Duara R, Lopez-Alberola R F, Barker W W, Loewenstein D A, Zatinsky M, Eisdorfer C E, Weinberg G B
Wien Center for Alzheimer's Disease and Memory Disorders, Mount Sinai Medical Center, Miami Beach, FL 33140.
Neurology. 1993 Jul;43(7):1377-84. doi: 10.1212/wnl.43.7.1377.
Using a liberal criterion, a conservative probability-based criterion, and a criterion for autosomal dominant inheritance, we classified 36%, 13.5%, and 6.4% of 311 patients, respectively, as having familial Alzheimer's disease (FAD). The mean age of onset was over 70 years for all three categories of FAD. FAD and sporadic Alzheimer's disease (SAD) cases did not differ in clinical features, incidence of risk factors for dementia, or MRI or PET features. We observed earlier age of onset of AD to be related positively to longer duration of disease. Except for the autosomal dominant AD group, there was an earlier age of onset in FAD probands. The inheritance of AD from mothers was from 1.7 to 3.6 times more frequent than from fathers. Among SAD patients only, we found a preponderance of women, who were more frequently affected than would be expected from the male/female ratio in the general population of the same average age. Language performance tended to be less affected in FAD than in SAD patients, contrary to some previous reports.
我们分别采用宽松标准、基于概率的保守标准和常染色体显性遗传标准，将311例患者中的36%、13.5%和6.4%归类为患有家族性阿尔茨海默病（FAD）。所有这三类FAD的平均发病年龄均超过70岁。FAD病例与散发性阿尔茨海默病（SAD）病例在临床特征、痴呆风险因素发生率或MRI或PET特征方面并无差异。我们观察到AD发病年龄越早，疾病持续时间越长。除常染色体显性AD组外，FAD先证者的发病年龄更早。AD从母亲遗传的频率比从父亲遗传的频率高1.7至3.6倍。仅在SAD患者中，我们发现女性占多数，她们比同平均年龄的一般人群中的男女比例所预期的更易患病。与之前的一些报道相反，FAD患者的语言能力受影响程度往往低于SAD患者。
