Table 1.

Summary of main symptomatic treatment for patients with autosomal recessive hereditary ataxias.

Autosomal recessive ataxia	Symptomatic treatment	Level of evidence/ Grade of recommendation
Friedreich ataxia	Idebenone 5–20 mg/kg day or CoQ10 30 mg/kg day	Class I/A
Ataxia with vitamin E deficiency	Vitamin E supplementation	Class III/B
Abetalipropoteinemia	Vitamin E supplementation 150 mg/kg; Vitamin A; Medium-chain triglyceride supplement and/or low fat diet	Class IV/Good practice point
Refsum’s disease	Diet modification to decrease intake of phytanic acid; Plasmapheresis	Class IV/Good practice point
Niemann-Pick type C	Miglustat	Class III/B
Cerebrotendinous xanthomatosis	Chenodeoxycholic acid 750 mg/day, HMG-CoaA reductors	Class III/C
Ataxia associated with CoQ10 deficiency	CoQ10 supplementation 30 mg/kg/day	Class IV/Good practice point
Glutl deficiency syndrome	Ketogenic diet	Class III /C