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Fatigue in neuromuscular disorders: focus on Guillain-Barré syndrome and Pompe disease.

de Vries JM, Hagemans ML, Bussmann JB, van der Ploeg AT, van Doorn PA - Cell. Mol. Life Sci. (2010)

Bottom Line: Substantial high prevalence rates of fatigue are reported in a wide range of neuromuscular disorders, such as Guillain-Barré syndrome and Pompe disease.Treatment of fatigue in neuromuscular disease starts with symptomatic treatment of the underlying disease.When symptoms of fatigue persist, non-pharmacological interventions, such as exercise and cognitive behavioral therapy, can be initiated.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Erasmus MC, University Medical Centre, Room Number EE 22-30, Dr. Molenwaterplein 50-60, Postbus 2040, 3000 CA Rotterdam, The Netherlands. j.m.devries@erasmusmc.nl

ABSTRACT
Fatigue accounts for an important part of the burden experienced by patients with neuromuscular disorders. Substantial high prevalence rates of fatigue are reported in a wide range of neuromuscular disorders, such as Guillain-Barré syndrome and Pompe disease. Fatigue can be subdivided into experienced fatigue and physiological fatigue. Physiological fatigue in turn can be of central or peripheral origin. Peripheral fatigue is an important contributor to fatigue in neuromuscular disorders, but in reaction to neuromuscular disease fatigue of central origin can be an important protective mechanism to restrict further damage. In most cases, severity of fatigue seems to be related with disease severity, possibly with the exception of fatigue occurring in a monophasic disorder like Guillain-Barré syndrome. Treatment of fatigue in neuromuscular disease starts with symptomatic treatment of the underlying disease. When symptoms of fatigue persist, non-pharmacological interventions, such as exercise and cognitive behavioral therapy, can be initiated.

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Related in: MedlinePlus

Model of fatigue in immune-mediated polyneuropathies. a Represents the hypothetical model of the mechanisms between the different domains in relation to physical training. b Is the model resulting from the correlations between the change scores of the different domains as a result of the training intervention. Thickness of lines between domains represents the strength of the relationship. Figures adapted from Ref. [12]
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Fig3: Model of fatigue in immune-mediated polyneuropathies. a Represents the hypothetical model of the mechanisms between the different domains in relation to physical training. b Is the model resulting from the correlations between the change scores of the different domains as a result of the training intervention. Thickness of lines between domains represents the strength of the relationship. Figures adapted from Ref. [12]

Mentions: A well-fitted rehabilitation program can help patients to use their energy more efficiently. For example, implementation of ergo-therapeutic devices aimed at improving the ease of performing daily activities may increase the level of energy and thereby lessen symptoms of fatigue. Also training in developing coping strategies aimed at balancing mental and physical activities and rest may improve the patient’s well-being. The potential effect of exercise programs on the level of fatigue was investigated in several studies [12, 25, 34, 79]. In patients recovered from GBS and in patients with ongoing but well-treated CIDP who suffer from severe fatigue, implementation of tailor-made exercise programs resulted in a reduction of 20% of self-reported fatigue. This improvement had been maintained at 2-year follow-up [33, 34]. These findings are consistent with results reported for healthy sedentary individuals (15%) and patients with multiple sclerosis (22%) after completion of an exercise program [75, 80]. However, the decrease in experienced fatigue in GBS after exercise therapy could not be explained by an increase in physical fitness [12]. Hypothetically, it could be that training also has a beneficial effect on the neuro-endocrine system and levels of neurotransmitters, which may reduce perception of fatigue. Additionally, recognition of the patients’ complaints of fatigue and the beneficial effect of support from fellow patients within the training group are also potential factors that can reduce experienced fatigue [12, 34]. Figure 3a, b visualizes the relationships among training, physical fitness, activity and psychosocial factors in a model. Figure 3a shows the relationships as was hypothesized before the start of the training study and Figure 3b the remodeling after analysis of the results of the study [12, 34].Fig. 3


Fatigue in neuromuscular disorders: focus on Guillain-Barré syndrome and Pompe disease.

de Vries JM, Hagemans ML, Bussmann JB, van der Ploeg AT, van Doorn PA - Cell. Mol. Life Sci. (2010)

Model of fatigue in immune-mediated polyneuropathies. a Represents the hypothetical model of the mechanisms between the different domains in relation to physical training. b Is the model resulting from the correlations between the change scores of the different domains as a result of the training intervention. Thickness of lines between domains represents the strength of the relationship. Figures adapted from Ref. [12]
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2824125&req=5

Fig3: Model of fatigue in immune-mediated polyneuropathies. a Represents the hypothetical model of the mechanisms between the different domains in relation to physical training. b Is the model resulting from the correlations between the change scores of the different domains as a result of the training intervention. Thickness of lines between domains represents the strength of the relationship. Figures adapted from Ref. [12]
Mentions: A well-fitted rehabilitation program can help patients to use their energy more efficiently. For example, implementation of ergo-therapeutic devices aimed at improving the ease of performing daily activities may increase the level of energy and thereby lessen symptoms of fatigue. Also training in developing coping strategies aimed at balancing mental and physical activities and rest may improve the patient’s well-being. The potential effect of exercise programs on the level of fatigue was investigated in several studies [12, 25, 34, 79]. In patients recovered from GBS and in patients with ongoing but well-treated CIDP who suffer from severe fatigue, implementation of tailor-made exercise programs resulted in a reduction of 20% of self-reported fatigue. This improvement had been maintained at 2-year follow-up [33, 34]. These findings are consistent with results reported for healthy sedentary individuals (15%) and patients with multiple sclerosis (22%) after completion of an exercise program [75, 80]. However, the decrease in experienced fatigue in GBS after exercise therapy could not be explained by an increase in physical fitness [12]. Hypothetically, it could be that training also has a beneficial effect on the neuro-endocrine system and levels of neurotransmitters, which may reduce perception of fatigue. Additionally, recognition of the patients’ complaints of fatigue and the beneficial effect of support from fellow patients within the training group are also potential factors that can reduce experienced fatigue [12, 34]. Figure 3a, b visualizes the relationships among training, physical fitness, activity and psychosocial factors in a model. Figure 3a shows the relationships as was hypothesized before the start of the training study and Figure 3b the remodeling after analysis of the results of the study [12, 34].Fig. 3

Bottom Line: Substantial high prevalence rates of fatigue are reported in a wide range of neuromuscular disorders, such as Guillain-Barré syndrome and Pompe disease.Treatment of fatigue in neuromuscular disease starts with symptomatic treatment of the underlying disease.When symptoms of fatigue persist, non-pharmacological interventions, such as exercise and cognitive behavioral therapy, can be initiated.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Erasmus MC, University Medical Centre, Room Number EE 22-30, Dr. Molenwaterplein 50-60, Postbus 2040, 3000 CA Rotterdam, The Netherlands. j.m.devries@erasmusmc.nl

ABSTRACT
Fatigue accounts for an important part of the burden experienced by patients with neuromuscular disorders. Substantial high prevalence rates of fatigue are reported in a wide range of neuromuscular disorders, such as Guillain-Barré syndrome and Pompe disease. Fatigue can be subdivided into experienced fatigue and physiological fatigue. Physiological fatigue in turn can be of central or peripheral origin. Peripheral fatigue is an important contributor to fatigue in neuromuscular disorders, but in reaction to neuromuscular disease fatigue of central origin can be an important protective mechanism to restrict further damage. In most cases, severity of fatigue seems to be related with disease severity, possibly with the exception of fatigue occurring in a monophasic disorder like Guillain-Barré syndrome. Treatment of fatigue in neuromuscular disease starts with symptomatic treatment of the underlying disease. When symptoms of fatigue persist, non-pharmacological interventions, such as exercise and cognitive behavioral therapy, can be initiated.

Show MeSH
Related in: MedlinePlus