Intracerebral hemorrhage in CADASIL

Abstract

Background: Intracerebral hemorrhage (ICH) has been reported in few cases of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), mostly in hypertensive patients. We aimed to assess the clinical and radiological characteristics of patients with CADASIL who presented with ICH.

Methods: A retrospective analysis of all neuroimaging exams of CADASIL patients hospitalized in our academic neurology department for acute cerebrovascular events was performed to find ICH. A systematic review of the literature was performed on this topic.

Results: Including our five patients, a total number of 52 subjects with CADASIL and ICH (mean age: 56 years, SD 11, 36-69%- male) were reported. Intracerebral hemorrhages were mainly deep (34 subjects), followed by lobar (8 subjects), infratentorial (6 subjects) and mixed locations (4 subjects). Three ICHs were asymptomatic. Fourteen patients were taking antithrombotic medication, 18 had no regular antiplatelet or anticoagulant treatment while in 20 patients medical treatment was not detailed. Arterial hypertension was present in 37 out of 51 patients with available information. Neuroimaging showed extensive FLAIR hyperintensities in all CADASIL subjects with ICH, cerebral microbleeds in all but three patients, and lacunar infarction in 19 out of 25 subjects with available information.

Conclusions: Intracerebral hemorrhage represents a possible yet uncommon manifestation of CADASIL and should be considered as a possibility in patients with ICH associated with leukoencephalopathy and microbleeds, even in the absence of other clinical symptoms.

Keywords: CADASIL; Intracerebral hemorrhage; Intracranial hemorrhage; Microbleeds; Small vessels disease.