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Successful Combination of Neuraxial and Regional Anesthesia in a Child With Advanced Spinal Muscular Atrophy Type 1 Receiving Maintenance Nusinersen Therapy: A Case Report.

Publication ,  Journal Article
Brown, AF; Einhorn, LM
Published in: A A Pract
April 2020

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.

Duke Scholars

Published In

A A Pract

DOI

EISSN

2575-3126

Publication Date

April 2020

Volume

14

Issue

6

Start / End Page

e01206

Location

United States

Related Subject Headings

  • Oligonucleotides
  • Muscular Atrophy, Spinal
  • Injections, Spinal
  • Humans
  • Child
  • Anesthesia, Conduction
 

Published In

A A Pract

DOI

EISSN

2575-3126

Publication Date

April 2020

Volume

14

Issue

6

Start / End Page

e01206

Location

United States

Related Subject Headings

  • Oligonucleotides
  • Muscular Atrophy, Spinal
  • Injections, Spinal
  • Humans
  • Child
  • Anesthesia, Conduction