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. 2007 Jul;254(7):941-5.
doi: 10.1007/s00415-006-0434-2. Epub 2007 Mar 2.

Fatigue: an important feature of late-onset Pompe disease

Marloes L C Hagemans  1 , Sabine P M van Schie, A Cecile J W Janssens, Pieter A van Doorn, Arnold J J Reuser, Ans T van der Ploeg
Affiliations

Fatigue: an important feature of late-onset Pompe disease

Marloes L C Hagemans et al. J Neurol. 2007 Jul.

Abstract

Objective: To investigate the prevalence and severity of fatigue in adult patients with Pompe disease.

Methods: The Fatigue Severity Scale (FSS) was assessed in an international population of 225 adults with Pompe disease, a metabolic disorder presenting as a slowly progressive proximal myopathy. The FSS scores were compared to those of healthy controls and the relationship between the level of fatigue and other patient characteristics was investigated.

Results: The mean age of the participants was 47 (SD 13) years and the mean disease duration 11 (SD 8) years. 43% used a wheelchair and 46% had respiratory support, 29% needed both. 67% of the participants had a FSS score > or =5, indicating severe fatigue. The mean FSS score was 5.2 (SD 1.5), which was significantly higher than that of healthy controls (p < 0.001). Fatigue was not related to age, sex or disease duration. Patients who used a wheelchair or respiratory support were on average more fatigued than those who did not (p = 0.01). However, of the patients who did not use these aids, 59% also had a FSS score > or =5. FSS scores were highest among patients who reported a high frequency of sleep disorders, but patients who never experienced sleep difficulties were also fatigued (mean FSS score = 4.8).

Conclusion: Fatigue is highly prevalent among both mildly and severely affected adult patients with Pompe disease. The FSS appears a useful tool in assessing fatigue in Pompe disease.

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Figures

Fig. 1
Fig. 1
Mean scores (95% CI) on the Fatigue Severity Scale of adult patients with Pompe disease from different countries. FSS scores differed significantly between countries when tested with ANOVA (p < 0.001). Scores of healthy controls were obtained from the literature [22]
Fig. 2
Fig. 2
Mean scores (95% CI) on the Fatigue Severity Scale of adult patients with Pompe disease related to wheelchair use and use of respiratory support
See this image and copyright information in PMC

Comment in

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