Introduction:
Paroxysmal dysarthria and ataxia (PDA) were first described in the 1940s by Parker and Störring as ”period ataxia,” characterized with brief stereotypes episodes of slurring of speech, dysprosody, and ataxia.2 PDA is well described in multiple sclerosis and reported in other vascular and autoimmune diseases with midbrain lesions near or involving the red nucleus with other neurological manifestations. It has been reported previously in a Turkish patient with typical radiological manifestations of neuro Behçet’s disease (NBD), with many lesions in the periventricular white matter and brainstem. 4
Few reports described solitary midbrain lesion presenting with paroxysms of dysarthria and ataxia, with one reporting none-ataxic spontaneous paroxysmal dysarthria associated with oligoclonal bands in CSF and adequate response to Carbamazepine.3,6 We herein report the first case of paroxysmal ataxia and dysarthria, with isolated midbrain lesion with negative oligoclonal bands, and found to be HLA B51 positive.