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- Alison F Brown and Lisa M Einhorn.
- From the Department of Anesthesiology, Duke University Medical Center, Durham, North Carolina.
- A A Pract. 2020 Apr 1; 14 (6): e01206.
AbstractSpinal muscular atrophy (SMA) is an autosomal recessive condition characterized by degeneration of the anterior horn cells of the spinal cord, which causes progressive muscle atrophy and weakness. SMA type 1 is the most common type and is associated with severe disability and early mortality. Concomitant restrictive respiratory physiology often manifests with significant implications for anesthetic management. Here, we describe a successful spinal anesthetic for orthopedic surgery in an SMA type 1 patient receiving intrathecal nusinersen maintenance therapy, an antisense oligonucleotide designed to increase expression of the survival motor neuron protein, and the first US Food and Drug Administration-approved drug to treat SMA.
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