Hereditary hypertrophic neuropathy in the trembler mouse. Part 1. Histopathological studies: light microscopy

The Trembler mouse suffers from a dominantly inherited chronic hypertrophic neuropathy. Quantitative light-microscopic studies have been performed on Trembler peripheral nerves from birth to adulthood, and compared with the findings in age-matched control mice. Teased fibres of Trembler nerves contained virtually no myelin, nodes of Ranvier were difficult to identify, and supernumerary Schwann cells were prominent. Results of quantitative studies performed on the dorsal root ganglia and spinal cord of Trembler mice did not differ from those in controls. The density of myelinated fibres in the case of Tremblers was reduced at all ages when compared with controls, and there was a predominant loss of large diameter fibres.