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Review
. 2022 Dec 15:443:120493.
doi: 10.1016/j.jns.2022.120493. Epub 2022 Nov 13.

Neuro-respiratory pathology in spinocerebellar ataxia

Debolina D Biswas  1 , Léa El Haddad  1 , Ronit Sethi  1 , Meredith L Huston  1 , Elias Lai  1 , Mariam M Abdelbarr  1 , Doreen Z Mhandire  1 , Mai K ElMallah  2
Affiliations
Review

Neuro-respiratory pathology in spinocerebellar ataxia

Debolina D Biswas et al. J Neurol Sci. .

Abstract

The spinocerebellar ataxias (SCA) are a heterogeneous group of neurodegenerative disorders with an autosomal dominant inheritance. Symptoms include poor coordination and balance, peripheral neuropathy, impaired vision, incontinence, respiratory insufficiency, dysphagia, and dysarthria. Although many patients with SCA have respiratory-related complications, the exact mechanism and extent of this pathology remain unclear. This review aims to provide an update on the recent clinical and preclinical scientific findings on neuropathology causing respiratory insufficiency in SCA.

Keywords: Hypoglossal nerve; Medulla; Phrenic; Respiratory failure; Spinocerebellar ataxia.

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Conflict of interest statement

Declaration of Competing Interest The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure. 1.
Figure. 1.. Overview of central respiratory control centers and the impact of SCA pathology on the medulla and spinal cord.
A schematic representation of the lower brainstem centers and the cervical spinal cord (C3-C5) centers contributing to the control of respiratory drive. (A) In the medulla, the origin of the respiratory control centers and bilateral projections (dotted arrows) from the respiratory rhythm generator (PBC) to XII motor nuclei. The XII nerve from the XII motor nuclei innervates the genioglossus in the tongue, a major muscle responsible of upper airway patency. (B) In the cervical spinal cord (C3-C5), the phrenic motor nuclei and its efferent projections (dotted arrow) from the PBC, VRG and DRG. The phrenic nerves from the phrenic motor nuclei innervate the diaphragm, a major respiratory muscle. SCA: Spinocerebellar ataxia, BC: Bötzinger Complex, PBC: Pre-Bötzinger Complex, VRG: Ventral Respiratory Group, NA: Nucleus Ambiguus, DRG: Dorsal Respiratory Group, XII: Hypoglossal, C3-C5: Origin of phrenic cervical nerves.
See this image and copyright information in PMC

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