title: Disease severity affects quality of life of hereditary spastic paraplegia patients.
abstract: OBJECTIVE
  Hereditary spastic paraplegia (HSP) causes progressive gait disturbance because of degeneration of the corticospinal tract. To assess its impact on Health-Related Quality of Life (HRQoL), we analyzed the correlation of HRQoL with disease severity and clinical symptoms in HSP.
  
  
  METHODS
  HRQoL was assessed by the Short-Form 36 (SF-36) Mental and Physical Component summary scores (MCS and PCS) in 143 German patients with HSP. Disease severity was assessed by the Spastic Paraplegia Rating Scale (SPRS) and landmarks of walking ability. Patients with 'pure' or 'complicated' HSP were compared.
  
  
  RESULTS
  Higher SPRS scores indicating higher disease severity correlated significantly with lower PCS (r = -0.63; P < 0.0005) and MCS (r = -0.38; P < 0.0005) scores. MCS and PCS were reduced in patients with 'complicated' forms compared to 'pure' HSP and with decreasing walking ability.
  
  
  CONCLUSIONS
  HRQoL is substantially impaired in patients with HSP and decreases with disease severity and the presence of 'complicating' symptoms. Patients are most affected by the physical restraints of their disease, but mental health is impaired as well. HRQoL is a valid parameter in HSP that should be considered in upcoming therapeutical trials.
authors.affiliation: Department of Neurology, University Medical Center of the Johannes-Gutenberg University Mainz, Mainz, Germany. klimpe@uni-mainz.de
authors.forename: S
authors.initials: S
authors.lastname: Klimpe
authors.forename: R
authors.initials: R
authors.lastname: Schüle
authors.forename: J
authors.initials: J
authors.lastname: Kassubek
authors.forename: S
authors.initials: S
authors.lastname: Otto
authors.forename: Z
authors.initials: Z
authors.lastname: Kohl
authors.forename: S
authors.initials: S
authors.lastname: Klebe
authors.forename: T
authors.initials: T
authors.lastname: Klopstock
authors.forename: S
authors.initials: S
authors.lastname: Ratzka
authors.forename: K
authors.initials: K
authors.lastname: Karle
authors.forename: L
authors.initials: L
authors.lastname: Schöls
mesh_terms: D000328:Adult; D017668:Age of Onset; D005260:Female; D006304:Health Status; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D012720:Severity of Illness Index; D015419:Spastic Paraplegia, Hereditary / Q000150:complications* / Q000523:psychology*