Questionnaire survey to systematically assess and characterize quality of life limitations in the context of Lambert-Eaton myasthenia syndrome (LEMS).

• Conditions: G70; G73.1; Myasthenia gravis and other myoneural disorders; Lambert-Eaton syndrome

• Registration Number: DRKS00024527
• Lead Sponsor: Klinik für NeurologieNeuroCure Clinical Research Center (NCRC)Charité Universitätsmedizin BerlinCharité Campus Mitte

Brief Summary

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.

Detailed Description

Not available

Study Timeline

• Study Start: 2021-02-15
• Study Completion: 2021-05-31
• Results First Posted: 2024-02-29
• Last Update Posted: 19 August 2024

Recruitment & Eligibility

• Status: Complete
• Sex: All
• Target Recruitment: 47

Inclusion Criteria

Diagnosis of a LEMS

Exclusion Criteria

Under 18 years of age

Study & Design

• Study Type: observational
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Self-reported quality of life (questionnaire SF36). Summated scales for physical and psychological quality of life.

Secondary Outcome Measures

Name	Time	Method
1. Mya Quality of Life 15 (Mya Qol 15), self-reported information on disease severity (mild, moderate, severe).<br>2. Mya Qol 15, Myasthenia Gravis Activities of Daily Living Profile (MG-ADL).<br>3. MG-ADL, self-reported information on disease severity (mild, moderate, severe).<br>4. Mya Qol 15,self-reported history of onset of disease and time of diagnosis.<br>5. Mya Qol 15, ENRICHD Social Support Inventory – In German: ESSI-D.<br>6. Mya Qol 15, self-reported information on net income, loss of income due to MG (yes, no), pension payments (yes, no), and social security benefits (yes, no).<br>7. Mya Qol 15,self-reported information on side effects of drug therapies (yes, no).<br>8. HADS (Hospital Anxiety and Depression Scale) and comparison with data from the literature.<br>9. HADS, Mya Qol 15.<br>10. Chalder Fatigue Scale (CFQ) and comparison with data from the literature.<br>11. CFQ, Mya Qol 15.