OBJECTIVE: To describe the first case (to our knowledge) of voltage-gated potassium channel-complex protein antibody encephalitis with kinesigenic dyskinesia and cramp-fasciculation syndrome. DESIGN: Case report. SETTING: Hospitalized care. PATIENT: A 38-year-old man with a history of bronchial asthma, eczema, vitiligo, and immune complex mesangiopathic glomerulonephritis presented with abnormal movements. MAIN OUTCOME MEASURES: Clinical examination, magnetic resonance imaging, single-photon emission computed tomography, electromyography and nerve conduction studies, video-electroencephalographic monitoring, plasmapheresis exchange therapy, and intravenous immunoglobulin administration. RESULTS: Clinical examination revealed paroxysmal kinesigenic dyskinesia and fasciculations. Magnetic resonance imaging of the brain revealed a left caudate and left putamen increased signal lesion on T2-weighted and fluid-attenuated inversion recovery sequences as well as increased flow in the same region on single-photon emission computed tomographic scans. Electromyography and nerve conduction studies revealed significant afterdischarges, cramp potentials, and continuous motor activity. The video-electroencephalographic monitoring revealed no epileptiform discharges. The patient dramatically improved after 5 plasmapheresis exchange treatments and a course of intravenous immunoglobulin at 2 gm/kg over 5 divided doses. CONCLUSION: To our knowledge, this is the first report of paroxysmal kinesigenic dyskinesia with voltage-gated potassium channel-complex protein antibody encephalitis associated with the cramp fasciculation syndrome.
OBJECTIVE: To describe the first case (to our knowledge) of voltage-gated potassium channel-complex protein antibody encephalitis with kinesigenic dyskinesia and cramp-fasciculation syndrome. DESIGN: Case report. SETTING: Hospitalized care. PATIENT: A 38-year-old man with a history of bronchial asthma, eczema, vitiligo, and immune complex mesangiopathic glomerulonephritis presented with abnormal movements. MAIN OUTCOME MEASURES: Clinical examination, magnetic resonance imaging, single-photon emission computed tomography, electromyography and nerve conduction studies, video-electroencephalographic monitoring, plasmapheresis exchange therapy, and intravenous immunoglobulin administration. RESULTS: Clinical examination revealed paroxysmal kinesigenic dyskinesia and fasciculations. Magnetic resonance imaging of the brain revealed a left caudate and left putamen increased signal lesion on T2-weighted and fluid-attenuated inversion recovery sequences as well as increased flow in the same region on single-photon emission computed tomographic scans. Electromyography and nerve conduction studies revealed significant afterdischarges, cramp potentials, and continuous motor activity. The video-electroencephalographic monitoring revealed no epileptiform discharges. The patient dramatically improved after 5 plasmapheresis exchange treatments and a course of intravenous immunoglobulin at 2 gm/kg over 5 divided doses. CONCLUSION: To our knowledge, this is the first report of paroxysmal kinesigenic dyskinesia with voltage-gated potassium channel-complex protein antibody encephalitis associated with the cramp fasciculation syndrome.
Authors: Eoin P Flanagan; Amy L Kotsenas; Jeffrey W Britton; Andrew McKeon; Robert E Watson; Christopher J Klein; Bradley F Boeve; Val Lowe; J Eric Ahlskog; Cheolsu Shin; Christopher J Boes; Brian A Crum; Ruple S Laughlin; Sean J Pittock Journal: Neurol Neuroimmunol Neuroinflamm Date: 2015-10-01