A comprehensive systematic review was performed according to American Academy of Neurology methodology.
CONCLUSIONS: For patients with
episodic ataxia type 2,
4-aminopyridine 15 mg/d probably reduces
ataxia attack frequency over 3 months (1 Class I study). For patients with
ataxia of mixed etiology,
riluzole probably improves
ataxia signs at 8 weeks (1 Class I study). For patients with
Friedreich ataxia or
spinocerebellar ataxia (SCA),
riluzole probably improves
ataxia signs at 12 months (1 Class I study). For patients with SCA type 3,
valproic acid 1,200 mg/d possibly improves
ataxia at 12 weeks. For patients with
spinocerebellar degeneration,
thyrotropin-releasing hormone possibly improves some
ataxia signs over 10 to 14 days (1 Class II study). For patients with SCA type 3 who are ambulatory,
lithium probably does not improve signs of
ataxia over 48 weeks (1 Class I study). For patients with
Friedreich ataxia,
deferiprone possibly worsens
ataxia signs over 6 months (1 Class II study). Data are insufficient to support or refute the use of numerous agents. For nonpharmacologic options, in patients with degenerative
ataxias, 4-week inpatient rehabilitation probably improves
ataxia and function (1 Class I study);
transcranial magnetic stimulation possibly improves cerebellar motor signs at 21 days (1 Class II study). For patients with
multiple sclerosis-associated
ataxia, the addition of pressure
splints possibly has no additional benefit compared with neuromuscular rehabilitation alone (1 Class II study). Data are insufficient to support or refute use of stochastic whole-body vibration
therapy (1 Class III study).