title: Decreased nocturnal movements in patients with facioscapulohumeral muscular dystrophy.
abstract: OBJECTIVE
  Reduced mobility during sleep characterizes a variety of movement disorders and neuromuscular diseases. Facioscapulohumeral muscular dystrophy (FSHD) is the third most common form of muscular dystrophy in the general population, and people with FSHD have poor sleep quality. The aims of the present study were to evaluate nocturnal motor activity in patients with FSHD by means of videopolysomnography and to verify whether activity was associated with modifications in sleep structure.
  
  
  METHODS
  We enrolled 32 adult patients affected by genetically confirmed FSHD (18 women and 14 men, mean age 45.1 +/- 13.4 years) and 32 matched control subjects, (18 women and 14 men, mean age 45.5 +/- 11.4 years). Major body movements (MBM) were scored in videopolygraphic recordings in accordance with established criteria. An MBM index was calculated (number of MBM per hour of sleep).
  
  
  RESULTS
  The FSHD group showed a decrease in the MBM index (FSHD: 1.2 +/- 1.1; control subjects: 2.3 +/- 1.2, analysis of variance F = 13.672; p = 0.008). The sleep pattern of patients with FSHD, as compared with that of controls, was characterized by longer sleep latencies, shorter sleep durations, an increased percentage of wake during sleep, and a decreased percentage of rapid eye movement sleep. In the patient group, the MBM index was inversely correlated with severity of disease (Spearman test: r30 = -0.387; p < 0.05).
  
  
  CONCLUSIONS
  The present findings suggest that patients with FSHD have a reduced number of nocturnal movements, which is related to disease severity. Reduced movement in bed may contribute to the sleep modifications observed in these patients.
authors.affiliation: Department of Neurosciences, Catholic University Rome, Policlinico Universitario A. Gemelli, Italy. dellamarca@rm.unicatt.it
authors.forename: Giacomo
authors.initials: G
authors.lastname: Della Marca
authors.forename: Roberto
authors.initials: R
authors.lastname: Frusciante
authors.forename: Serena
authors.initials: S
authors.lastname: Dittoni
authors.forename: Catello
authors.initials: C
authors.lastname: Vollono
authors.forename: Anna
authors.initials: A
authors.lastname: Losurdo
authors.forename: Elisa
authors.initials: E
authors.lastname: Testani
authors.forename: Emanuele
authors.initials: E
authors.lastname: Scarano
authors.forename: Salvatore
authors.initials: S
authors.lastname: Colicchio
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Iannaccone
authors.forename: Pietro A
authors.initials: PA
authors.lastname: Tonali
authors.forename: Enzo
authors.initials: E
authors.lastname: Ricci
mesh_terms: D002940:Circadian Rhythm; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009043:Motor Activity; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000150:complications*; D017286:Polysomnography / Q000379:methods / Q000706:statistics & numerical data; D012720:Severity of Illness Index; D007319:Sleep Initiation and Maintenance Disorders / Q000150:complications*; D012895:Sleep, REM; D014743:Videotape Recording