Early-onset dementia in Chinese: Demographic and etiologic characteristics

Lisa Wing Chi Au; Adrian Wong; Jill Abrigo; Yuet Ping Yuen; Eric Yim Lung Leung; Vincent Chung Tong Mok

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Early-onset dementia in Chinese: Demographic and etiologic characteristics

Author: Lisa Wing Chi Au ¹ ; Adrian Wong ¹ ; Jill Abrigo ² ; Yuet Ping Yuen ^{3
,

4} ; Eric Yim Lung Leung ⁵ ; Vincent Chung Tong Mok ¹
Author Information

1. Department of Medicine and Therapeutics Prince of Wales Hospital, The Chinese University of Hong Kong
2. Department of Imaging and Interventional Radiology, Prince of Wales Hospital, The Chinese University of Hong Kong
3. Department of Pathology, Hong Kong Children&rsquo
4. s Hospital
5. Department of Nuclear Medicine and Positron Emission Tomography, Hong Kong Sanatorium and Hospital, Hong Kong
Publication Type:Journal Article
Keywords: Early-onset dementia; Chinese; genetics
From:Neurology Asia 2019;24(2):139-146
CountryMalaysia
Language:English
Abstract: Objective: Data on early-onset dementia in Chinese is limited. This study aimed to report the diagnostic profiles and characteristics of patients with early-onset dementia in a university-affiliated cognitive disorder clinic in Hong Kong. Methods: We prospectively collected data of consecutive patients who were referred between January 2012 and December 2018. All patients were referred for diagnostic evaluation of cognitive symptoms. Patients with symptom-onset at age 65 or before were recruited. We excluded patients with (1) cognitive deficits referable to an isolated event or toxin and (2) significant mood disorders. Results: Of the 93 patients included, four patients had temporal lobe epilepsy mimicking dementia. Three patients had cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), one patient had Niemann-Pick disease type C and two patients had undetermined aetiology. The remaining 83 patients had primary degenerative dementia. The most frequent diagnosis wasAlzheimer’s disease (AD) (70%), followed by frontotemporal dementia (FTD) (22%) and parkinsonian disorders (8%). The mean age of symptom onset was 57.8 ± 5.8 years.Ten (17%) AD patients had non-amnestic presentation. Fifteen FTD patients consented for mutation screening in the GRN (progranulin), MAPT (microtubule-associated protein tau) and C9orf72 genes, none were positive. Conclusions: Early-onset dementia had a broader differential diagnoses than late-onset dementia, and includes a number of rare hereditary diseases. Patients with suspected early-onset dementia should be thoroughly evaluated to identify any treatable causes.
Full text:7.2019.my0061.pdf