Adult-onset EPC associated with Cortical Dysplasia and Peripheral ACH Receptor Antibodies Acutely Transforming into Rasmussen's Encephalitis
Abstract number :
3.157
Submission category :
4. Clinical Epilepsy / 4B. Clinical Diagnosis
Year :
2017
Submission ID :
349890
Source :
www.aesnet.org
Presentation date :
12/4/2017 12:57:36 PM
Published date :
Nov 20, 2017, 11:02 AM
Authors :
Alexander M. Papanastassiou, UT Health San Antonio; Rachel Garvin, UT Health San Antonio; Shaheryar Hafeez, UT Health San Antonio; Ali Seifi, UT Health San Antonio; James Henry, UT Health San Antonio; Linda D. Leary, UT Health San Antonio; Lola C. Morgan,
Rationale: There are rare case reports of Rasmussen’s Encephalitis (RE) evolving from epilepsia partialis continua (EPC). A 23-year-old woman with adult-onset focal epilepsy, secondary to cortical dysplasia documented by previous temporal lobe resections, who presented with EPC affecting her left face and recurrent focal seizures without loss of awareness (LOA). Methods: Case review. Results: The patient presented with focal seizures with LOA and bilateral convulsive seizures at age 19 years old. Due to a signal abnormality in her right temporal lobe, she underwent a brain biopsy indicating cortical dyplasia. As her seizures became medically refractory with the evolution of EPC affecting her left face, she underwent a large right temporal lobectomy. Her focal seizures with LOA became better controlled, but the EPC persisted. She was admitted to our center to undergo ictal SPECT and AED changes, but developed focal status epilepticus. Her EEG demonstrated semiperiodic discharges in the right centroparietal and parietotemporal regions as well as ictal discharges in the right hemisphere. She underwent treatment with anesthetics, such as propofol, ketamine, and pentobarbital. She was also treated empirically with steroids and IVIG. Her serum and cerebrospinal fluid autoimmune panels were normal, except for elevated acetylcholine-receptor (ACH-R) antibodies. She was found to have thymic hyperplasia and underwent thymectomy with subsequent plasmapheresis. Despite these interventions, multiple AED trials, and multifocal laser ablations, she repeatedly failed anesthetic weaning. Ictal EEG indicated progressive frontal lobe involvement over the course of two months, also reflected by repeat MRI and ictal SPECT scans. She underwent a right functional hemispherectomy, with successful weaning from anesthetics and most AEDs. Histopathology and immunohistochemistry supported the diagnosis of RE. Conclusions: Fulminant RE can evolve from chronic EPC with underlying cortical dysplasia. EPC, even with underlying developmental etiology, may require rigorous immunological evaluation and intervention. Funding: None
Clinical Epilepsy