Intracranial stent placement in a patient with moyamoya disease

Abstract

Moyamoya disease is a rare cerebrovascular condition characterized by progressive occlusion of the major branches of intracerebral arteries. Angiographic findings include bilateral stenotic lesions at the distal portion of the internal carotid artery or middle and anterior cerebral arteries. The result of these stenoses is a severe impairment of the cerebrovascular reserve capacity. This process is accompanied by the development of an extensive collateral network of pathological vessels at the cerebral base with ‘‘puff of smoke’’ appearance. No known treatment can reverse the primary disease process. Current treatments are designed to prevent strokes by improving blood flow to the affected cerebral hemisphere [1]. Increasingly, surgical revascularization is gaining acceptance as the primary treatment for Moyamoya, given the contrast between poor response to medical therapy and the documented success of surgery [2]. A 34-year-old right-handed female presented at the emergency department complaining of transient dysarthria and sensitive symptoms on the four limbs. During her hospitalization she suffered a sudden episode of aphasia and severe paresis of her right arm. Cranial MRI showed several lesions in both frontal and parietal lobes with restricted diffusion, suggestive of subacute ischemic lesions. The lesions were located in the deep borderzones, implying a hemodynamic origin (Fig. 1). A conventional angiography was consequently performed demonstrating narrowing of the distal portion of both internal carotid arteries consistent with Moyamoya disease. Middle and anterior cerebral arteries of both hemispheres were also affected, with compensation through leptomeningeal and posterior circulation. There were also fine basal collaterals, at least on the right side. Given these findings, the patient was transferred to the Intermediate Care Unit. The next days her neurological status did not improve, while her blood pressure remained around 100/60 mmHg. Echocardiography, tests for acute myocardial infarction, and ECG monitoring were normal. In this situation, the revascularization seemed to be priority. An angioplasty with placement of a Wingspan 2.5 9 20 mm stent (Boston Scientific) in the left middle artery was performed without any complication. The patient’s clinical condition improved quickly. The next day she patient was able to answer simple questions, at day 15 she had limited spontaneous speech, and 1 month later, only little fluency difficulties remained, while the right arm paresis had recovered persisting weakness of the hand. CT with angiographic sequences at the moment of the discharge showed permeability of the stent with recovery of flow in the left middle cerebral artery and disappearance of the collateral compensation. Thirteen months after the angioplasty, another arteriography was performed showing minimal narrowing of the stent without repercussion of the lumen. She has suffered two partial seizures but no new ischemic events. Her current treatment includes double antiaggregation and Levetiracetam. Surgical direct and indirect revascularization techniques are the preferred treatment for moyamoya disease and can ameliorate the progression of this condition, but are associated with significant morbidity and mortality. D. Santirso (&) P. Oliva M. Gonzalez E. Murias P. Vega A. Gil S. Calleja Department of Neurology, Hospital Universitario Central de Asturias, Oviedo, Spain e-mail: carpintirso@gmail.com