Stroke recurrence in an elderly CADASIL patient on aspirin discontinuation due to severe auto-immune thrombocytopenia

Abstract

CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy) is a rare inherited cerebrovascular disease caused by mutations of the Notch3 gene and resulting in diffuse microangiopathy (1). The clinical picture of CADASIL is characterized by a variable association of recurrent strokes, cognitive decline, mood disturbances, migraine, often with aura, and epilepsy, usually occurring in mid-adult age (2, 3). No specific treatment currently exists for CADASIL. Counseling and support are the major part of CADASIL patient management and the main target of therapy is control of symptoms (4). Although not yet based on evidence, the strict control of conventional vascular risk factors may have a beneficial effect (5). Current secondary prevention of cerebrovascular events in CADASIL is extrapolated from studies on sporadic stroke; therefore, although there is no specific evidence about its efficacy in CADASIL, aspirin is commonly prescribed for its patients (4). Here we describe an elderly CADASIL patient who underwent stroke recurrence when aspirin treatment was stopped due to severe auto-immune thrombocytopenia.