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Journal Articles Neuropathology and Applied Neurobiology Year : 2023

Motor neuron involvement threatens survival in spinocerebellar ataxia type 1

Abstract

This study aimed to explore life-threatening conditions inspinocerebellar ataxia type 1. Laryngeal dysfunction was severe in all patients and wasthe most life-threatening condition, causing swallowingand respiratory dysfunction and death as a result ofaspiratory pneumonia. Sleep disturbance may be caused by progressive loss ofpontine REM-on neurons. Investigation of the diaphragm suggested impairment inthe upper motor neuron pathway. Quantified neuronal loss was severe in the medullary teg-mentum, with especially massive neuronal loss in theinferior olivary nucleus and the hypoglossal motornucleus. Brainstem involvement with severe motor neuron deple-tion may explain life-threatening conditions in SCA1patients.
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hal-04523915 , version 1 (27-03-2024)

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Attribution - NonCommercial - NoDerivatives

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Giulia Coarelli, Maya Tchikviladzé, Pauline Dodet, Isabelle Arnulf, Perrine Charles, et al.. Motor neuron involvement threatens survival in spinocerebellar ataxia type 1. Neuropathology and Applied Neurobiology, 2023, 49 (2), ⟨10.1111/nan.12897⟩. ⟨hal-04523915⟩
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