Pathophysiology and Management of Fatigue in Neuromuscular Diseases

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Abstract

Fatigue is a major determinant of quality of life and motor function in patients affected by several neuromuscular diseases, each of them characterized by a peculiar physiopathology and the involvement of numerous interplaying factors. This narrative review aims to provide an overview on the pathophysiology of fatigue at a biochemical and molecular level with regard to muscular dystrophies, metabolic myopathies, and primary mitochondrial disorders with a focus on mitochondrial myopathies and spinal muscular atrophy, which, although fulfilling the definition of rare diseases, as a group represent a representative ensemble of neuromuscular disorders that the neurologist may encounter in clinical practice. The current use of clinical and instrumental tools for fatigue assessment, and their significance, is discussed. A summary of therapeutic approaches to address fatigue, encompassing pharmacological treatment and physical exercise, is also overviewed.

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Fatigue and fatigability in neurologic illnesses: proposal for a unified taxonomy.

Benzi Kluger, Lauren Krupp, Roger Enoka (2013)

Fatigue is commonly reported in many neurologic illnesses, including multiple sclerosis, Parkinson disease, myasthenia gravis, traumatic brain injury, and stroke. Fatigue contributes substantially to decrements in quality of life and disability in these illnesses. Despite the clear impact of fatigue as a disabling symptom, our understanding of fatigue pathophysiology is limited and current treatment options rarely lead to meaningful improvements in fatigue. Progress continues to be hampered by issues related to terminology and assessment. In this article, we propose a unified taxonomy and a novel assessment approach to addressing distinct aspects of fatigue and fatigability in clinical and research settings. This taxonomy is based on our current knowledge of the pathophysiology and phenomenology of fatigue and fatigability. Application of our approach indicates that the assessment and reporting of fatigue can be clarified and improved by utilizing this taxonomy and creating measures to address distinct aspects of fatigue and fatigability. We review the strengths and weaknesses of several common measures of fatigue and suggest, based on our model, that many research questions may be better addressed by using multiple measures. We also provide examples of how to apply and validate the taxonomy and suggest directions for future research.

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Adult norms for the Box and Block Test of manual dexterity.

Jeffrey K. Weber, N Kashman, G Volland … (1985)

The Box and Block Test, a test of manual dexterity, has been used by occupational therapists and others to evaluate physically handicapped individuals. Because the test lacked normative data for adults, the results of the test have been interpreted subjectively. The purpose of this study was to develop normative data for adults. Test subjects were 628 Normal adults (310 males and 318 females) from the seven-county Milwaukee area. Data on males and females 20 to 94 years old were divided into 12 age groups. Means, standard deviations, standard error, and low and high scores are reported for each five-year age group. These data will enable clinicians to objectively compare a patient's score to a normal population parameter.