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Abstract Details

Spontaneous intracranial hemorrhage and acute multiple bilateral subcortical infarcts, a case of possible CADASIL
Cerebrovascular Disease and Interventional Neurology
P14 - Poster Session 14 (8:00 AM-9:00 AM)
4-007
To report a case of possible CADASIL with spontaneous thalamic hemorrhage and sudden onset of multiple bilateral subcortical infarcts 

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common monogenic inherited form of small vessel disease caused by mutations in Notch 3 gene on Chromosome 19. Spontaneous intraparenchymal hemorrhage and acute bilateral multiple subcortical infarcts are uncommon presentations. 
Case report
A 52 year old Liberian man with a history of hypertension who presented with headaches, dysarthria, and right hemiparesis. He is found to have left thalamic hemorrhage and admitted to the hospital. The next day, his mental status suddenly deteriorated and became comatose. MRI brain revealed multiple bilateral subcortical infarcts. Extensive stroke and vasculitis work up including TEE, cerebral angiogram, hypercoagulability and paraneoplastic panel were unremarkable. A leptomeningeal biopsy showed heavy PAS staining on leptomeningeal vessels, which raised the possibility of CADASIL. Notch 3 DNA Test revealed a Notch 3 gene mutation however the variant detected in the analysis has not been definitively demonstrated to be associated with CADASIL. 
Spontaneous intraparenchymal hemorrhage and acute bilateral multiple subcortical infarcts were previous reported in CADASIL. In our patient, although the Notch 3 gene mutation is not previous reported to be associated with CADASIL, it is possible that this mutation may have affected this patient’s unique presentation, and further investigation of this mutation may be warranted.  
Authors/Disclosures
Chen Chen, MD
PRESENTER 		No disclosure on file
Jacklyn Johnson, MD (Rutgers NJMS) Dr. Johnson has nothing to disclose.
Andrea Hidalgo, MD (Rutgers New Jersey Medical School) Dr. Hidalgo has nothing to disclose.
Nizar Souayah, MD, FÂ鶹´«Ã½Ó³»­ (NJMS) Dr. Souayah has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Takeda. Dr. Souayah has received publishing royalties from a publication relating to health care.
Jayoung Pak, MD (NJMS Rutgers) No disclosure on file
David A. Marks, MD Dr. Marks has nothing to disclose.