Superficial temporal artery-to-middle cerebral artery bypass surgery for middle cerebral artery stenosis in a patient with cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy

Daisuke Muta,Junichi Kuratsu,Naoki Shinojima,Takayuki Kawano

doi:10.1186/s40064-015-1407-7

Daisuke Muta, Junichi Kuratsu + Show 2 more

Open Access

https://doi.org/10.1186/s40064-015-1407-7

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Journal: SpringerPlus	Publication Date: Oct 14, 2015
Citations: 8	License type: CC BY 4.0

Affiliation: Kumamoto University

Abstract

Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy is a rare hereditary small vessel disease. Ischemic events are the main clinical manifestation of this condition. Here, we present a case in which superficial temporal artery-to-middle cerebral artery anastomosis was performed in a patient with cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy who developed cerebral infarctions caused by severe middle cerebral artery stenosis. Cerebral blood flow and cerebrovascular reactivity were effectively improved using double anastomoses. To our knowledge, surgical revascularization for patients with this condition has not yet been described in the literature. Superficial temporal artery-to-middle cerebral artery anastomosis is effective for patients with cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy who show marked regional cerebral hypoperfusion.

Highlights

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary, monogenic small-vessel disease caused by mutations in the Notch3 gene on chromosome 19 (Chabriat et al 2009; Joutel et al 1996)
Subcortical lacunar infarcts are typically seen in CADASIL, while watershed infarcts have been reported only sporadically, and the cause is considered to be microcirculatory hypoperfusion. (Gordhan and Hudson 2013) In our case, middle cerebral artery (MCA) stenosis was responsible for the watershed infarctions
Chabriat et al observed that the severity of white matter hypoperfusion measured by MR imaging (MRI) might be related to the clinical severity of CADASIL. (Chabriat et al 2000) it was expected that the augmentation of hypoperfusion by major artery stenosis would aggravate the symptom

Summary

Background

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary, monogenic small-vessel disease caused by mutations in the Notch gene on chromosome 19 (Chabriat et al 2009; Joutel et al 1996). One month after the infarction, [123I] N-isopropyl-p-iodoamphetamine single photon emission computed tomography (IMP-SPECT) revealed a significant decrease in regional CBF (Fig. 4a) and CVR (Fig. 4b) under the acetazolamide challenge test occurring predominantly in the territory of the right MCA. On postoperative day 1, an IMP-SPECT study showed a 50 % increase in uptake in the right MCA territory but not hyperperfusion, and MRI demonstrated the patency of the double anastomosis (data not shown). Sixteen months have already passed since surgery, and the patient is doing well Cerebrovascular events such as TIA, infarction and hemorrhage have not appeared. Follow up MRI 14 months after surgery showed no remarkable area of increased intensity indicating white matter abnormalities in the left anterior temporal lobe, both anterior frontal lobes, external capsules, or periventricular regions on FLAIR images (Fig. 5a–c). MR angiography was used to confirm the patency of the bypass (Fig. 5d)

Findings

Discussion and evaluation

Conclusions