Retinal Disorders in Humans and Experimental ALS Models

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease that severely impairs the patient’s mobility, as it mainly affects the upper and lower motor neurons in the spinal cord. In addition, alterations have also been demonstrated in different parts of the central nervous system (CNS), such as the brain and brainstem. The retina is a projection to the brain and is considered as a “window” to the CNS. Moreover, it is possible to use the retina as a biomarker in several neurodegenerative diseases, even in the absence of major visual impairment. Classically, it was thought that the eyes were not affected in ALS, with respect to extraocular muscles, whereas the remainder of the muscles of the body were distressed. Nevertheless, retinal changes have recently been found in this pathology and could help in diagnosis, follow-up, and even monitoring therapies in this disease.