Elevated Creatine Kinase is Associated with a Better Prognosis in Patients with Amyotrophic Lateral Sclerosis (P6.099)

OBJECTIVE: To determine: 1. Whether amyotrophic lateral sclerosis (ALS) patients with raised creatine kinase (CK) differ from ALS patients with normal CK? 2. The implications of raised CK on disease outcome or prognosis? 3. Whether the magnitude of muscle enzyme release be used to predict the magnitude of muscle functional impairment? BACKGROUND: CK is commonly observed to be mild to moderately elevated in the patients with ALS. The precise cause of elevated CK in only a proportion of ALS cases and its behaviour with disease progression is unknown. DESIGN/METHODS: This is a prospective observational cohort study, using the clinical data base of Olesoxime (TRO19622) investigational medicinal product trial. RESULTS: Baseline CK was raised in 52[percnt] of the participants: mean CK 257 ± SD of 239 IU/L. Mean CK in male participants was significantly higher than in females (p<0.001). Mean CK was significantly higher amongst participants with limb onset ALS compared to participants with bulbar onset ALS (p<0.001). There was no significant difference in CK levels between upper limb and lower limb onset disease (p=0.746). There was a very weak positive relationship between CK and muscle scores which was more pronounced in the later stages of the disease. A higher CK level was associated with significantly better survival, even when adjusted for prognostic co-variants (p=0.002). CONCLUSIONS: CK level is a prognostic factor for survival in ALS, independent of the fact that limb onset disease is associated with a higher CK and better prognosis. This finding suggests that CK may be involved as a defence mechanism in the face of the metabolic stress of ALS. CK levels cannot be used to predict the disease severity or manual muscle scores at any time point. Study Supported by: The Motor Neurone Disease Association (UK) and TROPHOS. Disclosure: Dr. Rafiq has nothing to disclose. Dr. Lee has nothing to disclose. Dr. McDermott has nothing to disclose. Dr. Shaw has received personal compensation for activities with Sanofi-Aventis Pharmaceuticals Inc.