Dementia Research Centre, Department of Neurodegeneration, UCL Institute of Neurology, Queen Square, London, UK.
Lancet Neurol. 2010 Aug;9(8):793-806. doi: 10.1016/S1474-4422(10)70159-9.
A diagnosis of dementia is devastating at any age but diagnosis in younger patients presents a particular challenge. The differential diagnosis is broad as late presentation of metabolic disease is common and the burden of inherited dementia is higher in these patients than in patients with late-onset dementia. The presentation of the common degenerative diseases of late life, such as Alzheimer's disease, can be different when presenting in the fifth or sixth decade. Moreover, many of the young-onset dementias are treatable. The identification of causative genes for many of the inherited degenerative dementias has led to an understanding of the molecular pathology, which is also applicable to later-onset sporadic disease. This understanding offers the potential for future treatments to be tailored to a specific diagnosis of both young-onset and late-onset dementia.
痴呆症的诊断在任何年龄都是毁灭性的，但在年轻患者中诊断则提出了一个特别的挑战。由于代谢疾病的晚期表现很常见，而且遗传性痴呆症在这些患者中的负担比在晚年发病的患者中更高，因此鉴别诊断范围很广。当常见的退行性疾病（如阿尔茨海默病）在五、六十岁时出现时，其表现可能会有所不同。此外，许多早发性痴呆症是可以治疗的。许多遗传性退行性痴呆症的致病基因的确定导致了对分子病理学的理解，这也适用于后来的散发性疾病。这种理解为年轻和晚年发病的痴呆症的特定诊断提供了未来治疗的可能性。
