Lower Motor Neuron Hyperexcitability in Amyotrophic Lateral sclerosis: Analysis Using Motor Evoked Potentials

근위축성 측삭 경화증의 하 운동 신경원 과흥분성: 운동유발전위를 이용한 분석

Background & Objectives: Hyperexcitablity of motor system is a well-established characteristic pathophysiologic finding of amyotrophic lateral sclerosis (ALS). Whereas little is known about the source of excitability according to the progression of the disease. We evaluated the excitability and its source in advanced ALS patients using transcranial magnetic stimulation (TMS). Meterial & Methods: Motor evoked potentials (MEP) by TMS were recorded for abductor pollicis brevis muscles in 20 patients, 11 men and 9 women, with ALS. Mean age was $54.2{\pm}12.1years$, and mean disease duration was $13.9{\pm}13.4years$. Serial magnetic stimulations were applied to get the parameters; excitability threshold (ET), amplitude and latency of MEP. We also had a facilitated MEP (fMEP). Results: The parameters were analyzed according to the clinical settings. ET was higher in ALS(mean $63.5{\pm}18.1$) than normal control (mean $46.0{\pm}8.4$, p<0.01). Amplitudes of MEP were reduced in ALS ($2.6{\pm}3.6mV$; control $6.5{\pm}3.1mV$, p<0.01). Duration of the disease and ET showed significant inverse correlation (Spearson correlation coefficient = -0.57, p<0.01). Duration of the disease and fMEP/MEP ratio showed less but also significant inverse correlation (Spearson correlation coefficient, r = -0.52, p < 0.05). Conclusions: Lower ET in advanced ALS patients, in spite of decreased fMEP/MEP ratio, may indicate the hyperexcitability of lower motor neurons in these patients. This study suggests that lower motor neurons is hyperexcitable due to upper motor neuron dysfunction at advanced stage.