Patients with episodic ataxia type 2 (EA2) belong to the group of cerebellar degenerative diseases with slowly progressive symptoms and signs of cerebellar dysfunction.1–6 Genetically, this autosomal-dominant disorder shows monoallelic disease-causing variants in the CACNA1A gene on chromosome 19p13 which encode a voltage-gated subunit of the neuronal Cav2.1 P/Q-type calcium channel.3 This evidence concerns the gene CACNA1A and episodic ataxia type 2.