Spinocerebellar Ataxia Type 36

**Semantic type:** Disease or Syndrome

**Definition:** An autosomal dominant condition caused by mutation(s) in the NOP56 gene, encoding nucleolar protein 56. It is characterized by slowly progressive adult-onset gait ataxia, associated with eye movement abnormalities, tongue fasciculations and variable upper motor neuron signs.

**Synonyms:** - SCA36