title: Secondary changes of the motor endplate in Lambert-Eaton myasthenic syndrome: a quantitative study.
abstract: Underlying the Lambert-Eaton myasthenic syndrome (LEMS) is a decrease in the release of the neurotransmitter acetylcholine. Only few reports on light and transmission electron microscopical observations of motor endplates in LEMS are available and changes reported so far differ from those found in experimental blocking of acetylcholine release. We performed a quantitative study on intercostal muscle biopsies of five patients. The main light microscopical finding was an enlargement of the area of contact between nerve and muscle, which was interpreted as a compensatory phenomenon. At the ultrastructural level we found the mean postsynaptic area and membrane length of the neuromuscular junctions to be decreased, putatively due to small postsynaptic regions of newly created neuromuscular junctions. The secondary changes in LEMS endplates as seen in this study are similar to those reported in experimental blocking of acetylcholine release.
authors.affiliation: Laboratory for Neuromuscular Diseases, University Hospital, Rudolf Magnus Institute, Utrecht, The Netherlands.
authors.forename: L F
authors.initials: LF
authors.lastname: Hesselmans
authors.forename: F G
authors.initials: FG
authors.lastname: Jennekens
authors.forename: J
authors.initials: J
authors.lastname: Kartman
authors.forename: J H
authors.initials: JH
authors.lastname: Wokke
authors.forename: M
authors.initials: M
authors.lastname: de Visser
authors.forename: E G
authors.initials: EG
authors.lastname: Klaver-Krol
authors.forename: M
authors.initials: M
authors.lastname: DeBaets
authors.forename: F
authors.initials: F
authors.lastname: Spaans
authors.forename: H
authors.initials: H
authors.lastname: Veldman
mesh_terms: D000368:Aged; D001284:Atrophy / Q000473:pathology; D005260:Female; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009045:Motor Endplate / Q000473:pathology* / Q000648:ultrastructure; D009132:Muscles / Q000473:pathology; D009369:Neoplasms / Q000150:complications / Q000473:pathology; D009469:Neuromuscular Junction / Q000473:pathology / Q000648:ultrastructure