Hereditary hypertrophic neuropathy in Tibetan Mastiff dogs.

Hypertrophic neuropathy in a family of inbred Tibetan Mastiff dogs is shown to be due to an autosomal recessive gene. The disease has an onset at 7 to 12 weeks of age and results in varying degrees of paresis. HEREDITARY hypertrophic neuropathies have been described in humans and in mice. In humans these include the autosomal dominant Charcot-Marie-Tooth disease with onset in the first or second decades of life, the autosomal recessive Dejerine-Sottas disease with an onset in infancy or childhood, and the autosomal recessive Refsum's disease with an onset in young adults and additional occular, cutaneous, and cerebellar signs-. The autosomal dominant trembler mouse is the only lower mammal reported to have a hereditary hypertrophic neuropathy. The authors are affiliated with the Department of Clinical Sciences, New York State College of Veterinary Medicine, Cornell University, Ithaca, NY 14853. © 1981, American Genetic Association. The clinical manifestations of hypertrophic neuropathies are motor and sensory impairment, slowed nerve conduction, and enlargement of nerves due to proliferation of Schwann cells following segmental demyelination of peripheral nerves-. This report identifies autosomal recessive hypertrophic neuropathy in Tibetan Mastiff dogs.