Authors :
Presenting Author: Hui LI, MD – University of Wisconsin- Madison
Abdelrahman Alsherbini, MD – Baylor College of Medicine - - Hosuton, TX
Eyal Muscal, MD – Baylor College of Medicine - - Hosuton, TX
Yi-Chen Lai, MD, PhD – Baylor College of Medicine - - Hosuton, TX
James Riviello, MD – Baylor College of Medicine/Texas Children's Hospital
Cristina Trandafir, MD – Baylor College of Medicine/Texas Children's Hospital
Rationale:
NORSE/FIRES is a rare and devastating form of acute-onset super-refractory status epilepticus, often followed by epileptic encephalopathy in children who did not have a prior diagnosis of epilepsy. Recognizing the characteristic electrographic features in these patients is a crucial step toward expediting diagnosis and initiating appropriate management.
Methods:
A retrospective review identified twenty-two patients with a diagnosis of NORSE/FIRES in our medical record system. EEG data were reviewed from both the acute phase and chronic phase. The acute phase is defined as the period of acute hospitalization due to super-refractory status epilepticus while the chronic phase referred to the period following hospital discharge. Twenty-one patients had EEG data retrievable in acute phase. Twenty patients had EEGs retrievable in chronic phase after excluding the two patients who died during the acute hospitalization. The EEG findings were categorized into background activity, interictal discharges, and ictal patterns. During the acute phase, the localization of seizure onsets was also analyzed. In chronic phase, data analysis focused on interictal findings as ictal events were less commonly captured.
Results:
During acute hospitalization, the most common background EEG finding was a burst suppression pattern, observed in 71% of patients. Interictal EEG showed lateralized or generalized periodic discharges in 71% of cases, while multifocal epileptiform discharges were noted in 95%. In the acute phase, focal-onset seizure patterns were observed in 95% of patients, compared to generalized seizure patterns in 43%. Regarding seizure localization, 62% of patients exhibited a broader (hemispheric) seizure onset. Among the focal seizures, temporal lobe onset was the most frequently recorded compared to other lobes. In the chronic phase, only one patient demonstrated a generalized pattern characterized by interictal generalized paroxysmal fast activity (GFPA). No generalized ictal events were recorded in most recent EEG recordings during chronic phase.
Conclusions:
In the acute phase, seizures tend to have either a generalized onset or a focal onset involving a broad region. Although generalized interictal and ictal patterns are commonly observed during the acute phase of NORSE/FIRES, in this patient cohort, these generalized patterns nearly completely resolve during the chronic phase.
Funding: n/a