The Treatment of Symptomatic Diffuse Proliferative Cerebral Angiopathy With Cilostazol: A Case Report

Diffuse proliferative cerebral angiopathy (DPCA) is a rare form of cerebral vascular malformation responsible for 3.4% of all cerebral arteriovenous malformations (AVMs). The relative risk of rupture for DPCA is lower than for classic AVMs, so they are often managed medically. Despite the somewhat lower rupture risk, the risk of rebleeding is paradoxically higher than in classical AVMs, and there is a potential for significant morbidity and mortality. The aim of this article is to describe a case of a patient with symptomatic DPCA who experienced symptomatic improvement after treatment with the vasodilating agent cilostazol. This is a case report of a patient who presented with aphasia and was found to have DPCA. CT perfusion with acetazolamide challenge confirmed that the patient's symptoms were due to steal phenomena. Subsequently, the patient was treated with 50 mg of cilostazol daily to mediate a vasodilatory response within the arterial tree. Within three days of treatment with cilostazol, the patient showed significant improvement in his aphasia. The patient returned to the clinic a month later and reported continued improvement in his aphasia with speech therapy. Patients who present with neurological deficits from steal phenomena caused by DPCA are difficult to treat surgically because DPCAs often involve normal parenchyma. We present an example of a noninterventional alternative, oral cilostazol paired with functional rehabilitation, for alleviating symptoms associated with DPCA.