Personality Changes, Cognitive Decline, and Jerking Movements

Abstract

A 64-year-old man had development of abnormal movements characterized by grimacing of the left hemiface and posturing of the ipsilateral arm. He had a generalized tonic-clonic seizure, followed by cognitive decline requiring assistance for most activities of daily living. He was readmitted to the hospital for neurologic evaluation. His heart rate was 120 beats/min, he was afebrile. On neurologic examination, he had a Mini-Mental State Examination (MMSE) score of 16/30. The patient was oriented to person only, and he had multiple, frequent, left hemibody jerks during examination. Cerebrospinal fluid analysis indicated increased protein concentration but otherwise normal findings. Laboratory abnormalities included a serum sodium and antibodies to leucine-rich, glioma-inactivated protein 1. Electroencephalography revealed diffuse slowing and bilateral temporal lobe epileptiform discharges. Magnetic resonance imaging of the brain showed T2/fluid-attenuated inversion recovery hyperintensities in the bilateral hippocampi and amygdalae and gadolinium enhancement in the same regions. Brain 18F-fludeoxyglucose–positron emission tomography demonstrated hypermetabolism in the mesiotemporal lobes bilaterally. A diagnosis of limbic encephalitis associated with leucine-rich, glioma-inactivated protein 1-immunoglobulin G antibodies was. The unilateral facial and arm movements were consistent with faciobrachial dystonic seizures and are pathognomonic of the disorder. The patient’s seizure frequency did not improve with antiepileptic drug therapy. The patient was treated with intravenous methylprednisolone, followed by plasmapheresis. This resulted in substantial improvement of his cognitive dysfunction and decrease in the faciobrachial dystonic seizures frequency. Therapy was transitioned to oral prednisone and the patient was discharged from the hospital. This patient had classic features of leucine-rich, glioma-inactivated protein 1 antibody encephalitis: faciobrachial dystonic seizures, personality changes, subacute cognitive decline, hyponatremia, and improvement with immunotherapy.