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Disease severity affects quality of life of hereditary spastic paraplegia patients
Author(s) -
Klimpe S.,
Schüle R.,
Kassubek J.,
Otto S.,
Kohl Z.,
Klebe S.,
Klopstock T.,
Ratzka S.,
Karle K.,
Schöls L.
Publication year - 2012
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.2011.03443.x
Subject(s) - medicine , quality of life (healthcare) , paraplegia , disease , hereditary spastic paraplegia , physical therapy , gait disturbance , rating scale , spastic , gait , severity of illness , physical medicine and rehabilitation , spinal cord , psychiatry , cerebral palsy , psychology , developmental psychology , biochemistry , chemistry , nursing , gene , phenotype
Background and purpose: 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. Conclusion: 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.