Pawlowski Matthias, Johnen Andreas, Duning Thomas
Klinik für Neurologie mit Institut für Translationale Neurologie, Universitätsklinikum Münster, Albert-Schweitzer-Campus 1, Gebäude A1, 48149, Münster, Deutschland.
Nervenarzt. 2020 Oct;91(10):936-945. doi: 10.1007/s00115-020-00967-0.
Due to the demographic change dementia is a common and dramatically increasing reason for medical presentations. In approximately 8% of cases dementia occurs before the age of 65 years. The psychosocial and economic consequences are often severe, particularly in younger patients. Clinicians face major diagnostic challenges. A rapid diagnosis is crucial for patient counselling and management.
This review article presents the special features of dementia in younger people, the most important underlying diseases and a rational clinical diagnostic approach.
Narrative review. The literature search was carried out in PubMed.
The differential diagnostic spectrum of dementia in younger people under the age of 65 years is very broad. The most common causes are Alzheimer's disease with typical or atypical clinical presentations and frontotemporal lobar degeneration. The younger the age of onset, the higher the proportion of treatable and potentially reversible causes of dementia.
The diagnostics of primary neurodegenerative diseases have continuously improved, especially due to the availability of an increasing number of clinical, molecular and imaging biomarkers. Nevertheless, in order to avoid unnecessary and burdensome examinations, the diagnostic work-up of young onset dementia must be hypothesis-driven, i.e. following a precise clinical syndromic classification of the symptoms.
由于人口结构变化，痴呆症是医疗就诊常见且显著增加的原因。约8%的痴呆症病例发生在65岁之前。其社会心理和经济后果往往很严重，尤其是在年轻患者中。临床医生面临重大诊断挑战。快速诊断对患者咨询和管理至关重要。
本文综述介绍了年轻人痴呆症的特点、最重要的潜在疾病以及合理的临床诊断方法。
叙述性综述。在PubMed上进行文献检索。
65岁以下年轻人痴呆症的鉴别诊断范围非常广泛。最常见的病因是具有典型或非典型临床表现的阿尔茨海默病和额颞叶变性。发病年龄越小，可治疗和潜在可逆性痴呆病因的比例越高。
原发性神经退行性疾病的诊断不断改进，特别是由于越来越多的临床、分子和影像生物标志物的可用性。然而，为了避免不必要和繁重的检查，早发性痴呆的诊断工作必须以假设为导向，即遵循对症状进行精确的临床综合征分类。
