Abstract
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.
