Motor neuron involvement threatens survival in spinocerebellar ataxia type 1
Résumé
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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Neuropathology Appl Neurobio - 2023 - Coarelli - Motor neuron involvement threatens survival in spinocerebellar ataxia type.pdf (1.35 Mo)
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