gms | German Medical Science

57. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e. V. (DGNC)
Joint Meeting mit der Japanischen Gesellschaft für Neurochirurgie

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

11. bis 14.05.2006, Essen

Is Moyamoya disease a subform of cerebral amyloid angiopathy?

Ist die Moyamoya-Erkrankung eine Unterform der zerebralen Amyloid-Angiopathie?

Meeting Abstract

  • corresponding author P. Vajkoczy - Neurochirurgische Klinik, Mannheim
  • P. Horn - Neurochirurgische Klinik, Mannheim
  • S. Rüggeberg - EMBL, Heidelberg
  • T. Franz - EMBL, Heidelberg
  • M. Herzig - Department of Pathology, Lausanne
  • M. Jucker - Department of Pathology, Lausanne
  • H.A. Lehr - Hertie-Institut für Klinische Hirnforschung, Tübingen
  • P. Schmiedek - Neurochirurgische Klinik, Mannheim

Deutsche Gesellschaft für Neurochirurgie. Japanische Gesellschaft für Neurochirurgie. 57. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), Joint Meeting mit der Japanischen Gesellschaft für Neurochirurgie. Essen, 11.-14.05.2006. Düsseldorf, Köln: German Medical Science; 2006. DocP 10.169

Die elektronische Version dieses Artikels ist vollständig und ist verfügbar unter:

Veröffentlicht: 8. Mai 2006

© 2006 Vajkoczy et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen ( Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.



Objective: The etiopathology of the Moyamoya disease remains unknown. Recently, we have performed a proteomic CSF screen of caucasian Moyamoya patients and could identify the 55kDa beta-sheet protein Transthyretin (TTR) as being overexpressed in 80% of patients while none of the atherosclerotic control patients demonstrated increased levels. Wild-type TTR molecules have been reported as being deposited in cerebral vessels as amyloid fibrils leading to a subform of cerebral amyloid angiopathy. Thus, the aim of the present study was to examine whether cerebral blood vessels from Moyamoya patients are characterized by structural blood vessel wall changes and intramural amyloid deposits.

Methods: A part of the vessel wall of the cerebral cortical arteries as well as segments of the superficial temporal arteries were harvested from n=4 adult Moyamoya patients and n=5 athersclerotic control patients undergoing STA-MCA bypass surgery for the treatment of cerebral hemodynamic insufficiency. In addition, segments of the proximal and distal middle cerebral artery were harvested from a Moyamoya patient who died from an extracerebral cause. H&E, Elastic Van Gieson, and Congo Red stainings, as well as immunohistochemistry for TTR were performed.

Results: In the Moyamoya specimens, H&E stainings revealed a loss of the regular blood vessel morphology and architecture, in that the muscular layer of the media was almost completely absent. Elastic Van Gieson stainings demonstrated that the original muscular layer had been replaced by elastic connective tissue fibers. However, Congo Red stainings and immunohistochemistry failed to reveal amyloid deposits and relevant amounts of TTR in these vessel walls.

Conclusions: The cerebral blood vessels of Moyamoya patients are characterized by a unique loss of the regular morphology and architecture of their vessel wall. However, our analyses failed to confirm the hypothesis that this is the result of a TTR cerebral amyloid angiopathy.