title: 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.
abstract: BACKGROUND
  The burden of disease in Lambert-Eaton myasthenic syndrome (LEMS) patients is unclear. This study focused on the patient's perspective to obtain patient-reported information on clinical symptoms, burden of illness, impact of LEMS on activities of daily living (ADL), and management of LEMS.
  
  
  METHODS
  Semi-structured, face-to-face interviews with LEMS patients from two specialized centres in Germany between September and December 2010.
  
  
  RESULTS
  Twelve patients participated; mean age 66.7 ± 9.8 years. First symptoms occurred at age 52.5 ± 14.0 years. Mean time between first symptoms and diagnosis was 4.4 ± 6.2 years. Patients reported neuromuscular, cranial, and autonomic symptoms plus general fatigue. Two-thirds of patients reported 10 or more symptoms. The most frequent symptoms were leg weakness (91.7%) and general fatigue (83.3%). Restrictions in ADL were reported always or often in 75% of patients. Over half of the patients (n = 7) reported poor or very poor health status. Mean EQ-5D utility scores were 0.34 ± 0.35, with little day-to-day variation. Patients visited a number of different clinicians; most had been hospitalized at some point in the course of their disease. The most frequent drug treatments were 3,4-diaminopyridine (3,4-DAP) (83.3%) and pyridostigmine (41.5%). The study has several limitations, including small sample size and the potential influence of recall bias.
  
  
  CONCLUSIONS
  LEMS patients report long individual disease histories. Most patients suffer multiple symptoms which are frequently severe and troublesome, and almost all are restricted in ADL with poor health status. There is high utilization of healthcare resources from diagnosis to ongoing treatment. Physicians should be aware of this rare disease to ensure that patients receive an early diagnosis and prompt and appropriate treatment.
authors.affiliation: Klinik für Neurologie, Charité - Universitätsmedizin Berlin, Clinic for Neurology, Berlin, Germany. lutz.harms@charite.de
authors.forename: Lutz
authors.initials: L
authors.lastname: Harms
authors.forename: Jörn-Peter
authors.initials: JP
authors.lastname: Sieb
authors.forename: Angela E
authors.initials: AE
authors.lastname: Williams
authors.forename: Ryan
authors.initials: R
authors.lastname: Graham
authors.forename: Rita
authors.initials: R
authors.lastname: Shlaen
authors.forename: Volker
authors.initials: V
authors.lastname: Claus
authors.forename: Carmen
authors.initials: C
authors.lastname: Pfiffner
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D017281:Cost of Illness; D003430:Cross-Sectional Studies; D005260:Female; D005858:Germany; D006296:Health Services / Q000706:statistics & numerical data*; D006304:Health Status; D006801:Humans; D007407:Interviews as Topic; D015624:Lambert-Eaton Myasthenic Syndrome / Q000188:drug therapy / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011788:Quality of Life