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Published in: Journal of Neurology 9/2019

01-09-2019 | Autopsy | Original Communication

Histopathology of Moyamoya angiopathy in a European patient

Authors: Markus Kraemer, Kathy Keyvani, Peter Berlit, Frank Diesner, Michael Marquardt

Published in: Journal of Neurology | Issue 9/2019

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Abstract

Background and purpose

Moyamoya angiopathy (MMA) is rare outside Asia. Little is known about pathophysiology in European patients. This study aims to elucidate the histopathology of non-Asian MMA and its similarities and differences to those cases from Asia.

Methods

Here, we present a 57-year-old European woman with MMA and describe the post-mortem examination results of the brain and cerebral arteries.

Results

Histopathological findings in cerebral blood vessels were identical to those found in Asians. This included thickening and undulation of the internal elastic lamina as well as fibrocellular thickening and proliferation of smooth muscle cells of the intima in the distal segments of the internal carotid arteries and in proximal and middle segments of the anterior and middle cerebral arteries. Collateral vessels showed fragmented internal elastic lamina and thinning of the media with isolated microaneurysms.

Conclusions

Histopathological changes found in this European patient are identical with those described in Asians. Despite suspected different genetic triggers and unknown pathophysiological cascades, MMA in Europeans seems to result in a common final pathway compared with the disease in Asians.
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Metadata
Title
Histopathology of Moyamoya angiopathy in a European patient
Authors
Markus Kraemer
Kathy Keyvani
Peter Berlit
Frank Diesner
Michael Marquardt
Publication date
01-09-2019
Publisher
Springer Berlin Heidelberg
Keyword
Autopsy
Published in
Journal of Neurology / Issue 9/2019
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-019-09406-w

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