 39:33–44CrossRef

24.

Meisel A, Sieb JP, Le Masson G, Postila V, Sacconi S (2022) The European Lambert-Eaton myasthenic syndrome registry: long-term outcomes following symptomatic treatment. Neurol Ther 11:1071–1083CrossRefPubMedPubMedCentral

25.

Morfeld M, Kirchberger I, Bullinger M (2011) SF-36 fragebogen zum gesundheitszustand: deutsche version des short form-36 health survey. Hogrefe

26.

Motomura M, Lang B, Johnston I, Palace J, Vincent A, Newsom-Davis J (1997) Incidence of serum anti-P/O-type and anti-N-type calcium channel autoantibodies in the Lambert-Eaton myasthenic syndrome. J Neurol Sci 147:35–42CrossRefPubMed

27.

Moussavi S, Chatterji S, Verdes E, Tandon A, Patel V, Ustun B (2007) Depression, chronic diseases, and decrements in health: results from the World Health Surveys. Lancet 370:851–858CrossRefPubMed

28.

Nakao YK, Motomura M, Fukudome T, Fukuda T, Shiraishi H, Yoshimura T, Tsujihata M, Eguchi K (2002) Seronegative Lambert-Eaton myasthenic syndrome: study of 110 Japanese patients. Neurology 59:1773–1775CrossRefPubMed

29.

O'Neill JH, Murray NM, Newsom-Davis J (1988) The Lambert-Eaton myasthenic syndrome. A review of 50 cases. Brain 111 (Pt 3):577–596

30.

Oh SJ (2020) Amifampridine to treat Lambert-Eaton myasthenic syndrome. Drugs Today (Barc) 56:623–641CrossRefPubMed

31.

RCoreTeam (2022) A language and environment for statistical computing. In:R Foundation for Statistical Computing, Vienna, Austria

32.

Rubin DB (2009) Multiple imputation for nonresponse in surveys. Wiley

33.

Ruiter AM, Strijbos E, de Meel RHP, Lipka AF, Raadsheer WF, Tannemaat MR, Verschuuren J (2021) Accuracy of patient-reported data for an online patient registry of autoimmune myasthenia gravis and Lambert-Eaton myasthenic syndrome. Neuromuscul Disord 31:622–632CrossRefPubMed

34.

Statistisches Bundesamt (2023) Maßzahlen zu Ehescheidungen 2000 bis 2022. In:Statistisches Bundesamt (Destatis)

35.

Titulaer MJ, Lang B, Verschuuren JJ (2011) Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies. Lancet Neurol 10:1098–1107CrossRefPubMed

36.

Titulaer MJ, Maddison P, Sont JK, Wirtz PW, Hilton-Jones D, Klooster R, Willcox N, Potman M, Sillevis Smitt PA, Kuks JB, Roep BO, Vincent A, van der Maarel SM, van Dijk JG, Lang B, Verschuuren JJ (2011) Clinical Dutch-English Lambert-Eaton Myasthenic syndrome (LEMS) tumor association prediction score accurately predicts small-cell lung cancer in the LEMS. J Clin Oncol 29:902–908CrossRefPubMed

37.

Titulaer MJ, Wirtz PW, Kuks JB, Schelhaas HJ, van der Kooi AJ, Faber CG, van der Pol WL, de Visser M, Sillevis Smitt PA, Verschuuren JJ (2008) The Lambert-Eaton myasthenic syndrome 1988–2008: a clinical picture in 97 patients. J Neuroimmunol 201–202:153–158CrossRefPubMed

38.

Twork S, Wiesmeth S, Klewer J, Pohlau D, Kugler J (2010) Quality of life and life circumstances in German myasthenia gravis patients. Health Qual Life Outcomes 8:129CrossRefPubMedPubMedCentral

39.

Ware JEJ, Sherbourne CD (1992) The MOS 36-ltem short-form health survey (SF-36): I. Conceptual Framework and Item Selection. Med Care 30:473–483CrossRefPubMed

40.

Wirtz PW, Smallegange TM, Wintzen AR, Verschuuren JJ (2002) Differences in clinical features between the Lambert-Eaton myasthenic syndrome with and without cancer: an analysis of 227 published cases. Clin Neurol Neurosurg 104:359–363CrossRefPubMed

41.

Wirtz PW, Sotodeh M, Nijnuis M, Van Doorn PA, Van Engelen BG, Hintzen RQ, De Kort PL, Kuks JB, Twijnstra A, De Visser M, Visser LH, Wokke JH, Wintzen AR, Verschuuren JJ (2002) Difference in distribution of muscle weakness between myasthenia gravis and the Lambert-Eaton myasthenic syndrome. J Neurol Neurosurg Psychiatry 73:766–768CrossRefPubMedPubMedCentral

42.

Wirtz PW, van Dijk JG, van Doorn PA, van Engelen BG, van der Kooi AJ, Kuks JB, Twijnstra A, de Visser M, Visser LH, Wokke JH, Wintzen AR, Verschuuren JJ (2004) The epidemiology of the Lambert-Eaton myasthenic syndrome in the Netherlands. Neurology 63:397–398CrossRefPubMed

43.

Wolfe GI, Herbelin L, Nations SP, Foster B, Bryan WW, Barohn RJ (1999) Myasthenia gravis activities of daily living profile. Neurology 52:1487–1489CrossRefPubMed

44.

Young JD, Leavitt JA (2016) Lambert-Eaton myasthenic syndrome: ocular signs and symptoms. J Neuroophthalmol 36:20–22CrossRefPubMed

45.

Zigmond AS, Snaith RP (1983) The hospital anxiety and depression scale. Acta Psychiatr Scand 67:361–370CrossRefPubMed

Title: Burden of disease in Lambert-Eaton myasthenic syndrome: taking the patient’s perspective
Authors: Sophie Lehnerer
Meret Herdick
Regina Stegherr
Lea Gerischer
Frauke Stascheit
Maike Stein
Philipp Mergenthaler
Sarah Hoffmann
Andreas Meisel
Publication date: 29-02-2024
Publisher: Springer Berlin Heidelberg
Keywords: Lambert-Eaton Myasthenic Syndrome
Myasthenia Gravis
Published in: Journal of Neurology / Issue 5/2024
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-024-12206-6

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Burden of disease in Lambert-Eaton myasthenic syndrome: taking the patient’s perspective

Abstract

Background

Methods

Results

Discussion

Trial Registration Information

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Results

Discussion

Trial Registration Information

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