Case report:Juvenile Amyotrophic lateral sclerosis: classical wine glass sign on MRI

Abstract

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig disease, is a chronic degenerative neurologic disease and is characterized by the selective involvement of the motor system. Usually patients present with upper motor neuron (UMN) and lower motor neuron (LMN) compromise. Degeneration of the UMN in the cerebral cortex is one of the main pathologic changes in ALS. These changes usually affects corticospinal tracts (CST) leading to degeneration of the fibres which show characteristic hyper intensities along the tracts leading to the “wine glass sign”. Patients with ALS usually present in the 6th decade of life; presentation in pediatric age in the form of juvenile ALS (JALS) being rare.