Quantitative neuroimaging of cervical spinal cord in Amyotrophic Lateral Sclerosis

Abstract

Amyotrophic Lateral Sclerosis (ALS) is the most prevalent adult motor neuron disease and involves both upper and lower motor neurons. It is characterized by progressive weakness, generalized muscular atrophy, diffuse cramps, fasciculations, and sometimes spasticity. Pathophysiology of ALS remains largely elusive so therapeutic efforts are at most palliative. Early diagnosis of ALS is hampered by a large number of diseases with similar symptoms. Therefore, biological markers would be extremely helpful to diagnose the disease and monitoring its course. Thus, we propose a transversal study in order to evaluate the usefulness of quantitative Magnetic Resonance Imaging (MRI) of the cervical spinal cord in ALS. First, we are selecting 30 subjects with confirmed ALS (according to the El Escorial criteria), and also a group of healthy control subjects. Each subject will undergo a neurological evaluation and MRI scanning on the same day. The ALS functional rating scale will be used to quantify motor dysfunction in these patients. We will employ a previously validated software (Spineseg) to determine cord cross-sectional area and eccentricity at the C2-C3 level. These values will be compared between patients and controls. In addition, we will perform correlation analyses to determine if these measures are associated with clinical parameters, expressed by the ALSFRS score.(AU)