Korean version of Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised: A Pilot Study on the Reliability and Validity

BACKGROUND: The Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) is a quantitative measure of ALS-related physical deficit with established reliability and validity in prospective clinical research. This study aimed to test the reliability and validity of the Korean version of ALSFRS-R (K-ALSFRS-R). METHODS: The subjects of the study were 51 patients with ALS diagnosed on the basis of El Escorial criteria. The patients were evaluated by one rater using K-ALSFRS-R, Norris scale, and Appel ALS rating scale on the same day. Subsequently, K-ALSFRS-R was measured by two other raters using a videotape design. Test-retest data were obtained within the time interval of 7 days. RESULTS: Internal consistency and test-retest reliability were high. Inter-rater comparisons showed significant reliability with Kappa or Kendall's tau-b value. Moreover, K-ALSFRS-R scores correlated significantly with clinical status as measured by Norris and Appel ALS rating scale. CONCLUSIONS: Our results showed that K-ALSFRS-R would be a reliable and useful instrument for the evaluation of functional status in patients with ALS.

Preliminary Study on Clinical Characteristics and Caregivers' Burden of Korean Patients with Amyotrophic Lateral Sclerosis; Survey Based on Database of Korea ALS Association

BACKGROUND: Accurate information on natural history and prognosis of Amyotrophic Lateral Sclerosis (ALS) is useful for patients, their families, and clinicians. This study was designed to determine natural history and prognosis in Korean ALS patients. METHODS: We surveyed 311 ALS patients who registered in Korean ALS association, on clinical and epidemiological characteristics association with demographic factors, initial symptoms, time delay of diagnosis, caregivers' burden. RESULTS: Ratio of gender was 1.6:1.0 (M:F) and the mean age onset was 50.2 years. Limb onset type was two times more frequent than bulbar onset type. Mean time delay from onset to diagnosis was 14.7 months, and neurologist did most of the diagnosis (90.4%). Multiple linear regression analyses of patient and caregiver variables on caregiver burden and care stress were significantly and independently associated with declined physical function, pain, ventilator application and short disease duration of patients. CONCLUSIONS: Present preliminary results on clinical characteristics and caregivers' burden of Korean ALS patients would be used as the basic data for the large scale of epidemiologic study on Korean ALS.