Dement Neurocogn Disord.  2014 Mar;13(1):1-6. 10.12779/dnd.2014.13.1.1.

Clinical Features and Prognostic Effects of Behavioral and Psychological Symptoms in Patients with Amyotrophic Lateral Sclerosis

Affiliations
  • 1Department of Neurology, College of Medicine, Hanyang University, Seoul, Korea. kimsh1@hanyang.ac.kr

Abstract

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.
CONCLUSION
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.

Keyword

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

MeSH Terms

Amyotrophic Lateral Sclerosis*
Apathy
Cohort Studies
Dementia
Depression
Dihydroergotamine
Frontotemporal Dementia
Humans
Kaplan-Meier Estimate
Pathology
Prevalence
Prognosis
Proportional Hazards Models
Research Personnel
Dihydroergotamine
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