Acquired paroxysmal dysarthria-ataxia syndrome: A systematic review of clinical and MRI findings.

Abstract

Paroxysmal dysarthria-ataxia syndrome (PDAS) is a rare neurological syndrome initially described in Multiple Sclerosis (MS) and subsequently reported in other conditions. This systematic review focuses on acquired forms of PDAS associated with structural or immune-mediated aetiologies. After screening 3802 articles, 25 studies (31 patients) were included. Twenty-two patients (71%) were male, and the mean age of PDAS onset was 52 years. The most frequent diagnosis was MS, observed in 12 patients (39%), followed by anti-contactin associated protein receptor 2 encephalitis in 6 cases (19%). Lesions were most commonly located in the midbrain, reported in 20 patients (65%). Both the duration and frequency of PDA episodes varied greatly. Outcomes were favorable, with complete resolution of symptoms after pharmacological treatment in 29 patients (93%). Treatment mainly consisted of antiepileptic drugs (AEDs), most commonly carbamazepine, used in 14 patients (45%). These findings show that PDAS is not exclusively associated with MS and that, when treated, its prognosis is generally very good.