Objective: To highlight the salient clinical and pathologic features of primary progressive apraxia of speech through a clinical case presentation.

Background: Apraxia of speech is a disorder of speech production and articulation characterized by slow speaking rate, abnormal prosody and distorted sound substitutions, additions, repetitions and prolongations, accompanied by groping, and trial and error articulatory movements. Progressive speech apraxia is associated with reduced premotor and supplementary motor cortex volume and is usually due to a tauopathy.

Methods: A 75 year old man presented with a two year history of difficulty speaking and swallowing with weight loss. Examination showed a dysexecutive cognitive profile and emotional lability. He had difficulty with word production. (see video)There was poor diction, groping for words, difficulty articulating words and a spastic tongue. He had wasting in interossei muscles and grade 4/5 power in all muscles. He was hyper-reflexic with up going plantars. Over the next four years his speech and swallow gradually deteriorated and he died.

Results: MRI brain showed peri ventricular ischemic changes. There was moderate neuronal loss and gliosis in globus pallidus.Post mortem brain biopsy showed neurofibrillary tangles, classic & globose with extensive Tau positive neurons- involving substantia nigra, globus pallidus & archicortex likely secondary to Corticobasal degeneration.

Conclusions: Speech Apraxia is a disorder of speech with difficulty in motor planning and /or programming that is distinguishable from aphasia and dysarthria. Progressive apraxia of speech is a tauopathy and hence the clinical presentation should guide the clinician to plan management strategies accordingly.

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