Neuropsychological impairment in adults with moyamoya angiopathy: preoperative assessment and correlation to MRI and H215O PET

  • Constantin Roder
  • Patrick HaasEmail author
  • Monika Fudali
  • Monika Milian
  • Ulrike Ernemann
  • Philipp T. Meyer
  • Marcos Tatagiba
  • Nadia Khan
Original Article


Patients with moyamoya angiopathy (MMA) are known to have an increased risk of impaired executive function (dysexecutive cognitive syndrome (DCS)). Numbers of moyamoya patients with DCS vary strongly in the literature; evidence of a correlation to affected vascular territories is low. This study aims to identify cognitive impairment in adult moyamoya patients and to correlate findings with imaging results. In addition, the predictive value of individual tests for the identification of DCS was analyzed. Neuropsychological test data of 41 adult moyamoya patients was analyzed for a possible correlation with territorial hypoperfusion on H215O PET with acetazolamide (ACZ) challenge (cerebrovascular reserve—CVR) and infarction patterns observed in MRI. Each vascular territory was analyzed separately and correlated to neuropsychological test results and to the presence of DCS. In total, 41.5% of patients presented with DCS. Significant association of DCS and affection of the right middle cerebral artery (MCA) territory was seen for insufficient CVR in PET (p = 0.030) and for patients with infarctions seen in MRI (p = 0.014). Analysis of individual neuropsychological test results confirmed the main association with the right MCA territory, as well as some association with the right anterior cerebral artery (ACA) territory. Analysis of a subgroup of patients with chronic disease on MRI (presence of large post-infarction gliosis and brain atrophy in affected territories) revealed a significantly higher risk for DCS (85% affected) than non-chronic patients (21% affected) (p < 0.001). Analysis of neuropsychological test data in this moyamoya cohort reveals DCS in 41.5% of all patients. Correlation between DCS and an impairment of CVR seen in PET and/or infarctions seen in MRI was significant for the right MCA territory. Patients with chronic disease had a significantly higher risk for DCS than non-chronic patients (p < 0.001).


Dysexecutive cognitive syndrome Cerebral revascularization Moyamoya disease Moyamoya syndrome Positron emission tomography Neuropsychology 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethics standards. As to the retrospective character of this analysis, no specific formal consent was obtained.

Supplementary material

10143_2019_1192_MOESM1_ESM.docx (20 kb)
ESM 1 (DOCX 20 kb)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Neurosurgery and Moyamoya CenterUniversity of TübingenTübingenGermany
  2. 2.Department of NeuroradiologyUniversity of TübingenTübingenGermany
  3. 3.Department of Nuclear Medicine, Medical CenterUniversity of Freiburg, Faculty of Medicine, University of FreiburgFreiburgGermany
  4. 4.Moyamoya Center, Division of Pediatric Neurosurgery, Department of SurgeryUniversity Children’s Hospital ZürichZürichSwitzerland

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