Successful Combination of Neuraxial and Regional Anesthesia in a Child With Advanced Spinal Muscular Atrophy Type 1 Receiving Maintenance Nusinersen Therapy: A Case Report.
Journal Article (Journal Article)
Spinal 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.
Full Text
Duke Authors
Cited Authors
- Brown, AF; Einhorn, LM
Published Date
- April 2020
Published In
Volume / Issue
- 14 / 6
Start / End Page
- e01206 -
PubMed ID
- 32784322
Electronic International Standard Serial Number (EISSN)
- 2575-3126
Digital Object Identifier (DOI)
- 10.1213/XAA.0000000000001206
Language
- eng
Conference Location
- United States