title: Jerking & confused: Leucine-rich glioma inactivated 1 receptor encephalitis.
abstract: This is a case of autoimmune encephalitis with features of faciobrachial dystonic seizures (FBDS) pathognomonic for Leucine Rich Glioma inactivated (LGI)1 antibody encephalitis. This voltage-gated potassium channel complex encephalitis is marked by rapid onset dementia, FBDS and hyponatremia, which is sensitive to management with immunotherapy including steroids, IVIG and other agents. In this case report we review the clinical features, imaging and management of this condition.
authors.affiliation: Department of Clinical Neurosciences and Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada. Electronic address: ccasault@ucalgary.ca.
authors.forename: Colin
authors.initials: C
authors.lastname: Casault
authors.affiliation: Department of Clinical Neurosciences and Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Katayoun
authors.initials: K
authors.lastname: Alikhani
authors.affiliation: Department of Clinical Neurosciences and Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Neelan
authors.initials: N
authors.lastname: Pillay
authors.affiliation: Department of Clinical Neurosciences and Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada; Department of Community Health Sciences, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Marcus
authors.initials: M
authors.lastname: Koch
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D000679:Amygdala / Q000000981:diagnostic imaging / Q000187:drug effects / Q000378:metabolism; D000906:Antibodies / Q000097:blood*; D003072:Cognition Disorders / Q000188:drug therapy / Q000209:etiology; D004660:Encephalitis / Q000097:blood* / Q000150:complications / Q000276:immunology* / Q000628:therapy; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D008279:Magnetic Resonance Imaging; D008297:Male; D049268:Positron-Emission Tomography; D011506:Proteins / Q000276:immunology*; D012640:Seizures / Q000188:drug therapy / Q000209:etiology
chemical_list: D000305:Adrenal Cortex Hormones; D000906:Antibodies; D047908:Intracellular Signaling Peptides and Proteins; C116499:LGI1 protein, human; D011506:Proteins
keywords: Faciobrachial dystonic seizures; LGI1 encephalitis; Limbic encephalitis; Paraneoplastic disease; Steroid sensitive encephalopathy