This clinical trial investigates the effects of nicotinamide riboside (vitamin B3) on the disease course of patients with ataxia telangiectasia. Patients will be treated during four consecutive months with nicotinamide riboside (25mg/kg/day), followed by a washout period of two months. Main study parameters/endpoints: Ataxia, dysarthria, quality of life, laboratory parameters.
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Ataxia, SARA (Scale of the assesment and rating of ataxia)
Timeframe: change from baseline -1 month - 4 months - 6 months
Ataxia, ICARS (International Cooperative Ataxia Rating Scale)
Timeframe: change from baseline -1 month - 4 months - 6 months
Ataxia, 9-hole pegboard test.
Timeframe: change from baseline -1 month - 4 months - 6 months
Dysarthria, Radboud dysarthria assesment (RDA)
Timeframe: change from baseline -1 month - 4 months - 6 months