Degeneration of anterior horn cell in neuronal type of Charcot-Marie-Tooth disease (hereditary motor and sensory neuropathy type II): a Golgi study

Abstract

A morphological study using the Golgi impregnation method was carried out on the anterior horn cells at cervical (C), thoracic (Th), and lumbar (L) levels of the spinal cord in a patient with neuronal type of Charcot-Marie-Tooth disease (hereditary motor and sensory neuropathy type II) and an age-matched control. The present study demonstrated an uneven cell body surface, loss of cells (particularly large cells), loss of dendrites, reduced dendritic extent and an irregular surface and shape of dendrites at the C and L levels. In contrast, hematoxylin and eosin and Klüver-Barrera staining showed only simple atrophy or no change. The Th level of the patient showed none of these changes. Our results suggest that the degeneration or loss of dendrites of anterior horn cells by the Golgi staining method, which is most severe at the L level, is closely related to clinical findings such as muscle atrophy and weakness in neuronal type of Charcot-Marie-Tooth disease.