Proton Magnetic Resonance Spectroscopy of the Primary Motor Cortex in Patients With Motor Neuron Disease

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Abstract
AMYOTROPHIC lateral sclerosis (ALS) is a motor neuron disease (MND) characterized by a progressive degeneration of upper (UMNs) and lower motor neurons (LMNs) in the motor cortex, brainstem, and spinal cord. Clinical signs include spasticity and amyotrophic paresis as well as bulbar involvement. The disease typically manifests in patients who are 50 to 60 years of age, and the median survival period is approximately 3 years. Until recently, the extent of motor neuron loss in patients with ALS could not be quantified using conventional neuroradiological or electrophysiologic methods. In addition, there is no biological marker reflecting the rate of disease progression in patients with ALS. Even recent trials that tested the efficacy of therapeutic approaches1 used rating scales mainly based on muscle strength or other clinical measures. Therefore, a method for direct quantification of the degenerative process is needed to gain greater insight into motor neuron survival in patients with MND, especially for monitoring therapeutic trials.