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.