Pearls & Oy-sters: Paroxysmal dysarthria-ataxia syndrome: Acoustic analysis in a case of antiphospholipid syndrome

Pearls: Paroxysmal dysarthria-ataxia syndrome (PDA), first described by Parker in 1946, is characterized by paroxysmal and stereotyped repeated daily episodes of sudden ataxic symptoms associated with dysarthric speech lasting from few seconds to minutes.1 During the episodes, patients present with slow speech, irregular articulatory breakdown, dysprosodia, hypernasality, variable pitch and loudness, and prolonged intervals, consistent with perceptual characteristics of ataxic dysarthria.2,3 PDA is a rare neurologic manifestation of either genetic or acquired conditions.2 The most frequent genetic diseases occurring with PDA are episodic ataxias, a group of dominantly inherited disorders characterized by transient and recurrent episodes of truncal instability and limbs incoordination triggered by exertion or emotional stress.4 Among acquired conditions, PDA has been reported mainly in multiple sclerosis (MS), in other immunomediated diseases, or in ischemic stroke.5,–,7 The common finding among these diseases is the involvement of cerebellar pathways, specifically the crossed fibers of cerebello-thalamocortical pathway in the lower midbrain. Indeed, most of the reported cases of PDA suggest that the responsible lesion is located in the midbrain, near or in the red nucleus,8 where a lesion frequently reveals with dysarthria.9,10 Oy-sters: Until now, the pathophysiologic basis of PDA remains unknown, as well as the characterization of dysarthria during PDA. We present a case of PDA in a patient with antiphospholipid syndrome (APS) evaluated with an acoustic and perceptual analysis of speech to determine the specific pattern of paroxysmal dysarthria.