Breitner J C, Murphy E A
Department of Psychiatry, Duke University Medical Center, Durham, North Carolina 27710.
Am J Med Genet. 1992 Nov 15;44(5):628-34. doi: 10.1002/ajmg.1320440520.
The twin method for investigating genetic and environmental causes of disease has been applied mostly in early-onset illnesses. Analysis of late-onset disorders requires reexamination of common assumptions about the relation between genetic causes and the degree of concordance expected. This paper considers Alzheimer disease (AD) as an example of a late-onset disorder with putative genetic factors. For argument it employs the strong hypothesis that AD is an autosomal dominant trait with age-dependent expression, as described by a previously published parametric model. That model encompasses 2 principal variants of disease: a rare form with onset in middle life, and a more common late-onset type which is nonetheless eventually fully penetrant. The present work then specifies the probability that, when a given member of a twin pair (the proband) is affected, an identical or fraternal co-twin also shows the disease. Such probability is expressed as a function of the age at onset of the proband and the current age of the pair. Even under strong working assumptions regarding genetic influence, the expected proportion of identical co-twins actually affected with AD will not exceed 40% until the subjects are about 80 years old. Therefore, except in very old subjects, modest twin concordance is a feeble argument against genetic causes, or in favor of exclusively environmental ones. In this sense the interpretation of results of twin studies in AD and other late-onset disorders differs substantially from studies of diseases with early onset.
用于研究疾病遗传和环境病因的双生子法大多应用于早发性疾病。对晚发性疾病的分析需要重新审视关于遗传病因与预期一致性程度之间关系的常见假设。本文以阿尔茨海默病(AD)为例,探讨一种存在假定遗传因素的晚发性疾病。为便于论证,本文采用了一个强有力的假设,即AD是一种具有年龄依赖性表达的常染色体显性性状,如之前发表的参数模型所述。该模型涵盖了疾病的两种主要变体:一种是中年发病的罕见形式,另一种是更常见的晚发性类型,不过最终具有完全的外显率。本文接着明确了在双胞胎对中的一个特定成员(先证者)患病时,同卵或异卵双胞胎中的另一个也患该病的概率。这种概率表示为先证者发病年龄和双胞胎对当前年龄的函数。即使在关于遗传影响的强有力的工作假设下,直到受试者约80岁时,实际患AD的同卵双胞胎的预期比例才会超过40%。因此,除了非常年老的受试者外,适度的双胞胎一致性对于反对遗传病因或支持仅由环境因素导致的观点来说,说服力较弱。从这个意义上说,AD和其他晚发性疾病的双胞胎研究结果的解释与早发性疾病的研究有很大不同。