Progressive nonfluent aphasia

Lore & Background

Progressive nonfluent aphasia presents with hallmark language deficits, including hesitant, effortful speech, apraxia of speech, stutter, anomic aphasia, phonemic paraphasia, and agrammatism. Most patients exhibit more than one of these problems. As the disease progresses, speech quantity decreases and many patients become mute. Cognitive domains other than language are rarely affected early on, but later stages may involve bowel irritation, problems with writing, reading, and speech comprehension, as well as behavioral features similar to frontotemporal dementia.

Reader's Guide

Progressive nonfluent aphasia is significant as a distinct clinical syndrome within frontotemporal lobar degeneration, offering insight into the neural basis of language production. Its insidious onset and progressive nature differentiate it from acute aphasias, and its initial sparing of memory and other cognitive domains helps distinguish it from Alzheimer-type disorders. Imaging studies, particularly MRI and FDG-PET, have identified atrophy of left perisylvian areas and alterations in the left frontotemporal network for phonological and syntactical processing. These findings allow regional dissociation from other subtypes like frontotemporal dementia and semantic dementia. However, no cure or treatment exists, and management remains supportive. The terminology used by neurologists has caused some confusion, as Mesulam's original description of primary progressive aphasia included patients with progressive nonfluent aphasia, semantic dementia, and logopenic progressive aphasia.

Did You Know?

• PNFA is one of three clinical syndromes associated with frontotemporal lobar degeneration.
• Patients often present with more than one speech production problem, such as apraxia of speech and phonemic paraphasia.
• Imaging studies show atrophy of left perisylvian areas as a classic finding.
• No cure or treatment for this condition has been found; only supportive management is available.