Hereditary cerebellar ataxia of the Finnish Hound and the Norrbotten Spitz. It produces progressive degeneration of the cerebellar cortex with loss of Purkinje cells and affects movement coordination. Affected dogs show signs from an early age and worsen continuously. It is incurable and compromises working ability and quality of life.

Specific to the Finnish Hound; also described in the Norrbotten Spitz. Carrier frequencies have decreased in Finland after the introduction of the genetic test; no recent consolidated figures are published.

- Progressive ataxia from 3-4 months
- Hypermetria and dysmetria
- Intention tremor
- Wide-based gait
- Progressive difficulty in remaining standing
- Preserved mental state

Finnish hound ataxia was investigated in Finland by Hannes Lohi's group, which identified a mutation in the SEL1L gene as the molecular cause (Kyöstilä et al., 2012). The study was based on an experimental colony and clinical cases of the breed. The resulting genetic test was incorporated into Finnish breeding programs to reduce the frequency of carriers.

- Test breeding dogs before mating
- Do not cross two carriers
- A carrier may be crossed with a clear individual; test the offspring intended for breeding
- Always mate carriers with clear individuals to preserve genetic diversity

Onset is early (3-4 months) and progression relatively rapid. Differential diagnosis with other cerebellar ataxias and with acquired processes. MRI shows cerebellar atrophy. Supportive management.

1. Kyöstilä K, Cizinauskas S, Seppälä EH, Suhonen E, Jeserevics J, Sukura A, Syrjä P, Lohi H. 2012. A SEL1L mutation links a canine progressive early-onset cerebellar ataxia to the endoplasmic reticulum-associated protein degradation (ERAD) machinery. PLoS Genet 8(6):e1002759. PMID: 22719266
2. Donner J et al. 2016. Genetic Panel Screening of Nearly 100 Mutations Reveals New Insights into the Breed Distribution of Risk Variants for Canine Hereditary Disorders. PLoS One 11(8):e0161005. PMID: 27525650
3. OMIA:001692-9615. Ataxia, cerebellar, progressive early-onset, SEL1L-related, perro.