Your use of the JSTOR archive indicates your acceptance of JSTOR's Terms and Conditions of Use, available at http://www.jstor.org/page/info/about/policies/terms.jsp. JSTOR's Terms and Conditions of Use provides, in part, that unless you have obtained prior permission, you may not download an entire issue of a journal or multiple copies of articles, and you may use content in the JSTOR archive o...

Skeletal muscle maintenance depends on motor innervation at neuromuscular junctions (NMJs). Multiple mechanisms contribute to NMJ repair and maintenance; however muscle stem cells (satellite cells, SCs), are deemed to have little impact on these processes. Therefore, the applicability of SC studies to attenuate muscle loss due to NMJ deterioration as observed in neuromuscular diseases and aging...

Respiratory complications are a common consequence of neuromuscular dysfunction in inherited and acquired neuromuscular diseases (NMD) affecting motor neurons. 2 These diseases include muscular dystrophies, myotonias, spinal muscular atrophies, amyotrophic lateral sclerosis (ALS), multiple sclerosis, Guillain Barré syndrome, and poliomyelitis. Though not an NMD, cerebral palsy (CP) is a functio...

As the medical community is engaging in more novel drug/biologic development and clinical testing for therapies for inherited neuromuscular disorders, new paradigms for decisions making in therapy development are being explored. A concerted effort is needed between the relevant stakeholders to share information, optimize efforts, minimize repeat of the same mistakes, and thereby move novel ther...

There is general consensus that consensus guidelines for care standards are an important tool to provide both healthcare professionals and the patient community with up to date information about all aspects of best practices in a specific disease area. The specific development of care guidelines for neuromuscular diseases poses a number of challenges. All neuromuscular diseases are rare and man...

Surface electromyography (sEMG) measures myoelectrical signals recorded from sensors placed on the skin surface. The non-invasive nature of sEMG makes it a potentially useful technology for studying diseases of muscle and nerve. Reviews published by the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) and the Therapeutics and Technology Assessment Subcommittee of the...

The broad category of neuromuscular diseases covers conditions that involve the weakness or wasting of the body muscles. These problems may occur in the spinal cord, the peripheral nerves or the muscle fibers. Some may be hereditary, while others are acquired. Commonly recognized conditions fall into the categories of myopathies, which are diseases of the muscle like muscular dystrophy, disorde...

The zebrafish model is an emerging system for the study of neuromuscular disorders. In the study of neuromuscular diseases, the integrity of the muscle membrane is a critical disease determinant. To date, numerous neuromuscular conditions display degenerating muscle fibers with abnormal membrane integrity; this is most commonly observed in muscular dystrophies. Evans Blue Dye (EBD) is a vital, ...