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Original Article
Dementia and Neurocognitive Disorders 2014: 13: 1: 1-6

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근위축측삭경화증 환자에 동반되는 행동심리증상의 임상적 특징과 예후에 대한 영향
오성일, 김희진, 박아람, 오기욱, 곽현승, 김승현
한양대학교 의과대학 신경과학교실
Clinical Features and Prognostic Effects of Behavioral and Psychological Symptoms in Patients with Amyotrophic Lateral Sclerosis
Seong-il Oh, M.D., Hee-Jin Kim, M.D., Aram Park, M.A., Ki-Wook Oh, M.D.,Hyun Seung Gwak, M.D., Seung Hyun Kim, M.D.
Department of Neurology, College of Medicine, Hanyang University, Seoul, Korea
Background: The evaluation of behavioral and psychological symptoms (BPS) in ALS is important because its existence may serve as a prognostic factor and suggest a shared pathology with frontotemporal dementia (FTD) in ALS. In this study, we sought to identify the prevalence of the BPS of ALS patients and evaluate its relationship with the clinical profiles and survival of ALS patients. Methods: One hundred sixty-six patients were enrolled in a cross-sectional cohort analysis from September 2008 to February 2012. All patients had sporadic ALS without a genetic mutation and were collected clinical profiles. The t-test and chi-square test were used to assess differences in the clinical characteristics and caregiver-administered neuropsychiatric inventory (CGA-NPI) scores. The Kaplan-Meier method and Cox proportional hazard model were used for the survival analysis. Results: Forty-two patients had clinically significant BPS (42/166, 25.3%). ALS patients with BPS had worse clinical dementia rating (CDR), ALS Functional Rating Scale-Revised (ALSFRS-R) score, and progression rate of disease than those without BPS. Among CGA-NPI subscales, depression, irritability, apathy, and agitation were higher prevalent than the others. There was a trend for ALS patients with BPS having short survival time than those without BPS in the Kaplan-Meier analysis (p=0.006). However, in the Cox proportional hazard model, BPS in ALS patients were not associated with poor survival. Conclusions: These results support the presence of an overlapping spectrum between ALS and FTD and emphasize the importance of neuropsychiatric evaluations in ALS. Although the association between BPS and prognosis are not explained clearly, these results could be used to stratify ALS patients according to neuropsychiatric symptoms and help investigators to evaluate the BPS in ALS patients.
Key Words: Amyotrophic lateral sclerosis, Frontotemporal dementia, Neuropsychiatric symptoms, Behavioral and psychological symptoms, Neuropsychological inventory