The Etiology of Rapidly Progressive Dementia: A 3-Year Retrospective Study in a Tertiary Hospital in China

Article type: Research Article

Authors: Shi, Qin^{a; b; 1} | Liu, Wei-Shi^{a; 1} | Liu, Fang^{c; 1} | Zeng, Yi-Xuan^d | Chen, Shu-Fen^a | Chen, Ke-Liang^a | Yu, Jin-Tai^a | Huang, Yu-Yuan^{a; *}

Affiliations: [a] Department of Neurology and National Center for Neurological Disorders, Huashan Hospital, State Key Laboratory of Medical Neurobiology and MOE Frontiers Center for Brain Science, Shanghai Medical College, Fudan University, Shanghai, China | [b] Jiangyin Hospital Affiliated to Nanjing University of Chinese Medicine, Jiangyin, Jiangsu, China | [c] Shandong Xiehe University, Jinan, Shandong, China | [d] Department of Neurology, The First Affiliated Hospital of Shenzhen University, Shenzhen Second People’s Hospital, Shenzhen, China

Correspondence: [*] Correspondence to: Dr. Yu-Yuan Huang, MD, PhD, Department of Neurology and National Center for Neurological Disorders, Huashan Hospital, Shanghai Medical College, Fudan University, 12th Wulumuqi Zhong Road, Shanghai 200040, China. Tel.: +86 19921310136; E-mail: huang_yuyuan@126.com.

Note: [1] These authors contributed equally to this work.

Abstract: Background:Rapidly progressive dementia (RPD), characterized by a rapid cognitive decline leading to dementia, comprises a diverse range of disorders. Despite advancements in diagnosis and treatment, research on RPD primarily focuses on Western populations. Objective:This study aims to explore the etiology and demographics of RPD in Chinese patients. Methods:We retrospectively analyzed 323 RPD inpatients at Huashan Hospital from May 2019 to March 2023. Data on sociodemographic factors, epidemiology, clinical presentation, and etiology were collected and analyzed. Results:The median onset age of RPD patients was 60.7 years. Two-thirds received a diagnosis within 6 months of symptom onset. Memory impairment was the most common initial symptom, followed by behavioral changes. Neurodegenerative diseases accounted for 47.4% of cases, with central nervous system inflammatory diseases at 30.96%. Autoimmune encephalitis was the leading cause (16.7%), followed by Alzheimer’s disease (16.1%), neurosyphilis (11.8%), and Creutzfeldt-Jakob disease (9.0%). Alzheimer’s disease, Creutzfeldt-Jakob disease, and frontotemporal dementia were the primary neurodegenerative causes, while autoimmune encephalitis, neurosyphilis, and vascular cognitive impairment were the main non-neurodegenerative causes. Conclusions:The etiology of RPD in Chinese patients is complex, with neurodegenerative and non-neurodegenerative diseases equally prevalent. Recognizing treatable conditions like autoimmune encephalitis and neurosyphilis requires careful consideration and differentiation.

Keywords: Alzheimer’s disease, cognitive impairment, neurodegenerative causes, non-neurodegenerative causes, rapidly progressive dementia, spectrum

DOI: 10.3233/JAD-240079

Journal: Journal of Alzheimer's Disease, vol. 100, no. 1, pp. 77-85, 2024