Autoimmune chorea

Abstract

Chorea is a disorder characterized by relatively fast and irregular movements that mainly affect the limbs and facial muscles. The causes of chorea are varied and can be classified as genetic or acquired. In the present review, we summarize autoimmune chorea, which is an acquired form. The clinical features of autoimmune chorea involve a generally acute or subacute onset and are frequently complicated with other neurological symptoms. Brain MRI rarely shows abnormal intensities in the basal ganglia, and CSF examination usually demonstrates increased cell counts and/or protein concentrations. Hypermetabolism in the basal ganglia has been reported in fluorodeoxyglucose positron emission tomography, indicating that this imaging method may be useful in patients with suspected autoimmune chorea. Moreover, specific anti–neuronal antibodies are sometimes detected in patients and are an important diagnostic marker of autoimmune chorea. Autoimmune chorea is treatable and requires a combination of symptomatic treatment, immunotherapy, anticoagulant therapy, and antitumor therapy, depending on the causative disease.