Dystonic Seizures and Intense Hyperperfusion of the Basal Ganglia in a Patient with Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Abstract

This report describes a rare case presenting with dystonic seizures due to anti-N-methyl-D-aspartate (NMDA) receptor encephalitis. The patient was an 18-year-old woman with repeated right-dominant dystonic seizures even under sedation. Single-photon emission computed tomography (SPECT) showed intense hyperperfusion of the caudate nuclei, putamen, globus pallidus, thalamus, and insula on the left side, suggesting encephalitis. Antibodies against NMDA receptors were detected in the sera and cerebrospinal fluids. Immune-mediated treatments were administered. Three months later, the dystonic seizures disappeared. We diagnosed her with anti-NMDA receptor encephalitis. SPECT suggested that the main region of encephalitis was the basal ganglia. Therefore, we propose that the patient’s dystonic seizures may originate from the insula and be generated by intense hyperactivity of the basal ganglia.

Keywords

• Anti-N-methyl-D-aspartate receptor encephalitis
• Acute juvenile female non-herpetic encephalitis
• Dystonic seizure
• Dystonia
• Basal ganglia
• Single-photon emission computed tomography