Progressive Neuronopathy

Neuronopathies are diseases in which premature degeneration of neurons (nerve cells) occurs. Progressive neuronopathy is a genetic disease characterized by progressive degeneration of motor neurons (nerve cells) in the spinal cord leading to hind limb weakness and incoordination, which deteriorated with exercise. The sign usually progress over several months to paralysis.

Breeds at Risk

The disease occurs in Cairn terriers. An inherited disease is suspected. The disease is different from Krabbe disease, or globoid cell leukodystrophy, an autosomal recessive disorder also found in Cairn terriers which can lead to severe symptoms including seizures, hypotonia, blindness and death.

References:
1. Progressive neuronopathy in two Cairn terrier litter mates. Zaal MD, van den Ingh TS, Goedegebuure SA, van Nes JJ.
2. "Multisystemic chromatolytic neuronal degeneration in Cairn Terriers. A case with generalized cataplectic episodes" by JF Cummings, A de Lahunda, and EL Gasteiger. In: Journal of Veterinary Internal Medicine, March-April 1991, volume 5, issue 2
3. "Globoid cell leukodystrophy in Cairn and West Highland White Terriers" by DA Wenger, T Victoria, MA Rafi, P Luzi, MT Vanier, C Vite, DF Patterson, and MH Haskins. In: Journal of Heredity, January-February 1999, volume 90, issue 1