Authors :
Presenting Author: Priyanka Sabharwal, MD PhD. FAAN – Medstar Georgetown Universisty
Sigmund Lillian, MD – Medstar Georgetown University
Nicholas Streicher, MD – Medstar Georgetown University
Francis Tirol, MD – Medstar Georgetown University
Prerna Malla, MD – Medstar Georgetown University
Mark Lin, MD PhD – Medstar Washington Hospital Center & Medstar Georgetown University
Rationale:
Given recent advances in neuroimaging, Cerebral Amyloid Angiopathy (CAA) has increasingly been linked to the development of seizures and resulting structural epilepsy in elderly patients1-3. Further, seizures in the context of CAA have been predominantly described with the inflammatory variant (CAA-ri). Moreover, status epilepticus, either identified as the initial manifestation or as a later presentation in the disease course is not well described. Here, we present a series of patients with CAA (1 probable, 4 possible) that presented with status epilepticus but did not have the inflammatory variant of the disease.
Methods:
A retrospective chart review was conducted using our hospital systems electronic medical record. Patients were identified with ICD-10 codes (G40.911, and I68.0). Patients were stratified into possible and probable CAA based on imaging data (MRI brain) and Boston Criteria for CAA.
Results:
Here, we present a case series of five patients that presented with status epilepticus in the setting of CAA. All of our patients were between the age ranges of 65-75 years, presented with refractory status (needed 3 or more ASMs), and did not have status epilepticus as the initial manifestation of disease. Radiologically, all patients had microbleeds, and none of our patients had evidence of superficial siderosis on imaging. Electrographically, Lateralized Periodic Discharges (LPDs) were noted on four patients, but no overt electrographic seizures were noted on cEEG monitoring.
Conclusions:
Our case series expands current understanding of the link between seizures and CAA, and underscores CAA as an important diagnostic consideration in elderly patients with refractory seizures and/or status epilepticus. To our knowledge, our case series is the first to report status epilepticus in this patient population outside of the inflammatory variant of the disease. While immunomodulatory therapy is increasingly becoming a common treatment modality for CAA related inflammation, none of the patients in our case series received steroids offering a unique insight into the natural history of disease in this patient population.Funding: NA