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Anesthetic Management of Children and Adolescents With Giant Axonal Neuropathy: A Large Case Series
Journal article   Peer reviewed

Anesthetic Management of Children and Adolescents With Giant Axonal Neuropathy: A Large Case Series

Muhammad Yousef, Diana Bharucha-Goebel, Dimah Saade, Gilberto Averion, Carsten G. Bonnemann and Zenaide M. N. Quezado
A & A case reports, Vol.15(10), pp.e01539-e01539
10/01/2021
DOI: 10.1213/XAA.0000000000001539
PMID: 34695041

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Abstract

Giant axonal neuropathy (GAN) is a rare autosomal recessive neurodegenerative disorder caused by mutations in the GAN gene, which encodes for gigaxonin, a protein involved in intermediate filament processing in neural cells and fibroblasts. We report on 14 GAN patients who underwent 77 anesthetics during the conduct of an intrathecal gene transfer clinical trial from April 2015 to August 2020. We observed only a few nonsignificant perianesthetic complications. Our data expand the knowledge regarding safety of anesthesia for patients with this rare and potentially fatal disease and highlights the tolerability of shorter procedural sedation and anesthesia.
Anesthesiology Life Sciences & Biomedicine Science & Technology

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