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Measuring quality of life impairment in skeletal muscle channelopathies
Author(s) -
Sansone V. A.,
Ricci C.,
Montanari M.,
Apolone G.,
Rose M.,
Meola G.
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.1468-1331.2012.03751.x
Subject(s) - myotonia , medicine , myotonia congenita , hypokalemic periodic paralysis , weakness , paralysis , myotonic dystrophy , periodic paralysis , anesthesia , muscle weakness , quality of life (healthcare) , physical therapy , physical medicine and rehabilitation , surgery , nursing
Background and purpose Fatigue and pain have been previously shown to be important determinants for decreasing quality of life ( Q o L ) in one report in patients with non‐dystrophic myotonia. The aims of our study were to assess Q o L in skeletal muscle channelopathies ( SMC ) using INQ o L (individualized Q o L ) and SF ‐36 questionnaires. Methods We administered INQ o L and SF ‐36 to 66 Italian patients with SMC (26: periodic paralysis, 36: myotonia congenita and 4: Andersen‐Tawil) and compared the results in 422 patients with myotonic dystrophies ( DM 1: 382; and DM 2: 40). Results (i) INQ o L index in SMC is similar to that in DM s ( P = 0.79). (ii) Patients with myotonia congenita have the worst perception of Q o L . (iii) Myotonia has the most detrimental effect on patients with myotonia congenita, followed by patients with DM 2 and then by patients with DM 1 and hyperkalemic periodic paralysis. (iv) Pain is a significant complaint in patients with myotonia congenita, hypokalemic periodic paralysis and DM 2 but not in DM 1. (v) Fatigue has a similar detrimental effect on all patient groups except for patients with hyperkalemic periodic paralysis in whom muscle weakness and myotonia more than fatigue affect Q o L perception. (vi) Muscle symptoms considered in INQ oL correlate with physical symptoms assessed by SF ‐36 ( R from −0.34 to −0.76). Conclusions QoL perception in patients with SMC is similar to that of patients with DM s, chronic multisystem disabling conditions. Our results provide information to target treatment and health care of these patients. The sensitivity of INQ o L to changes in Q o L in the SMC needs to be further explored in longitudinal studies.