Schrag Anette, Schott Jonathan M
Royal Free and University College Medical School, University College London, Department of Clinical Neurosciences, London, UK.
Lancet Neurol. 2006 Apr;5(4):355-63. doi: 10.1016/S1474-4422(06)70411-2.
In this review we discuss the epidemiological, clinical, and genetic characteristics of early-onset parkinsonism, defined as parkinsonism starting before age 40 (sometimes 50) years. Juvenile parkinsonism is very rare and is the result of various secondary or genetic causes. In patients with onset at or above age 21 years, secondary causes require exclusion but are rare; most cases with a fairly pure parkinsonian syndrome (eg, young-onset Parkinson's disease; YOPD) are due to typical Lewy-body Parkinson's disease or, less commonly, genetic causes. In comparison with patients with late-onset disease, most patients with YOPD progress more slowly in terms of motor features and have a longer disease course with preservation of cognitive function, but typically develop motor fluctuations and dyskinesias earlier. Patients with YOPD generally experience a greater effect in their lives than those with late onset, with poorer social adjustment, higher rates of depression, and lower quality of life. Management of YOPD must therefore aim to maintain occupational, social, and daily functioning, while delaying or ameliorating motor complications of treatment, providing psychological support, and, where possible, preventing psychiatric complications including depression.
在本综述中，我们讨论早发性帕金森综合征的流行病学、临床和遗传特征，早发性帕金森综合征定义为在40岁（有时为50岁）之前起病的帕金森综合征。青少年帕金森综合征非常罕见，是由各种继发性或遗传原因导致的。在21岁及以上起病的患者中，需要排除继发性原因，但继发性原因很少见；大多数具有相当纯粹帕金森综合征的病例（例如，青年帕金森病；YOPD）是由典型的路易体帕金森病引起的，或者较少见的是由遗传原因引起的。与晚发性疾病患者相比，大多数YOPD患者在运动特征方面进展较慢，病程较长且认知功能得以保留，但通常更早出现运动波动和异动症。YOPD患者通常比晚发性患者对生活的影响更大，社会适应能力较差，抑郁发生率较高，生活质量较低。因此，YOPD的管理必须旨在维持职业、社交和日常功能，同时延迟或改善治疗的运动并发症，提供心理支持，并在可能的情况下预防包括抑郁在内的精神并发症。
