Mahajan Supriya, Appleby Brian S
Department of Neurology, University Hospitals Cleveland Medical Center, 3619 Park East Drive, Suite 206, Beachwood, OH, 44122, USA.
National Prion Disease Pathology Surveillance Center and the Departments of Neurology, Psychiatry, and Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.
Curr Treat Options Neurol. 2017 Sep 30;19(11):40. doi: 10.1007/s11940-017-0474-1.
Purpose of review The sudden emergence of a change in cognitive abilities or behavior is an important symptom that warrants medical evaluation and may represent the early stages of a rapidly progressive dementia (RPD). To correctly ascertain the cause of RPD in a given patient, the clinician must be methodical and knowledgeable about the range of potential causes and must move forward with supportive treatment, and in some cases empiric treatment, based on clinical features alone. Recent findings Significant advances in prion disease biomarkers, the molecular features of rapidly progressive Alzheimer's disease, and new detection of autoimmune limbic encephalitis disease entities have caused a shift in the diagnostic and treatment framework of RPD. Additionally, in the past decade, emerging retrospective data have led to suggested treatments in autoimmune encephalitis that, if instituted early, can protect patients against residual deficits and disease relapse. Summary Here, we provide an integrative clinical and diagnostic treatment approach that is applicable to the various forms of RPD. We have highlighted the clinical features of selected types of RPD that have experienced advances in the last 10-15 years.
综述目的 认知能力或行为突然出现变化是一个重要症状，需要进行医学评估，可能代表快速进展性痴呆（RPD）的早期阶段。为了正确确定特定患者RPD的病因，临床医生必须有条不紊且了解潜在病因的范围，并必须基于临床特征单独进行支持性治疗，在某些情况下进行经验性治疗。 最新发现 朊病毒病生物标志物、快速进展性阿尔茨海默病的分子特征以及自身免疫性边缘叶脑炎疾病实体的新检测方面的重大进展，导致了RPD诊断和治疗框架的转变。此外，在过去十年中，新出现的回顾性数据为自身免疫性脑炎提出了建议的治疗方法，如果早期实施，可以保护患者免受残留缺陷和疾病复发的影响。 总结 在此，我们提供了一种适用于各种形式RPD的综合临床和诊断治疗方法。我们强调了在过去10至15年中取得进展的特定类型RPD的临床特征。
