Measuring quality of life impairment in skeletal muscle channelopathies

V. A. Sansone, C. Ricci, M. Montanari, G. Apolone, M. Rose, G. Meola, M. Panzeri, C. Angelini, A. Palmieri, G. Siciliano, L. Volpi, M. Falorni, T. Mongini, L. Vercelli, L. Politano, S. Tozza, C. Solimene, R. Massa, M. B. Panico, V. PisaniM. Grandi, A. Toscano, O. Musumeci, C. Rodolico

Research output: Contribution to journalArticlepeer-review


Background and purpose: Fatigue and pain have been previously shown to be important determinants for decreasing quality of life (QoL) in one report in patients with non-dystrophic myotonia. The aims of our study were to assess QoL in skeletal muscle channelopathies (SMC) using INQoL (individualized QoL) and SF-36 questionnaires. Methods: We administered INQoL 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 (DM1: 382; and DM2: 40). Results: (i) INQoL index in SMC is similar to that in DMs (P = 0.79). (ii) Patients with myotonia congenita have the worst perception of QoL. (iii) Myotonia has the most detrimental effect on patients with myotonia congenita, followed by patients with DM2 and then by patients with DM1 and hyperkalemic periodic paralysis. (iv) Pain is a significant complaint in patients with myotonia congenita, hypokalemic periodic paralysis and DM2 but not in DM1. (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 QoL perception. (vi) Muscle symptoms considered in INQoL 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 DMs, chronic multisystem disabling conditions. Our results provide information to target treatment and health care of these patients. The sensitivity of INQoL to changes in QoL in the SMC needs to be further explored in longitudinal studies.

Original languageEnglish
Pages (from-to)1470-1476
Number of pages7
JournalEuropean Journal of Neurology
Issue number11
Publication statusPublished - Nov 2012


  • INQoL
  • Myotonic dystrophy
  • Non-dystrophic myotonias
  • Quality of life
  • SF-36
  • Skeletal muscle channelopathies

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Fingerprint Dive into the research topics of 'Measuring quality of life impairment in skeletal muscle channelopathies'. Together they form a unique fingerprint.

Cite this