Seven patients with hyperkinetic movement disorder at onset were treated with oral Baclofen (selective agonist of GABA B receptors), with a modest clinical response as already reported [8], whereas clonazepam (GABAergic agent) used in six patients with hyperkinetic-onset and in two patients with ataxia appeared more effective when patients did not display dystonia. Here, GABBR1 is linked to cerebellar ataxia.