PURPOSE OF REVIEW: Aside from some inflammatory myopathies and very few genetic disorders, there are no therapies that make most patients with myopathies stronger. Consequently, the management of these patients can be frustrating for patients and their families as well as the clinicians taking care of them. Treatment of these patients must involve a comprehensive approach focused on limiting the secondary effects of skeletal muscle weakness, managing comorbidities associated with specific diseases, and, most importantly, optimizing patients' functional abilities and quality of life in terms of their ability to accomplish activities of daily living. While the approach to each patient differs depending on their disease, certain common themes can be addressed in each patient. This review highlights an approach centered on four conceptual themes ("the Four S's"): Strength therapies, Supportive care, Symptomatic therapies, and pSychological support. RECENT FINDINGS: Although relatively few well-designed studies have been done that highlight conservative management of patients with various myopathies, an emerging literature helps guide the clinician in certain key areas, especially in relation to cardiac and pulmonary management of these patients. SUMMARY: While disease-altering therapies have proven elusive for many muscle diseases, a multimodal approach to the conservative and supportive care of these patients can markedly improve their quality of life. Pharmacologic treatment options for specific myopathies will not be addressed in this article but are covered elsewhere in this issue of CONTINUUM.
PURPOSE OF REVIEW: Aside from some inflammatory myopathies and very few genetic disorders, there are no therapies that make most patients with myopathies stronger. Consequently, the management of these patients can be frustrating for patients and their families as well as the clinicians taking care of them. Treatment of these patients must involve a comprehensive approach focused on limiting the secondary effects of skeletal muscle weakness, managing comorbidities associated with specific diseases, and, most importantly, optimizing patients' functional abilities and quality of life in terms of their ability to accomplish activities of daily living. While the approach to each patient differs depending on their disease, certain common themes can be addressed in each patient. This review highlights an approach centered on four conceptual themes ("the Four S's"): Strength therapies, Supportive care, Symptomatic therapies, and pSychological support. RECENT FINDINGS: Although relatively few well-designed studies have been done that highlight conservative management of patients with various myopathies, an emerging literature helps guide the clinician in certain key areas, especially in relation to cardiac and pulmonary management of these patients. SUMMARY: While disease-altering therapies have proven elusive for many muscle diseases, a multimodal approach to the conservative and supportive care of these patients can markedly improve their quality of life. Pharmacologic treatment options for specific myopathies will not be addressed in this article but are covered elsewhere in this issue of CONTINUUM.
Authors: Shahinaz M Gadalla; Marie Lund; Ruth M Pfeiffer; Sanne Gørtz; Christine M Mueller; Richard T Moxley; Sigurdur Y Kristinsson; Magnus Björkholm; Fatma M Shebl; James E Hilbert; Ola Landgren; Jan Wohlfahrt; Mads Melbye; Mark H Greene Journal: JAMA Date: 2011-12-14 Impact factor: 56.272
Authors: David J Birnkrant; Katharine M D Bushby; Raouf S Amin; John R Bach; Joshua O Benditt; Michelle Eagle; Jonathan D Finder; Maninder S Kalra; John T Kissel; Anastassios C Koumbourlis; Richard M Kravitz Journal: Pediatr Pulmonol Date: 2010-08
Authors: Katharine Bushby; Richard Finkel; David J Birnkrant; Laura E Case; Paula R Clemens; Linda Cripe; Ajay Kaul; Kathi Kinnett; Craig McDonald; Shree Pandya; James Poysky; Frederic Shapiro; Jean Tomezsko; Carolyn Constantin Journal: Lancet Neurol Date: 2009-11-27 Impact factor: 44.182
Authors: Fieke M Cox; Victoria Delgado; Jan J Verschuuren; Bart E Ballieux; Jeroen J Bax; Axel R Wintzen; Umesh A Badrising Journal: J Neurol Date: 2009-10-08 Impact factor: 4.849