Introduction

Neuroacanthocytosis (NA) is a rarely reported syndrome affecting the nervous system, first described by Bassen and Kornzweig in 1950.1 NA comprises four genetically diverse conditions: the two core conditions are chorea-acanthocytosis and the McLeod syndrome, but acanthocytes also characterise two other neurological conditions, pantothenate kinase-associated neurodegeneration and Huntington's disease (HD)-like 2.2 3

We describe a case of chorea-acanthocytosis, an autosomal recessive disorder, estimated to affect about 1000 people worldwide, with a mean age of symptom onset of around 35 years. It manifests as a progressive movement disorder that usually includes chorea, seizures, behavioural and cognitive disorders, peripheral neuropathy and (often subclinical) myopathy. Oral-lingual dystonia is a noticeable feature, often resulting in mouth or tongue lacerations. Prominent tongue protrusion dystonia, sometimes causing extrusion of food, is a useful pointer towards the diagnosis.