Dissolution of spoken language in primary progressive aphasia

Abstract

The dissolution of speech and language was investigated over a 4 year period in a male with an 8 year history of isolated speech and language deterioration exhibiting a non-fluent profile of primary progressive aphasia (PPA). The course of his communication impairment began with apraxia of speech and difficulty accessing word form (e.g. as evidenced by frequent ‘tip-of-the-tongue’ errors that typically contained correct production of word onsets or first syllables but not whole wores). Ultimately all aspects of oral language use deteriorated until the individual became non-vocal. His ability to communicate through non-verbal modalities remained intact. Results of repeated administrations of standardized tests and analyses of connected speech obtained over 2 years were compared. The ability of various assessment probes to substantiate clinical impressions of noticeable declines in expressive language use was examined. It was concluded that the most sensitive indices of spoken language dissolution are likely to be measures derived from connected speech. Unlike level-specific subtests (i.e. subtests that are designed to probe relatively isolated stages of processing such as word retrieval apart from syntactic formulation), the requisite simultaneity of processing across levels during connected speech taxes the aphasic's entire language processing system. Three principles guided the approach to management: (i) anticipatory implementation of treatment goals; (ii) therapy is dyad oriented; and (iii) therapy is directed at the level of the disability, defined as the limitations to perform specific functions within a natural context The cornerstone of this approach, labelled Proactive management, lies in the concept that the goals are formulated and implemented in anticipation of future declines so that the patient with PPA is prepared to maximize communication effectiveness at every stage despite the relentless progression of the disease.