Natural history of primary progressive aphasia.
OBJECTIVE
To characterize the natural history of primary progressive aphasia (PPA).


METHODS
Forty-nine patients (28 women) with newly diagnosed with PPA presenting to a memory disorders clinic between 1992 and 2001 were prospectively evaluated.


RESULTS
Median age at onset was 62 years (range 49 to 73 years) and at first visit was 66 years (52 to 80 years). The median duration of follow-up was 4 years (1 to 11 years). Impairments in activities of daily living developed a median of 6 to 7 (2 to 12) years post onset. Seventy-five percent of patients eventually met clinical diagnostic criteria for frontotemporal dementia (FTD), 14% met diagnostic criteria for dementia with Lewy bodies, and 8% developed signs of corticobasal degeneration; 60% of the patients died after a median course of 7 years (3 to 17 years) at a median age of 71 years (56 to 81 years). Patients showing high Mini-Mental State Examination scores, moderate aphasia, and fluent language at first visit subsequently retained greater autonomy in daily life.


CONCLUSIONS
Although activities of daily living are well maintained during the first years of the disease, patients with primary progressive aphasia (PPA) may lose autonomy 6 to 7 years after onset. The majority of patients with PPA in the current study developed frontotemporal dementia.
Le Rhun
Emilie
E
Department of Neurology, Memory Clinic, University Hospital of Lille, France.
Richard
Florence
F
Pasquier
Florence
F
D000203:Activities of Daily Living; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D018888:Aphasia, Primary Progressive / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000503:physiopathology*; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D015331:Cohort Studies; D015897:Comorbidity; D003704:Dementia / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000503:physiopathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009026:Mortality / Q000639:trends; D009483:Neuropsychological Tests; D011379:Prognosis; D011446:Prospective Studies; D013997:Time Factors; D015899:Tomography, Emission-Computed, Single-Photon