Ultrastructural study of chromatolytic neurons in an adult-onset sporadic case of amyotrophic lateral sclerosis

摘要: Ultrastructural features of chromatolytic neurons observed in a sporadic case with amyotrophic lateral sclerosis (ALS) are reported. A 70-year-old woman died weakness and atrophy the four limbs, bulbar facial muscles, hyperreflexia, 3 1/2 years' duration. Neuronal loss was marked anterior horn spinal cord, degeneration pyramidal tracts. Most remaining showed chromatolysis. Some contained faintly eosinophilic inclusions halo. Few spheroids were observed. Hypoglossal nuclei, nucleus ambiguus, motor nuclei N. VII N.V well populated, but several neurons. Ultrastructurally, aggregates fibrils thicker than 10-nm neurofilaments. These arranged randomly, closely associated granular materials as rough endoplasmic reticulum. Neurofilamentous accumulations reported to be common ALS rare this case. No Bunina body