BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts 
and leukoencephalopathy (CADASIL) is an inherited cerebral small vessel disease, 
clinically characterized by migraine, recurrent transient ischemic attacks or 
strokes, psychiatric disorders and cognitive decline. Strokes are typically 
ischemic, while hemorrhagic events have been only sporadically described. 
However, cerebral microbleeds have been found in 31-69% of CADASIL patients.
METHODS: We describe four unrelated CADASIL patients who had hemorrhagic 
strokes. We also briefly review the literature on intracerebral hemorrhage (ICH) 
in CADASIL.
RESULTS: Three patients had a thalamo-capsular hemorrhage (age at onset: 54, 67, 
77) and one of these had a second hemispheric cerebellar hemorrhage. Another 
patient experienced an interpeduncular cistern subarachnoid hemorrhage when he 
was 39. None of these patients was receiving antiplatelets, anticoagulants or 
statins at the time of hemorrhage; all were hypertensive. NOTCH3 gene analysis 
revealed mutations on exons 14, 22 (two patients presenting the same mutation), 
and 24. MRI signs of previous hemorrhages were present in all these patients.
CONCLUSIONS: Hemorrhagic stroke can occur in CADASIL similarly to sporadic 
cerebral small vessel diseases; this finding expands the phenotype of the 
disease. A diagnosis of CADASIL should probably be considered also in patients 
with ICH. These data bear potential implications in terms of need of better 
control of risk factors, particularly hypertension, and raise relevant questions 
about the use of antiplatelets as prevention measures in CADASIL patients.