Rationale:
Cerebral amyloid angiopathy (CAA) is an important cause of spontaneous intracerebral hemorrhage with an estimated prevalence of 2.3% that increases with age. Around a third of patients develop epilepsy during their disease course. Despite the growing research in CAA, neurophysiological detailed findings are lacking, and seizure outcome data has been limited, this research attempts to address this knowledge gap.
Methods:
We performed a retrospective chart review of patients with an ICD10 code consistent with CAA who underwent scalp electroencephalography (EEG) and were cared for at Brigham and Women’s Hospital. Only patients who fulfilled the Boston criteria 2.0 for CAA were included. Data on patients’ demographics, vascular risk factors, clinical presentations, anti-seizure medications, neuroimaging, neurophysiological findings, and outcomes were extracted.
Results:
Fifty patients were included in the current analysis (29% women), mean age of 74.36 at diagnosis (standard deviation of 8.34).
Thirty-nine patients had probable cerebral amyloid angiopathy, possible (n=9), definite (n=2). Vascular risk factors included: hypertension (n=40), hyperlipidemia (n=35), diabetes mellitus (n=9), current smoker (n=2), former smoker (n=22), obesity (n=11, class 1 n=1, class 2 n=9, class 3 n=1). Clinically, 35 patients presented with spontaneous hemorrhage, cognitive impairment (n=22), and transient focal neurological episodes (n=8). MRI findings included: lobar cerebral microbleed were seen in forty patients, cortical superficial siderosis (n=21), and convexity subarachnoid hemorrhage (n=12). Widened perivascular spaces were present in 33 patients and white matter changes (n=41). A total of 63 EEGs were performed (n=9 had more than 1 EEG). Twenty-one EEGs were inpatient long-term monitoring (LTM) EEGs, while 40 EEGs were routine. Epileptiform findings were noted in 9/16 LTM studies with 4/16 capturing seizures with 2/16 showing status epilepticus. Epileptiform abnormalities were noted in 4/29 of the routine EEGs. Sharp waves were seen in right frontal (n=1), left frontal (n=1), left fronto-temporal (n=1), right temporal (n=2), left hemisphere (n=1), fronto-central (n=2), lateralized periodic discharges in left hemisphere (n=1), left frontal (n=1), right centro-parietal (n=1), and left posterior quadrant (n=1).
Seizure semiology in the 19 who developed seizures included: focal aware seizures (n=11), focal impaired aware (n=6). and generalized tonic clonic seizures (n=4). After a mean follow up of 1.01 years, all patients were seizure free on a median number of 1 antiseizure medication (range 1-2)..
Conclusions:
Seizures in CAA tend to be focal aware seizures and are responsive to medications. Epileptiform abnormalities are common findings in those who undergo EEG. Further research needs to be done to evaluate predictors of epileptiform abnormalities and seizures in patients with CAA.
Funding:
None