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The patients had a mean age at onset of 46.8 years(range,34-62 years).The average interval until death was 8 years.Headache was noted in affected individuals significantly more frequently than in those not affected.The most frequent presentation was memory loss followed by behavior and personality changes and progressive loss of language ability.In the final stages,gait disturbances,seizures,and myoclonus were frequent.Other than the early onset,this clinical phenotype is indistinguishable from sporadic AD except that affected individuals frequently complained of headache preceding and during the disease.Despite the uniform genetic basis for the disease,there was significant variability in the age at onset,suggesting an important role for environmental factors or genetic modifiers in determining the age at onset. |
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