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.

Diffuse cerebral angiomatosis associated to basilar apex aneurysm.

BACKGROUND: Diffuse cerebral angiomatosis (DCA) is a diffuse infiltration of normal brain by complex vascular structures. It differs from arteriovenous malformations (AVMs) that are composed of a nidus of vessels through which arteriovenous shunting occurs without interposed functional brain parenchyma. A rare subgroup of AVMs is diffuse with no recognizable nidus with functional neuronal tissue interspersed within the malformed vessels. We present a rare association of DCA and cerebral arterial aneurysm, which dramatically influenced the patient's prognosis. CASE DESCRIPTION: A 43-year-old male patient with right hemispheric diffuse cerebral angiomatosis presented with a ruptured basilar tip aneurysm that was successfully embolised. Unfortunately, the patient developed a locked-in syndrome. CONCLUSION: The present report shows a possible association between diffuse cerebral angiomatosis and cerebral aneurysms, but this association appears to be less strong than it is with other AVMs.