Histomorphology of Amyotrophic Lateral Sclerosis: An Autopsy Case Report

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting predominantly the motor neurons of the anterior horns of the spinal cord. The condition, in most cases, starts with lower limb muscle weakness that steadily progresses and affects all muscle groups of the body. This in time leads to severe muscle atrophy and muscle paralysis, with respiratory muscle affection leading to respiratory failure. Several clinical investigations such as a physical examination, imaging modalities of the spinal cord, electroencephalography, electromyography, and genetic tests in the case of suspicion of a hereditary form are often informative enough to place the diagnosis. Histological changes are often nonspecific with neuronal degeneration and demyelination in the anterior horns of the spinal cord being the most severe changes. Here, we present the classical constellation of histopathological changes associated with ALS along with demyelination, neuronal degeneration, Lewy-like intra and extracellular bodies, and intracellular Bunina bodies.