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Abstract Details

Migraine-like Prodrome Preceding New Onset Refractory Status Epilepticus (NORSE) Due to Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis in a Child
Autoimmune Neurology
Autoimmune Neurology Posters (7:00 AM-5:00 PM)
106
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Clinical presentation of Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis can be atypical in children. We report a case of new-onset refractory status epilepticus (NORSE) due to anti-NMDAR encephalitis presenting with migraine-like features in the initial 2 weeks of illness.
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A previously healthy 12-year-old boy presented to the emergency department (ED) with 2-week history of recurrent headache with blurred vision, flashing lights, and seeing diamond shapes. He had been seen in the ED and neurology clinic for this and received treatment for migraine in the preceding days. He was hospitalized due to persistent headache and visual symptoms; he also developed new symptoms of intermittent numbness of the left hand and face. His neurological examination, brain MRI and routine 20 minute EEG were normal. Continuous bedside video EEG revealed multiple bilateral independent parietal-occipital EEG seizures, many manifesting with headache and irritability. CSF obtained 18 days from headache onset showed lymphocytic pleocytosis (136 WBCs/uL), and elevated protein (115 mg/dL). CSF NMDAR antibody was positive at 1:1 titer, with repeat titers 10 weeks from symptom onset at 1:80. Serum NMDAR antibodies and other common autoantibodies were negative. Seizures became frequent despite trial of 4 anti-seizure medications, requiring medication induced coma. Brain PET showed bilateral asymmetric mesial temporal hyper metabolism. Screening for occult malignancy was negative. He required prolonged treatment for super refractory status epilepticus. Patient received immunotherapy with plasmapheresis, steroids, IVIG, rituximab, cyclophosphamide, and tocilizumab over a course of 10 weeks.  Despite attaining seizure control and weaning sedative medications, he remained encephalopathic, 12 weeks after onset of illness.

We report an atypical presentation of NORSE due to anti-NMDAR encephalitis with visual auras and headache without behavioral or cognitive symptoms in the initial 2 weeks. Recognition of parieto-occipital seizures as the likely cause of symptoms prompted workup for autoimmune encephalitis in this patient.

Authors/Disclosures
Ahmad Mahadeen, MD (University of Mississippi Medical Center)
PRESENTER
Dr. Mahadeen has nothing to disclose.
Carolyn Goldschmidt, DO (NorthShore University HealthSystem) Dr. Goldschmidt has nothing to disclose.
Deepak K. Lachhwani, MD, FAAN (Cleveland Clinic Foundation/) The institution of Dr. Lachhwani has received research support from ICON Clinical Research LLC.
Elia Pestana-Knight No disclosure on file
Ahsan M. Naduvil Valappil, MD (Cleveland Clinic) Dr. Naduvil Valappil has nothing to disclose.