- Department of Neurosurgery, Kumamoto University School of Medicine, 1-1-1 Honjo, Kumamoto, Japan
- Department of Neurosurgery, Kurume University School of Medicine, 67 Asahi, Kurume, Japan
DOI:10.4103/2152-7806.100867Copyright: © 2012 Takemoto Y. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Takemoto Y, Morioka M, Nakagawa T, Hasegawa Y, Ohmori Y, Kawano T, Kai Y, Kuratsu J. Prolonged and regionally progressive symptomatic cerebral hyperperfusion syndrome after superficial temporal artery-middle cerebral artery anastomosis in a patient with moyamoya disease. Surg Neurol Int 13-Sep-2012;3:106
How to cite this URL: Takemoto Y, Morioka M, Nakagawa T, Hasegawa Y, Ohmori Y, Kawano T, Kai Y, Kuratsu J. Prolonged and regionally progressive symptomatic cerebral hyperperfusion syndrome after superficial temporal artery-middle cerebral artery anastomosis in a patient with moyamoya disease. Surg Neurol Int 13-Sep-2012;3:106. Available from: http://sni.wpengine.com/surgicalint_articles/prolonged-and-regionally-progressive-symptomatic-cerebral-hyperperfusion-syndrome-after-superficial-temporal-artery-middle-cerebral-artery-anastomosis-in-a-patient-with-moyamoya-disease/
Background:The incidence of symptomatic hyperperfusion syndrome after superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis for patients with moyamoya disease (MMD) approaches 30%. In most cases, hyperperfusion occurs in a localized area and disappears within 1-2 weeks.
Case Description:A 59-year-old female diagnosed with asymptomatic MMD for 4 months became rapidly symptomatic with transient ischemic attacks (TIAs). After left STA-MCA anastomosis surgery, she developed symptomatic hyperperfusion, initially (1-2 weeks after surgery) manifesting with severe headache and lesions located in the left basal ganglia. She then developed (2-5 weeks after surgery) aphasia and right hemiparesis caused by new hyperperfusion lesions located in the left frontal area. At discharge (7 weeks after surgery), she recovered fully without any remaining neurologic deficit and no ischemic lesions.
Conclusion:This report details a rare case of a patient with MMD who presented with regionally progressive hyperperfusion lesions after STA-MCA anastomosis and symptoms that persisted for 5 weeks following surgery. Results from this case suggest that regional differences exist in the functional recovery of cerebrovascular reactivity (CVR) in a patient with rapidly progressive MMD.
Keywords: Cerebral hyperperfusion, moyamoya disease, STA-MCA anastomosis
Moyamoya disease (MMD) is characterized by the presence of moyamoya vessels, which are a collateral network of fine vascular pathways that form due to progressive stenosis or occlusion of the bilateral terminal internal carotid arteries.[
Surgical revascularization for MMD prevents cerebral ischemic attacks by improving cerebral blood flow (CBF). In general, superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis with or without indirect pial synangiosis is considered the standard surgical revascularization treatment for MMD.[
Recently, some authors have reported that symptomatic hyperperfusion after STA-MCA anastomosis for patients with MMD occurs only in the anastomosed region. These authors have also shown that the hyperperfusion state, which can be detected using single-photon emission computed tomography (SPECT), usually disappears within 1 week after bypass surgery, although in most cases, symptoms persisted for 1-2 weeks.[
A 59-year-old female presented with complaints of dull headache. Magnetic resonance (MR) imaging showed no ischemic lesion, but MR angiography showed bilateral stenosis at the terminal portion of internal carotid artery (ICA), proximal anterior cerebral artery (ACA), and middle cerebral artery (MCA). Angiography showed severe stenosis involving the bilateral distal ICA and dense moyamoya vessels. A diagnosis of MMD was made according to criteria from the Research Committee on Spontaneous Occlusion of the Circle of Willis of the Ministry of Health Labor and Welfare, Japan. N-isopropyl-p-[ 123I]-iodoamphetamine single-photon emission computed tomography (123I-IMP-SPECT) did not demonstrate dramatically decreased CBF in the bilateral ACA and MCA territories. 123I-IMP-SPECT with acetazolamide showed no impairment in vascular reactivity. At the time, the working diagnosis was asymptomatic MMD without a decrease in CBF.
Four months after initial presentation, the patient complained of frequent episodes of transient right hemiparesis. MR imaging showed rapid progression of the previously identified stenosis and small white matter ischemic lesions in the bilateral frontal areas. 123I-IMP-SPECT showed decreased CBF in the bilateral ACA and MCA territories and 123I-IMP-SPECT with acetazolamide showed markedly impaired vascular reactivity bilaterally
Preoperative neuro-radiologic examinations and postoperative MR-angiography. (a-d) Left internal carotid artery angiograms; (a) anterior-posterior view, (b) lateral view, (c) 123I-IMP SPECT at rest, and (d) MR-angiography. (e) MR angiography on postoperative Day 1 revealed the patent STA-MCA anastomosis (arrow)
The patient had no neurologic deficits immediately after surgery, but she complained of a severe headache on postoperative day (POD) 1. On POD 2, the headache worsened and the patient began vomiting. On POD 4, 123I-IMP-SPECT showed a significant increase in CBF in the left basal ganglia
The postoperative clinical course and the changes in CBF detected with 123I-IMP SPECT. POD: postoperative days. Arrows indicate the regions of hyperperfusion. The scale of the x-axis in each symptom revealed two grades; maximum (100% height), and incomplete improvement containing over 1 day (50% height)
On POD 7, although the systolic blood pressure was well controlled ranging from 120 to 110mmHg during POD 4-6, the patient returned with recurrent severe headache accompanied by aphasia, dysarthria, right hemiparesis, and numbness in the right upper extremity. Imaging showed that the hyperperfusion lesion had shifted from the left basal ganglia to the regions of the cortex surrounding the anastomosis and included left temporal, parietal, and lateral frontal lesions. The free radical scavenger, edaravone (Mitsubishi Tanabe Pharma Co., Tokyo, Japan), was initiated at this time. Two weeks after surgery, the hyperperfusion area had localized to the left lateral frontal area; however, her symptoms persisted for several weeks. Three weeks after surgery, hyperperfusion on 123I-IMP-SPECT had improved, demonstrating only a small hyperperfusion spot. However, the right hand numbness and right hemiparesis persisted until 5 weeks after surgery. At the time of discharge (7 weeks after surgery), the patient had no neurologic sequelae. Throughout her entire postoperative course, MRI showed no new ischemic or hemorrhagic lesions.
Recent reports have shown that the incidence of symptomatic cerebral hyperperfusion after STA-MCA anastomosis for MMD is as high as 27.5-38.2%.[
In this case, the first symptom to appear was severe headache without apparent focal neurologic deficits, which was associated with hyperperfusion only detectable in the basal ganglia ipsilateral to the operation. Although, Fujimura et al.[
Reported risk factors for hyperperfusion include poor cerebrovascular reactivity (CVR), hemorrhagic-onset, adult-onset, and a small recipient MCA (diameter <1 mm).[
In most patients, the hyperperfusion state, as detected by SPECT, disappeared within 1 week of bypass surgery, although symptoms persisted for 1-2 weeks.[
This case represents the first report of a regionally progressive hyperperfusion syndrome, and the exact mechanisms underlying why the specific time courses of each lesion differ remains unknown. This case showed rapid progression of a temporary ischemic attack over the course of several months, with rapidly progressive M1 stenosis, despite a CBF study 4 months prior that showed no obvious decrease in CBF [Figures
Despite the favorable long-term outcome, some authors suggest that focal cerebral hyperperfusion may cause not only transient focal neurological deficit but also intracranial hemorrhage with the potential for development of a permanent deficit.[
The mechanism and pathophysiology behind hyperperfusion remains unclear and future studies are needed to investigate strategies for prevention of symptomatic cerebral hyperperfusion syndrome.
This report details a rare case of a patient with MMD who presented with regionally progressive hyperperfusion lesions after STA-MCA anastomosis and symptoms that persisted for 5 weeks following surgery. Results from this case suggest that regional differences exist in the functional recovery of CVR in a patient with rapidly progressive MMD.
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