Abstracts

Rationale:
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a rare condition and the number of cases reported with epileptic manifestations is even less. Seizure has only been seen in about 5-10% of these patients. This rarity can sometimes create doubt regarding management of New onset seizures in those with CADASIL. In this case, patient presented with recurrent seizures due to noncompliance. Thus further highlighting the importance of anti-seizure medications after first time seizures for CADASIL patients.
Method:
Patient is a 62 year old male with a past medical history of CADASIL (positive for missense mutation in NOTCH3), multiple strokes, migraines, who presented to the emergency department after a witnessed seizure. This was his second presentation with seizures after he had his first seizure few weeks earlier. At that time, patient was discharged on sodium valproate 500mg BID. Upon admission, patient’s valproic acid level was sub-therapeutic, nearly undetectable. MRI brain showed a chronic left middle cerebral artery stroke with laminar necrosis. EEG was remarkable for right temporal epileptogenic abnormalities. This provided the patient with some validity to taking the anti-epileptic medications, and he was discharged on sodium valproate 500mg BID.
Results:
This patient was placed on anti-epileptic medication after his first seizure, but there was non-compliance suspected. A component of the non-adherence could have been that there was only one seizure prior and there was doubt about taking a medication for it. Due to the rarity of seizures in patients with the already rare condition of CADASIL, there is very limited documentation as well as lacking data regarding the incidence of recurrent seizures, choice of AEDs and incidence of refractory seizures in patients with CADASIL. 
Conclusion:
This case is important because it highlights the need for anti-seizure management even after the first seizure in those with CADASIL. Even when there is no long-standing history of seizures or seizure disorder, recurrent seizures can certainly be  a manifestation with CADASIL. Despite the novelty of seizures in these patients, they should be placed on anti-epileptic medication to help prevent the re-occurrence of seizure, as seen with this patient. Additional research and case series are needed for a better grasp of seizure characteristic in this group of patients.
Funding:
:none