Facioscapulohumeral muscular dystrophy

The Xp21-Linked Muscular Dystrophies Emery-Dreifuss Muscular Dystrophy (EDMD) Facioscapulohumeral Muscular Dystrophy (FSH)... 

Lunt, P. (1994). Report of the sixth international workshop on facioscapulohumeral muscular dystrophy San Francisco 11 November 1992 and current guidelines for clinical application of DNA rearrangements at locus D4S810. Neuromusc. Disord. 4, 83-86. 

Facioscapulohumeral muscular dystrophy (FSHD) Fragile X-associated tremor/ ataxia syndrome (FXTAS) Prader-Willi syndrome, Rett syndrome Spinocerebellar atrophy type 8 (SCA8) Paraneoplastic neurological disorders (PND)... 

Wijmenga C, Hewitt JE, Sandkuijl LA, Qark LN, Wright TJ et al (1992) Chromosome 4q DNA rearrangements associated with facioscapulohumeral muscular dystrophy. Nat Genet 2 26-30... 

Facioscapulohumeral muscular dystrophy or Lan-douzy-De jerine dystrophy differs from the more common types in that it involves primarily the upper extremities, face muscles, and shoulder girdle. The condition starts later in life (usually by age 10) and may appear... 

Wijmenga C, et al. (1990). Facioscapulohumeral muscular dystrophy gene in Dutch families is not linked to markers for familial adenomatous polyposis on the long arm of chromosome 5. /. Neurol. Sci. 95(2) 225-229. 

Muscle E ovides form, support, stability, and capability of directed movement to the body Becker muscular dystrophy Duchenne muscular dystrophy Emery-Dreifuss muscular dystrophy Facioscapulohumeral muscular dystrophy Limb-girdle muscular dystrophy Myotoniacongenita Myotonic dystrophy Biodegradable polymers such as poly (lactide-co-glycolide), poly (caprolac-tone), polyphosphazenes, and conposites of diese polymers with each odier and various inorganic conpounds AVI-4658, Eteplirsen... 

Few, if any, patients with NMD should be left to develop unexpected ventilatory failure as appropriate assessment, self-management education, and follow-up will identify disease progression and risk of respiratory complications. When ventilatory failure occurs, tracheostomy tubes can be avoided, for the most part, irrespective of the degree of ventilator dependence, with the exception of those with insufficient bulbar-innervated musculature for speech, deglutition, and airway protection. Those with indwelling tracheostomy tubes should be offered decannulation as part of their rehabilitation, irrespective of the extent of their respiratory muscle failure. The only exceptions to this therapy are patients with advanced bulbar ALS or those with rare facioscapulohumeral muscular dystrophy, who lose all bulbar-innervated muscle function and aspirate saliva to the extent of Sao2 remaining below 95% (13). 

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