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August 1997

Can We Treat Respiratory Failure in Friedreich Ataxia?

Author Affiliations

From the Behavioral Neurology, Neurobiology, and Neuropsychology Unit (Dr Botez), the Sleep Research Laboratory of the Pneumology Service (Dr Mayer), and the Research Laboratory of the Department of Anesthesia (Drs Bellemare and Couture), Hôtel-Dieu Hospital, Montreal, Quebec.

Arch Neurol. 1997;54(8):1030-1033. doi:10.1001/archneur.1997.00550200086015

Background:  Neurochemical disorders associated with spinocerebellar ataxias are multiple.

Objective:  To use replacement and neuroprotective therapy in a case of severe respiratory failure in Friedreich ataxia.

Patient and Treatment:  A 44-year-old man with severe Friedreich ataxia displayed arduous periodic breathing associated with minor desaturation as well as obstructive or mixed apneas associated with severe desaturation during the night. He was given oxitriptan (5-hydroxy-L-tryptophan) (1500 mg/d), thiamine hydrochloride (100 mg/d), and amantadine hydrochloride (100 mg/d). The first sleep study was conducted during the night before treatment, whereas the second was performed during the night after 9 months of treatment.

Results:  After treatment, striking clinical improvement of spastic dysphonia was accompanied by significant diminution in the time spent in periodic breathing and in the number of obstructive and mixed apneas during the night. Controlled studies are needed.

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