"Progressive ataxia and palatal tremor may be sporadic or familial 1-3. The aetiopathogenesis of sporadic progressive ataxia and palatal tremor has not been fully elucidated, but there is typically no lesion to the triangle of Guillain and Mollaret (dentatorubro-olivary pathway) 1-3. It has been postulated that there may be a neurodegenerative basis, and multiple studies have identified pathological evidence of tau deposition in the inferior olives in affected patients post-mortem 1."
"Familial progressive ataxia and palatal tremor is often seen in the context of other broader syndromes with genetic basis, such as Alexander disease, certain spinocerebellar ataxias, coeliac disease, and POLG-related disorders 1-3."
"upper motor neurone signs (e.g. spasticity, weakness)"
"movement disorders (e.g. parkinsonism, dystonia, chorea)"