근위축측삭경화증 환자에 동반되는 행동심리증상의 임상적 특징과 예후에 대한 영향
Clinical Features and Prognostic Effects of Behavioral and Psychological Symptoms in Patients with Amyotrophic Lateral Sclerosis
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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.
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. 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.
참고문헌 (23)
- Consonni M, "The cognitive and behavioural profile of amyotrophic lateral sclerosis : application of the consensus criteria", Behav Neurol, 27, pp.143~153 (2013)
- Phukan J, "Cognitive impairment in amyotrophic lateral sclerosis", Lancet Neurol, 6, pp.994~1003 (2007)
- 백원기, "한국의 근위축측삭경화증: 임상 특징 및 예후 예측 인자", 대한신경과학회지, 29(1), pp.16~24 (2011), 대한신경과학회
- Oh SI, "Impairment in Korean ALS Patients without Known Genetic Mutations", PLoS One, 9, pp.e87163~ (2014)
- Lillo P, "How common are behavioural changes in amyotrophic lateral sclerosis?", Amyotroph Lateral Scler, 12, pp.45~51 (2011)
- Goldstein LH, "Changes in cognition and behaviour in amyotrophic lateral sclerosis : nature of impairment and implications for assessment", Lancet Neurol, 12, pp.368~380 (2013)
- Robberecht W, "The changing scene of amyotrophic lateral sclerosis", Nat Rev Neurosci, 14, pp.248~264 (2013)
- Brooks BR, "El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis", Amyotroph Lateral Scler Other Motor Neuron Disord, 1, pp.293~299 (2000)
- Jang JH, "Analysis of the C9orf72 hexanucleotide repeat expansion in Korean patients with familial and sporadic amyotrophic lateral sclerosis", Neurobiol Aging, 34, pp.1311e1317~1311e1319 (2013)
- Kwon MJ, "Screening of the SOD1, FUS, TARDBP, ANG, and OPTN mutations in Korean patients with familial and sporadic ALS", Neurobiol Aging, 33, pp.7e1017~7e1023 (2012)
- Kang SJ, "Caregiver-Administered Neuropsychiatric Inventory(CGA-NPI)", J Geriatr Psychiatry Neurol, 17, pp.32~35 (2004)
- Chin J, "Neurobehavioral dysfunction in patients with subcortical vascular mild cognitive impairment and subcortical vascular dementia", Clin Neuropsychol, 26, pp.224~238 (2012)
- 강연욱, "K-MMSE(Korean-Mini Mental State Examination)의 노인 규준 연구", 한국심리학회지:일반, 25(2), pp.1~12 (2006), 한국심리학회
- Morris JC, "The Clinical Dementia Rating (CDR): current version and scoring rules", Neurology, 43, pp.2412~2414 (1993)
- Gordon PH, "Alsfrs-R", Amyotroph Lateral Scler Other Motor Neuron Disord, 5(Suppl 1), pp.90~93 (2004)
- Kimura F, "Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS", Neurology, 66, pp.265~267 (2006)
- Strong MJ, "Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis", Amyotroph Lateral Scler, 10, pp.131~146 (2009)
- Cooper-Knock J, "The widening spectrum of C9ORF72-related disease; genotype", phenotype correlations and potential modifiers of clinical phenotype, 2014, pp.127, 333~345
- Kertesz A, "Psychosis and hallucinations in frontotemporal dementia with the C9ORF72mutation : a detailed clinical cohort", Cogn Behav Neurol, 26, pp.146~154 (2013)
- Raaphorst J, "A systematic review of behavioural changes in motor neuron disease", Amyotroph Lateral Scler, 13, pp.493~501 (2012)
- Kim EJ, "Clinical and genetic analysis of MAPT, GRN, and C9orf72 genes in Korean patients with frontotemporal dementia", Neurobiol Aging, 35, pp.1213e1213~1213e1217 (2014)
- Lillo P, "Amyotrophic lateral sclerosis and frontotemporal dementia: A behavioural and cognitive continuum", Amyotroph Lateral Scler, 13, pp.102~109 (2012)
- Raaphorst J, "The ALS-FTD-Q : a new screening tool for behavioral disturbances in ALS", Neurology, 79, pp.1377~1383 (2012)