cqvip:Background: Knowledge of the evolution of cognitive deficits in Alzheimer dise ase is important for our understanding of disease progression. Previous reports, however, have either lacked detail or have not covered the presymptomatic stage s. Objective: To delineate the onset and progression of clinical and neuropsycho logical abnormalities in familial Alzheimer disease. Methods: Nineteen subjects with familial Alzheimer disease underwent serial clinical and neuropsychological assessments. Eight of these had undergone presymptomatic assessments. The follo w up period was 1 to 10 years (mean, 5 years). The relative timing of the occur rence of 3 markers of disease onset and progression (onset of symptoms, Mini Me ntal State Examination score ≤24, and impaired score on a range of neuropsychol ogical tests) were compared using the binomial exact test. Results: Neurological abnormalities were not prominent, although myoclonus appeared early in some. Mi ni Mental State Examination score was not sensitive to early disease. Memory an d general intelligence deficits appeared at an earlier stage, in some patients w hen pre symptomatic. Perceptual, naming, and especially spelling skills were pr eserved to a late stage. Conclusion: Familial Alzheimer disease may have a long prodromal phase of several years with subtle deficits initially of general intel ligence and memory, while spelling, naming, and perception are relatively preser ved until a late stage.
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