title: Evaluation of electrophysiological and clinical tests in an exploratory trial of Org 2766 in motor neuron disease.
abstract: Twenty four patients with motor neuron disease (MND) participated in a double-blind, placebo-controlled trial with the ACTH 4-9 analog, Org 2766. Patients were examined three times during an 8 week treatment period, using a summated score for several manually and functionally tested muscles (sum score), myometry, jitter, fibre density (FD), macro motor unit potential (MUP), and supramaximal evoked muscle action potentials. No differences were found between Org 2766 and placebo treated patients. In an open 1 yr follow-up study, 5 out of 13 patients treated with Org 2766 died; the others showed continued progression of weakness. The methods used for assessment of muscle function were compared. The highest interest reliability was obtained in the sum score and myometry. Mean differences that might be detectable were relatively small for the sum score and myometry, and large for FD and MUP. We concluded that clinical function testing and myometry are superior to electromyographic measurements for assessment of changes in MND patients.
authors.affiliation: Division of Neuromuscular Diseases, Rudolf Magnus Institute, Utrecht, The Netherlands.
authors.forename: L F
authors.initials: LF
authors.lastname: Hesselmans
authors.forename: G H
authors.initials: GH
authors.lastname: Wieneke
authors.forename: P L
authors.initials: PL
authors.lastname: Oey
authors.forename: D M
authors.initials: DM
authors.lastname: Groenhout
authors.forename: Y
authors.initials: Y
authors.lastname: van der Graaf
authors.forename: W H
authors.initials: WH
authors.lastname: Gispen
authors.forename: F G
authors.initials: FG
authors.lastname: Jennekens
mesh_terms: D000200:Action Potentials; D000324:Adrenocorticotropic Hormone / Q000031:analogs & derivatives* / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D000927:Anticonvulsants / Q000627:therapeutic use; D004311:Double-Blind Method; D004594:Electrophysiology / Q000379:methods; D005071:Evoked Potentials; D005500:Follow-Up Studies; D006801:Humans; D007537:Isometric Contraction / Q000187:drug effects; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000188:drug therapy* / Q000503:physiopathology*; D015999:Multivariate Analysis; D009132:Muscles / Q000503:physiopathology*; D010446:Peptide Fragments / Q000627:therapeutic use*; D010919:Placebos; D013997:Time Factors
chemical_list: D000927:Anticonvulsants; D010446:Peptide Fragments; D010919:Placebos; C013765:Org 2766; D000324:Adrenocorticotropic Hormone