title: Effectiveness of multimodality treatment for autoimmune limbic epilepsy.
abstract: We evaluated the outcome of multimodality treatment in autoimmune limbic epilepsy in 3 consecutive patients (2 male and 1 female; age 33-55 years) presenting with a combination of focal non-convulsive status epilepticus, memory impairment, and psychosis. MRI showed right or bitemporal T2 or FLAIR hyperintensity. Video-EEG showed seizures of right temporo-occipital or bitemporal independent onset. Extensive workup failed to reveal infectious aetiology or an underlying tumour. However, the autoantibody panel was positive for one or more of these antibodies: anti-VGKC, anti-GABAB, anti-VGCC (P/Q, N types), and anti-GAD65. All patients received: (1) conventional antiepileptic drugs including levetiracetam, lacosamide, phenobarbital, lamotrigine, and valproate; (2) immunomodulatory therapy including methylprednisolone, plasmapheresis, and intravenous immunoglobulin; and (3) rituximab. After a 4-6-week in-hospital course, the seizures resolved in all patients but 2 had persistent memory impairment. None had treatment-related complications. At the time of last follow-up, 2-3 months later, 2 patients remained seizure-free while 2 had residual memory impairment. Our findings suggest that multimodality treatment with a combination of conventional AEDs, immunomodulatory therapy, and rituximab is effective and safe in autoimmune limbic epilepsy.
authors.affiliation: Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
authors.forename: Divyanshu
authors.initials: D
authors.lastname: Dubey
authors.affiliation: Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
authors.forename: John
authors.initials: J
authors.lastname: Konikkara
authors.affiliation: Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
authors.forename: Pradeep N
authors.initials: PN
authors.lastname: Modur
authors.affiliation: Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
authors.forename: Mark
authors.initials: M
authors.lastname: Agostini
authors.affiliation: Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
authors.forename: Puneet
authors.initials: P
authors.lastname: Gupta
authors.affiliation: Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
authors.forename: Francy
authors.initials: F
authors.lastname: Shu
authors.affiliation: Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
authors.forename: Steven
authors.initials: S
authors.lastname: Vernino
mesh_terms: D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000188:drug therapy*; D004359:Drug Therapy, Combination; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D020363:Limbic Encephalitis / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000188:drug therapy; D013226:Status Epilepticus / Q000175:diagnosis / Q000188:drug therapy
chemical_list: D000927:Anticonvulsants; D007166:Immunosuppressive Agents
keywords: autoimmune epilepsy; limbic encephalitis; paraneoplastic syndrome; rituximab; status epilepticus