Journal List > Dement Neurocogn Disord > v.13(1) > 1120721

Dement Neurocogn Disord. 2014 Mar;13(1):1-6. Korean.
Published online Mar 31, 2014.
© 2014 Korean Dementia Association
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. and Seung Hyun Kim, M.D.
Department of Neurology, College of Medicine, Hanyang University, Seoul, Korea.

Address for correspondence: Seung Hyun Kim, M.D. Department of Neurology, College of Medicine, Hanyang University, 222 Wangsimni-ro, Seongdong-gu, Seoul 133-791, Korea. Tel: +82-2-2290-8371, Fax: +82-2-2296-8370, Email:
Received Mar 17, 2014; Revised Mar 29, 2014; Accepted Mar 29, 2014.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.



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.


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.


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.


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.

Keywords: Amyotrophic lateral sclerosis; Frontotemporal dementia; Neuropsychiatric symptoms; Behavioral and psychological symptoms; Neuropsychological inventory


Fig. 1
Prevalence of behavioral and psychological symptoms in patients with ALS using the caregiver-administered neuropsychiatric inventory (CGA-NPI).
Click for larger image

Fig. 2
Kaplan-Meier survival plots of the ALS patients according to behavioral and psychological symptoms (BPS) during the post-symptom onset period until death.
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Table 1
Clinical characteristics of the patients with or without behavioral and psychological symptoms (BPS) with amyotrophic lateral sclerosis
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Table 2
Subscales prevalence and scores of CGA-NPI in ALS patients with or without behavioral and psychological symptoms (BPS)
Click for larger image


This study was supported by a grant from the Korea Healthcare Technology R&D Project, Ministry for Health, Welfare & Family Affairs, Republic of Korea (A101712).

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