Department of Neurology, Drexel University College of Medicine, Philadelphia, PA 19102, USA.
Am J Alzheimers Dis Other Demen. 2012 May;27(3):154-61. doi: 10.1177/1533317512444000.
Early-onset dementia, presenting before age 65 years, is increasingly recognized. It is often difficult to diagnose, since non-Alzheimer's etiologies and unusual dementias are common. These conditions are more commonly genetic, and important potentially inherited causes of early-onset dementia include early-onset Alzheimer's disease, frontotemporal dementia, Kufs' disease, and Niemann-Pick disease type C. For each of these diseases, this review provides information on common clinical presentations, etiology, pathophysiology, and current and experimental treatments. A discussion of the diagnosis and workup for early-onset dementia is included with an emphasis on conditions that may have other involved family members.
早发性痴呆，即发病年龄在 65 岁之前，越来越受到关注。由于非阿尔茨海默病病因和不常见的痴呆较为常见，因此通常难以诊断。这些疾病更常见于遗传性，早发性痴呆的重要潜在遗传性病因包括早发性阿尔茨海默病、额颞叶痴呆、库夫斯病和尼曼-皮克病 C 型。对于每种疾病，本综述均提供了常见临床表现、病因、病理生理学以及现有和实验性治疗的信息。讨论了早发性痴呆的诊断和检查，重点介绍了可能涉及其他家族成员的疾病。
