PRIMARY PROGRESSIVE APHASIA: QUANTITATIVE ANALYSIS

Abstract

Primary progressive aphasia (PPA) is a focal dementia defined as a progressive dissolution in speech and language functions, including anomia (inability to retrieve nouns upon demand), agrammatism (inappropriate word order or use of prepositions), or loss of semantic knowledge about words and objects.1–5 Neuroimaging studies in PPA show metabolic dysfunction and atrophy in language areas, including the left inferior frontal, perisylvian, and temporal cortices.2,6 We describe a poet with recently diagnosed PPA, in whom naming, grammatism, rhyming ability, and semantic knowledge were progressively disturbed. Quantitative analysis of his poetry from four different time points over a span of 10 years showed parallel deterioration of all these faculties, commencing 3 years before first notice of disease onset. ### Case report. A 69-year-old community rabbi and amateur poet was self-referred to our neurology department for evaluation of complaints of deterioration in linguistic capacity, manifesting primarily as difficulty writing poems 9 months previously. Initial neuropsychological examination (table e-1 on the Neurology ® Web site at www.neurology.org) showed moderate global language disturbance, with difficulties in language comprehension, speech, naming, repetition, word generation, reading, writing, and abstraction. Executive functions were mildly impaired, as were problem-solving and semantic knowledge. Short-term memory skills and parietal functions were intact. …