Development of onion bulb neuropathy in the Trembler mouse

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Abstract
The Trembler mouse is a neurological mutant showing dominant inheritance. The peripheral nerves show a delay in myelination, segmental demyelination and the development of an onion bulb neuropathy. Using electron microscopy a comparative morphometric study of nerve fibres in the sciatic nerve in normal and Trembler mice was made. The animals ranged in age from 3 days to adult. The following features were noted: Mean axon diameters of the Trembler mice were smaller than normal at all ages. Myelination was delayed and myelin was thinner than normal in the Trembler mice. The ratio of myelin thickness to axon diameter was reduced in Trembler mice. There were no significant quantitative or qualitative differences observed between middle and distal levels of Trembler nerves. The balance of evidence from these findings and the previous morphological studies is in keeping with the suggestion that the primary lesion resides in the Schwann cell.