Measuring quality of life impairment in periodic paralyses

Submitted by deb on Tue, 07/03/2012 – 18:45

 Measuring quality of life impairment in skeletal muscle channelopathies. 

Eur J Neurol. 2012 May 19. doi: 10.1111/j.1468-1331.2012.03751.x. [Epub ahead of print]

Sansone VA, Ricci C, Montanari M, Apolone G, Rose M, Meola G; INQoL Group. Source
Department of Neurology, University of Milan, IRCCS Policlinico San Donato, Milan, Italy. 

Abstract Link to pdf copy of entire article

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-36-66 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.