Neuromuscular disorders : NMD
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Myasthenia gravis is characterized by muscle weakness and fatigue. As sustained muscle use increases the weakness, the value of physical training programs has previously been questioned. This is a review to clarify the safety and usefulness of systematic training in myasthenia gravis, based on a systematic search in available databases using the relevant key words. ⋯ This can improve both muscle strength and daily function. Type and intensity of systematic training should be adapted in the individual patient. A minimum of 150 min of exercise per week is recommended for myasthenia gravis patients with mild and moderate disease.
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Neuromuscul. Disord. · Mar 2021
Nusinersen treatment of older children and adults with spinal muscular atrophy.
The purpose of this study was to determine how effective administration of nusinersen was at improving motor function in older adolescent and adult patients with spinal muscular atrophy, using standardized motor outcome measures. Data were gathered through a retrospective chart review of older spinal muscular atrophy patients (ages 5-58) being treated at Rady Children's Hospital and the University of California, San Diego with nusinersen from April 2017-June 2019. Linear mixed effects analyses found that, for older children and adult patients with SMA 1, 2, and 3, motor scores as measured by the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders for non-sitters improved by 6 points (p = .01) and the Hammersmith Infant Neurological Examination-2 by 2.6% (p = .008) over the 22-month study period. ⋯ Older spinal muscular atrophy patients (5-58 years) being treated with nusinersen at our institutions are improving. Not only have symptoms stabilized, but their motor function has shown incremental improvements. Based on the results of this study, we suggested that nusinersen is well-tolerated and efficacious when treating older children and adult patients with spinal muscular atrophy 1, 2, and 3.