Histopathology of Moyamoya angiopathy in a European patient.
Autopsy
Europeans
Histopathology
Moyamoya angiopathy
Journal
Journal of neurology
ISSN: 1432-1459
Titre abrégé: J Neurol
Pays: Germany
ID NLM: 0423161
Informations de publication
Date de publication:
Sep 2019
Sep 2019
Historique:
received:
23
03
2019
accepted:
28
05
2019
revised:
25
05
2019
pubmed:
6
6
2019
medline:
29
1
2020
entrez:
6
6
2019
Statut:
ppublish
Résumé
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. Here, we present a 57-year-old European woman with MMA and describe the post-mortem examination results of the brain and cerebral arteries. 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. 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.
Sections du résumé
BACKGROUND AND PURPOSE
OBJECTIVE
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
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
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
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.
Identifiants
pubmed: 31165233
doi: 10.1007/s00415-019-09406-w
pii: 10.1007/s00415-019-09406-w
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
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