Autoimmune Epileptic Encephalitis: A case series
Abstract number :
3.157
Submission category :
4. Clinical Epilepsy / 4B. Clinical Diagnosis
Year :
2016
Submission ID :
199109
Source :
www.aesnet.org
Presentation date :
12/5/2016 12:00:00 AM
Published date :
Nov 21, 2016, 18:00 PM
Authors :
Carlos Lastra, Saint Peter's University Hospital; RWJ - Rutgers Medical School, New Brunswick, New Jersey and Anshul Vagrecha, Saint Peter's University Hospital
Rationale: Autoimmune epileptic encephalitis is being increasingly identified in pediatric patients with acute refractory epilepsy and mental status changes which in part can be attributed to the availability of testing for antibodies targeting the CNS. Although these disorders share common symptoms (mild fever, psychiatric symptoms, seizures) they differ in their etiologic factors. Normal neuroimaging and mild to no pleocytosis is found. We report a case series of three pediatric autoimmune encephalitides caused by two different antibody groups that presented with progressive epilepsy. The case series also depicts the role of earlier and more aggressive immune modulating therapy and probably its effect on the outcome. Methods: Systematic retrospective chart review Results: Case 1. A 4 year old previously healthy girl presented with mood swings, obsessive compulsive behaviors and complex partial seizures eventually developing gait disturbances, dysphagia and dysarthria. The EEG showed ictal episodes and generalized slowing. IVIG followed by methyl prednisolone was administered. Symptoms worsened requiring 2 courses of Rituximab. CSF and serum were positive for anti NMDA receptor autoantibody (anti NR1). Oncologic workup was negative. IgG levels were normal while the MRI revealed mildly dilated ventricles. Case 2. A 14 year old boy was admitted after a 2 week history of psychotic symptoms including visual and auditory hallucinations; multiple generalized and focal seizures in the second week. Encephalitis workup including an autoimmune encephalitis panel was performed.The seizures were being controlled by Topiramate and Lacosamide. IVIG and systemic corticosteroids were also used. The serum and CSF were positive for anti NMDA Ab (anti NR1). The response to these therapies was suboptimal prompting the addition of Rituximab (total 3 courses) which led to a drastic improvement. Case 3. A 9 year old previously healthy boy presented with a 3-4 day acute febrile illness followed by refractory status epilepticus requiring multiple AEDs and pentobarbital coma. 2 courses of IVIG and 2 courses of systemic corticosteroids did not improve the condition. The serum and CSF were positive for anti GAD 65 autoantibodies and the patient responded to 3 Rituximab courses. The patient required rehabilitation. 10 months after the first episode, the AEDs are being weaned off, however, the patient was found to have panhypogammaglobulinemia. Conclusions: Autoimmune epileptic encephalitis presents with new onset refractory epilepsy or status epilepticus and altered mental status in the pediatric population especially in the setting of negative infectious work up. Different antibodies including anti NMDA, anti GAD 65, anti AMPA, anti aquaporin-4, anti VGCC, Hashimoto antibodies, anti GABA, anti LGI 1 have been implicated for these diseases. This case series should raise awareness about this evolving group of immune modulating disorders which is often given different terms that may include antibody associated Inflammatory Brain Disease, autoimmune encephalitis and some patients grouped under Febrile Illness related epileptic seizures (FIRES). Early diagnosis and aggressive treatment may improve prognosis. Funding: none
Clinical Epilepsy