AMYOTROPHIC LATERAL SCLEROSIS - A PROGRESSIVE NEURODEGENERATIVE DISEASE: INSIGHTS FOR DISEASE MANAGEMENT

S. Maya*

ABSTRACT

Amyotrophic Lateral Sclerosis (ALS), is a progressive, multisystemic and multi factorial neurodegenerative disease that affecting to the motor neurons. The disease affects both the upper and lower motor neurons and it affects the motor functions. As the disease progresses, the patient will be paralyzed and eventually dies due to the respiratory arrest. The exact cause and mechanism for its progression is unknown, hence it is difficult to develop an effective treatment for ALS. Even though the neurologists can able to identify the ALS and its variants, a few percentage of patients were misdiagnosed and delays the actual identification of disease. The drug approved by the Food and drug administration (FDA) for ALS are Riluzole and Edaravone. Riluzole, an inhibitor of glutamate release and noncompetitively inhibits postsynaptic N-methyl-D-aspartate receptor (NMDA) and α-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptors and Edaravone is thought to reduce oxidative stress in cells by lowering of free radicals. Also, it is hard to mention that both has a modest effect in prolonging life of the patient. Proper diagnosis, effective communication of the diagnosis, the involvement of patient and their caretakers, and a positive care plan are essential for a better clinical management. As the disease is a multifactorial disease, multiple approaches need to support the patient and their caretakers. In this review, we discussed various diagnostic methods, symptomatic treatments, nutritional and respiratory supports, psychological supports for the patient and their caretakers and the methods to preserve the patient’s mobility and communication. Which helps the patient to cope with the impaired function and to improve the quality of life.

Keywords: Amyotrophic Lateral Sclerosis; management; diagnosis; treatments; neurodegenerative disease.