Natural history of primary progressive aphasia.

Abstract

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.

• Authors: Emilie Le Rhun, Florence Richard, Florence Pasquier
• Journal: Neurology (Neurology) Vol. 65 Issue 6 Pg. 887-91 (Sep 27 2005) ISSN: 1526-632X [Electronic] United States
• PMID: 16186529 (Publication Type: Journal Article)

Topics

• Activities of Daily Living
• Age Factors
• Age of Onset
• Aged
• Aged, 80 and over
• Aphasia, Primary Progressive (diagnostic imaging, epidemiology, physiopathology)
• Brain (diagnostic imaging, pathology, physiopathology)
• Cohort Studies
• Comorbidity
• Dementia (diagnostic imaging, epidemiology, physiopathology)
• Disease Progression
• Female
• Humans
• Magnetic Resonance Imaging
• Male
• Middle Aged
• Mortality (trends)
• Neuropsychological Tests
• Prognosis
• Prospective Studies
• Time Factors
• Tomography, Emission-Computed, Single-Photon