Acta Neurochirurgica

, Volume 161, Issue 2, pp 371–378 | Cite as

In vivo demonstration of blood-brain barrier impairment in Moyamoya disease

  • Alessandro Narducci
  • Kaku Yasuyuki
  • Julia Onken
  • Kinga Blecharz
  • Peter VajkoczyEmail author
Original Article - Vascular Neurosurgery - Ischemia
Part of the following topical collections:
  1. Vascular Neurosurgery - Ischemia



Moyamoya disease (MMD) is a cerebrovascular disorder characterized by fragile vascular system. Previous studies suggested that the blood-brain barrier (BBB) destabilizing cytokine angiopoietin-2 plays a critical role in increasing vascular plasticity and endothelial disintegration in MMD. The aim of this study was to assess cerebrovascular integrity in vivo in patients affected by MMD.


We retrospectively analyzed 11 patients that underwent bypass for MMD (MMD group), 11 patients that underwent bypass for atherosclerotic cerebrovascular disease (ACVD—control group I), and 5 patients that underwent clipping for unruptured aneurysms (non-ischemic—control group II). Sodium fluorescein (NaFL) extravasation was evaluated during videoangiography when checking for bypass patency. A grading system (0, +, ++, +++) was used to define the extent of extravasation. Frequency and intensity of leakage was compared among different groups.


NaFL extravasation appeared in 10/11 (91%) patients with MMD and in 8/11 (73%) patients with ACVD during bypass procedures. Extravasation was observed in none of the patients undergoing clipping for unruptured aneurysms. Although both chronic ischemic patient groups showed a comparably high incidence of NaFL extravasation, the MMD group was characterized by a much greater intensity of NaFL extravasation (grade +++ in 82%) than the ACVD group (grade +++ in 27%, p < 0.05).


We demonstrate blood-brain barrier impairment in MMD patients for the first time in vivo. This may be due to mechanisms intrinsic to the unique pathology of MMD, probably explaining the higher association with hemorrhage and post-operative hyperperfusion.


Blood-brain barrier Bypass Fluorescein angiography Hyperperfusion Moyamoya disease Vascular disorders 



The study was supported by grants from Deutsche Forschungsgemeinschaft (DFG: VA 244/10-1) FOR 2325 NVI and European Stroke Research Network (EUSTROKE HEALTH-F2-2008-202213).

Compliance with ethical standards

The study was approved by the local Ethics Committee (EA4/121/17). The paper is reported following the STROBE statement. All patients signed an informed consent form prior to the surgical procedure. A specific consent form was not required for study enrollment, because of its retrospective nature.

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

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

Authors and Affiliations

  1. 1.Department of NeurosurgeryCharitè-Universitätsmedizin BerlinBerlinGermany
  2. 2.Klinik für Neurochirurgie mit Arbeitsbereich Pädiatrische NeurochirurgieCharité – Universitätsmedizin BerlinBerlinGermany

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