Primary Progressive Speech Abulia.

BACKGROUND AND OBJECTIVE: Primary progressive aphasia (PPA) is a neurodegenerative disorder characterized by progressive language impairment. The three variants of PPA include the nonfluent/agrammatic, semantic, and logopenic types. The goal of this report is to describe two patients with a loss of speech initiation that was associated with bilateral medial frontal atrophy.

METHODS AND RESULTS: Two patients with progressive speech deficits were evaluated and their examinations revealed a paucity of spontaneous speech; however their naming, repetition, reading, and writing were all normal. The patients had no evidence of agrammatism or apraxia of speech but did have impaired speech fluency. In addition to impaired production of propositional spontaneous speech, these patients had impaired production of automatic speech (e.g., reciting the Lord's Prayer) and singing. Structural brain imaging revealed bilateral medial frontal atrophy in both patients.

CONCLUSION: These patients' language deficits are consistent with a PPA, but they are in the pattern of a dynamic aphasia. Whereas the signs-symptoms of dynamic aphasia have been previously described, to our knowledge these are the first cases associated with predominantly bilateral medial frontal atrophy that impaired both propositional and automatic speech. Thus, this profile may represent a new variant of PPA.