To report a patient with Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) presenting with acute ischemic stroke and the progression of ICH.

CADASIL is an inherited cerebral small-vessel disease. Although the primary clinical manifestation of CADASIL is ischemic stroke, spontaneous intracerebral hemorrhage (ICH) has rarely been reported.

Case: A 58-year-old man was admitted complaining of suddenly developed right hemiplegia. On presentation, initial vital signs were normal. Neurological examination showed right sided hemiparesis with MRC grade 4 and NHISS score was 1 point. Laboratory findings on admission did not reveal any abnormalities. There was no medical history such as DM. His mother and brother had a history of stroke. Initial brain diffusion MRI showed acute infarction in the left corona radiata. MRA revealed no significant stenosis. FLAIR image showed severe multiple hyperintensity lesions in the periventricular white matter. The susceptibility weighted imaging MRI sequence demonstrated multiple cerebral microbleeds in both side of pons, cerebellums, basal ganglia, thalamus, cerebral hemispheres. Considering MRI findings and clinical manifestations, we suspected CADASIL and then studied notch3 gene test, which revealed a heterozygous c.224G>C (p.Arg75Pro) mutation, consistent with the diagnosis of CADASIL. Suddenly at 7 days of hospitalization, his mental status was drowsy and developed left hemiplegia. We performed brain CT that showed acute ICH in the right thalamus. We discontinued dual-anti platelet therapy. Two months after, we checked that the hemorrhage was resolved on follow-up Brain CT. We resumed treatment with aspirin mono therapy in the outpatient.

There is no standard treatment available for CADASIL at present. Although there is acute ischemic stroke, we need to carefully choose whether to treat it with dual or mono-anti platelet treatment , considering CADASIL with a lot of CMBs on brain MRI.