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OBJECTIVE: We defined cutoff values for 3-month changes in forced vital capacity-percent of predicted (FVC[percnt]) and motor unit number estimate (MUNE) that identify amyotrophic lateral sclerosis (ALS) patients with rapidly progressive disease,defined as survival of 30 months or less from symptom onset.BACKGROUND: An independent measure of lower motor neuron function that can be monitored over time is essential to evaluating the effect of treatments and determining prognosis in ALS. Longitudinal changes in FVC[percnt] and MUNE may identify patient groups with more rapid disease progression.DESIGN/METHODS: We report EMG and FVC[percnt] data from a 26- patient cohort. Both 3-month percent change in MUNE and FVC[percnt] were incorporated into a Cox proportional hazards survival model. Individual ROC curves were constructed for 3-month percent change in MUNE and FVC[percnt] as tests for rapid progression. The choice of cut points to identify rapid progressors was made by visual inspection of the ROC curves. We calculated the sensitivity and specificity of a rule combining the two cut-points and compared the Kaplan-Meier survival curves for the rule-positive and rule-negative patients using the log-rank test.RESULTS: Of the 26 patients, 7 had rapid progression. Either a 40[percnt] decrease in statistical MUNE or a 20[percnt] decrease in FVC[percnt] over 3 months identified 6 of 7 rapid progressors (Sensitivity=86[percnt], 95[percnt] confidence interval [CI] 42.1[percnt] - 99.6[percnt]). Of the 19 patients without rapid progression, 18 met neither the FVC or MUNE criterion (Specificity = 94.7[percnt] CI 95[percnt] 74.0[percnt] - 99.9[percnt]). In a proportional hazards model, 3-month change in FVC and MUNE were both significantly predictive of decreased survival.CONCLUSIONS: We suggest the use of a three-month change in MUNE or FVC[percnt] as a secondary enrollment criterion in therapeutic trials or to identify a subgroup of rapid progressors who may respond differently to treatments. Disclosure: Dr. Olney has nothing to disclose. Dr. Kohn has nothing to disclose. Dr. Lomen-Hoerth has nothing to disclose. Dr. Olney has nothing to disclose.

Changes in Motor Unit Number Estimate and Forced Vital Capacity as Predictors of ALS Progression