CAN WE TREAT RESPIRATORY-FAILURE IN FRIEDREICH ATAXIA

Background: Neurochemical disorders associated with spinocerebellar at axias 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 desaturati on as well as obstructive or mixed apneas associated with severe desat uration during the night. He was given oxitriptan (5-hydroxy-L-tryptop han) (1500 mg/d), thiamine hydrochloride (100 mg/d), and amantadine hy drochloride (100 mg/d). The first sleep study was conducted during the night before treatment, whereas the second was performed during the n ight after 9 months of treatment. Results: After treatment, striking c linical improvement of spastic dysphonia was accompanied by significan t diminution in the time spent in periodic breathing and in the number of obstructive and mixed apneas during the night. Controlled studies are needed.