Yoshihara_1998_Ultrastruct.Pathol_22_121

Motor end plates of the intrinsic laryngeal muscles in amyotrophic lateral sclerosis (ALS) were examined light and electron microscopically. Light microscopically, typical neurogenic changes such as small angulated fibers and grouped atrophy were found in the intrinsic laryngeal muscles. Acetylcholinesterase (AchE) activities of the neuromuscular junctions (NMJ) of many fibers in ALS were decreased as compared with those of the controls. Some end-plate areas on each fiber detected by AchE histochemistry were larger than those of the controls. Ultrastructurally, muscle fibers in ALS specimens showed several changes; increased number of lipofuscin granules and/or nuclei, numerous mitochondria, and disappearance of the myofilaments. The NMJ also showed various degrees of structural changes. Some NMJ appeared almost normal. Others showed the absence of nerve terminals and Schwann cells covering the former junctional sites. Their primary synaptic clefts were flattened, and the secondary synaptic clefts were relatively well preserved. On occasion, several small nerve terminals were seen on the severely distorted postsynaptic folds, suggesting regenerative findings. In severely degenerated muscle fibers, the NMJ could not be found.