Staged pallidothalamic tractotomy and pallidotomy for chorea‐acanthocytosis

Abstract

AbstractChorea‐acanthocytosis (ChAc) is a progressive degenerative disease, which manifests movement disorders including chorea and dystonia. Deep brain stimulation has been reported to successfully improve chorea and dystonia in ChAc. However, there are no reports of lesioning surgery for ChAc. The patient was a 47‐year‐old female with a mutation in VPS13A, which led to the diagnosis of ChAc, presenting with chorea in bilateral hands. The Unified Huntington's Disease Rating Scale‐Motor Score (UHDRS‐MS) was 51. The patient underwent staged bilateral pallidothalamic tractotomy and pallidotomy, which resulted in 60.8% improvement of UHDRS‐MS (20 at 3‐month postoperative evaluation). There was no perioperative complication. No chorea recurrence was observed during the 2‐year postoperative follow‐up period.