Abstracts

Rationale: N/A Methods: N/A Results: HH is a 82 year old woman with a history of hypothyroidism, hypertension, and diabetes who first started having seizures on 7/27/17 that consisted of arm and shoulder jerking and facial twitching. She had no history of memory or cognitive decline. She presented in status epilepticus on 7/31 after multiple GTC seizures and was started on levetiracetam and phenytoin. EEG showed slowing in the bitemporal regions but no clear epileptiform activity. MRI brain was normal at that time. An autoimmune encephalitis panel was sent and returned positive for LGI1 and VGKC antibodies supporting a diagnosis of autoimmune limbic encephalitis. Thus, she was started on IV solumedrol for 5 days followed by prednisone 60 mg daily. Her seizures did not improve despite increasing levetiracetam and phenytoin. She was admitted to the EMU. Two facial brachial dystonic seizures were captured on video recording and slow activity was seen bilaterally on EEG. She received IVIG therapy for a total of 2 grams/kg dose over 5 days. She was transitioned from phenytoin to lacosamide in addition to prednisone 60 mg daily. She had significant improvement in seizure control after the IVIG treatment with complete seizure remission until 3/2018. Whole body PET and CT C/A/P as well as vaginal US did not show any signs of occult malignancy. The prednisone was slowly tapered down and discontinued in January, 2018. She had multiple breakthrough seizures in 3/2018 and was hospitalized and given one dose of IVIG and prednisone as well as increasing her Vimpat. Thereafter, it is being attempted to arrange monthly IVIG infusions. LB is a 53 year old woman with a history of epilepsy. She first started having seizures at 3 years old with normal childhood development other than a possible perinatal anoxic insult during delivery. She presented in status epilepticus in 2004 and was started on phenytoin, which she did not tolerate. She was switched to carbamazepine. MRI of the brain in 2009 showed numerous nonspecific white matter lesions. Levetiracetam was added in July, 2014 for breakthrough seizures and topiramate for chronic migraine headache. She started having new events with staring, confusion, and passing out for a couple of hours so 24-hour EEG and MRI brain were performed. EEG was abnormal due to sleep activated bilateral focal epileptiform discharges in the left anterior-mid temporal, right fronto-temporal, and right anterior mid-temporal regions. MRI brain showed slight progression of scattered bilateral nonspecific white matter lesions. She was admitted to the EMU in November, 2017 for status epilepticus and was started on valproic acid and lacosamide carbamazepine was stopped due to hyponatremia. Given the second episode of status epilepticus, it was decided to obtain epilepsy autoimmune serum panel, which was remarkable for an elevated VGKC antibody. Malignancy workup was negative. She was admitted again to the EMU for possible ongoing seizures given history of significant mood changes. CSF showed elevated protein and autoimmune epilepsy panel on the CSF was negative. During this admission, she received immunoglobulin IgA daily for five treatments. She has been seizure-free since IgA treatment.   Conclusions: These cases illustrate the benefits of maintaining a broad differential in any patient who presents in status epilepticus with a non-lesional brain MRI. It also demonstrates the utility in testing for autoimmune encephalitis even in a patient with an atypical presentation (middle age to elderly with no history of cognitive or memory impairment) because immunotherapy treatment can make a drastic difference in patient outcomes, especially as it pertains to comorbid autoimmune epilepsy. Funding: N/A