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Hypersomnia in dystrophia myotonica: a neurophysiological and immunogenetic study
Author(s) -
Manni R.,
Zucca C.,
Martinetti M.,
Ottolini A.,
Lanzi G.,
Tarta A.
Publication year - 1991
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1991.tb05002.x
Subject(s) - multiple sleep latency test , narcolepsy , excessive daytime sleepiness , audiology , sleep (system call) , psychology , neurophysiology , medicine , sleep disorder , psychiatry , insomnia , neurology , computer science , operating system
Ten patients with dystrophia myotonica (8 adults and 2 prepubertal children), from three unrelated families, were investigated for diurnal sleepiness, using a sleep questionnaire and multiple sleep latency test (MSLT). Immunogenetic study was also carried out to assess the involvement of HLA region genes in modulating susceptibility to excessive diurnal sleepiness (EDS). EDS was reported by 5 patients and confirmed in each case by MSLT. In the whole patients group, mean daytime sleep latency was significantly shorter than in healthy controls matched for age and sex. At clinical or neurophysiological evaluation, EDS did not show the features associated with the narcoleptic type. In only one case hypersomnolence could be explained by underlying sleep‐disordered breathing. HLA patterns were different from those frequently observed in the narcoleptic or non‐narcoleptic types of hypersomnia. In patients with EDS, the frequency of the DQW1 and particularly of the DRW6‐DQW1 haplotype appeared to be over‐represented.