Neuronophagia in the motor cortex in amyotrophic lateral sclerosis

This study was designed to identify which phagocytic cells in the cerebral cortex of amyotrophic lateral sclerosis (ALS) patients are involved in the process of neuronophagia. For this purpose a number of single and double immunocytochemical stains were carried out on five ALS cases which were selected on the basis of the presence of degenerative and phagocytic phenomena in the cerebral cortex. The cortical degenerative process is mainly present in the third and fifth layers and is not restricted to the fifth layer which contains the cell bodies of the Betz cells. The present study indicates that a number of cells are involved in the process of phagocytosis in ALS. Resident macrophages (from microglial or perivascular origin) and astrocytes seem to play an immunologically-mediated role in the disappearance of neurons. Some of the cells involved in the degenerative process, i.e. rounded macrophages and microglia, expressed major histocompatibility class II antigen. The phagocytic cells in neuronophagia were phenotypically identical to perivascular macrophages and not to microglia. Therefore, the process of phagocytosis of neurons appears to be primarily the task of the perivascularly located macrophage.