Amyotrophic Lateral Sclerosis in Korea: Clinical Characteristics and Prognostic Factors.
- Author:
Wonki BAEK
1
;
Aram PARK
;
Hyun Young KIM
;
Seung Hyun KIM
Author Information
1. Department of Neurology, Hanyang University College of Medicine, Seoul, Korea. address: kimsh1@hanyang.ac.kr
- Publication Type:Original Article
- Keywords:
Amyotrophic lateral sclerosis;
Clinical characteristics;
Prognostic factors
- MeSH:
Amyotrophic Lateral Sclerosis;
Disease Progression;
Humans;
Korea;
Motor Neuron Disease;
Neuropsychological Tests;
Prognosis;
Tracheostomy
- From:Journal of the Korean Neurological Association
2011;29(1):16-24
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
BACKGROUND: No prospective analyses of clinical characteristics and prognostic factors on amyotrophic lateral sclerosis (ALS) have been performed in Korea. METHODS: The prognosis of 227 sporadic ALS patient with El-Escorial-compatible criteria were evaluated using the serial Korean Amyotrophic Lateral Sclerosis Functional Rating Scale-revised (K-ALSFRS-R), and the progression rate of K-ALSFRS-R (DeltaFS=48-ALSFRS-R at the time of diagnosis). Their clinical characteristics and survival times were also analyzed. The endpoint was defined as either time to death or the tracheostomy state. The effects of individual prognostic factors on disease progression were assessed with the Kaplan-Meier life-table method and multivariate Cox proportional regression analysis. The declining ALSFRS-R curve, epidemiological data, and neuropsychological tests were evaluated. RESULTS: Among 331 motor neuron disease (MND) patients, 227 were finally diagnosed as sporadic ALS. The mean age at onset in 227 sporadic ALS patients was 50.7 years (range 19-76 years). Analysis of sequential ALSFRS-R data revealed that a mean DeltaFS of 0.73/month and a high DeltaFS score were related with early death or a tracheostomy state. Neuropsychological testing revealed nonmotor symptoms, and especially cognitive impairment, had developed in half of our patients. CONCLUSIONS: Our data suggest that DeltaFS is valuable parameter for evaluating prognosis and clinical course of ALS patients, and clinical characteristics obtained from this study would be informative data for understanding epidemiology of ALS in Korea.
