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GTR Home > Conditions/Phenotypes > Autosomal dominant sensory ataxia 1

Autosomal dominant sensory ataxia 1

Synonyms
Ataxia, sensory, autosomal dominant

Summary

Autosomal dominant sensory ataxia-1 (SNAX1) is a peripheral neuropathy resulting from the degeneration of dorsal root ganglia that affects both central and peripheral neurites of sensory neurons. Affected individuals show adult onset of slowly progressive clumsiness, gait ataxia, walking difficulties, and distal sensory loss which may be associated with abnormal sensory nerve conduction values. Some patients have vestibular ocular dysfunction. Muscle weakness and atrophy are not observed, and brain imaging is normal (summary by Cortese et al., 2020). [from OMIM]

Available tests

6 tests are in the database for this condition.

Clinical tests (6 available)

Molecular Genetics Tests

Genes See tests for all associated and related genes

  • Also known as: ADSA, SNAX1, SPG85, RNF170
    Summary: ring finger protein 170

Clinical features

Help
Imported from Human Phenotype Ontology (HPO)

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