Amyotrophic lateral sclerosis (ALS or motor neuron disease) is a progressive neurodegenerative disease associated with loss of upper and lower motor neurons. The diagnosis of ALS is still made at the clinic or bedside by an experienced neurologist. An early and leading theory is that glutamate, the central nervous system’s most abundant excitatory neurotransmitter, causes neuron death when it is elevated, leading to the development of ALS. The clinical hallmark of ALS remains the identification of UMN and LMN signs in multiple body regions. ALS is not curable, but it is treatable. Symptomatic treatments remain the cornerstone of management for patients with ALS. However, Riluzole, a glutamate-release antagonist, is the only disease-modifying drug approved for ALS.