Academic Journal
Arterioectatic Spinal Angiopathy of Childhood:Clinical, Imaging, Laboratory, Histologic, and Genetic Description of a Novel CNS Vascular Pathology
| العنوان: | Arterioectatic Spinal Angiopathy of Childhood:Clinical, Imaging, Laboratory, Histologic, and Genetic Description of a Novel CNS Vascular Pathology |
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| المؤلفون: | Abruzzo, T., van den Berg, R., Vadivelu, S., Hetts, S. W., Dishop, M., Cornejo, P., Narayanan, V., Ramsey, K. E., Coopwood, C., Medici-van den Herik, E. G., Roosendaal, S. D., Lawton, M., Bernes, S. |
| المصدر: | Abruzzo , T , van den Berg , R , Vadivelu , S , Hetts , S W , Dishop , M , Cornejo , P , Narayanan , V , Ramsey , K E , Coopwood , C , Medici-van den Herik , E G , Roosendaal , S D , Lawton , M & Bernes , S 2022 , ' Arterioectatic Spinal Angiopathy of Childhood : Clinical, Imaging, Laboratory, Histologic, and Genetic Description of a Novel CNS Vascular Pathology ' , AJNR. American journal of neuroradiology , vol. 43 , no. 7 , pp. 1060-1067 . https://doi.org/10.3174/ajnr.a7551 |
| سنة النشر: | 2022 |
| الوصف: | Pediatric patients with myelopathy expressing intradural spinal vascular ectasia without arteriovenous shunting were studied at four tertiary referral neuropediatric centers. Patients were identified by retrospective review of institutional records and excluded if spinal vascular pathology could be classified into a previously described category of spinal vascular malformation. Four patients meeting the study criteria were enrolled in the study. Clinical, magnetic resonance imaging, catheter-directed angiography, laboratory, histological and genetic data were analyzed to characterize the disease process and elucidate underlying pathomechanisms. Our study revealed a highly lethal, progressive multi-segmental myelopathy associated with a unique form of non-inflammatory spinal angiopathy featuring diffuse enlargement and tortuosity of spinal cord arteries, spinal cord hyperemia, and spinal cord edema (Arterioectatic Spinal Angiopathy of Childhood). The condition was shown to mimic venous congestive myelopathy associated with pediatric spinal cord arteriovenous shunts on MRI but to have distinct pathognomonic findings on catheter-directed angiography. Clinicopathological, genetic, and neuroimaging features, which are described in detail, closely overlap with those of mitochondrial disease. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| DOI: | 10.3174/ajnr.a7551 |
| الاتاحة: | https://pure.eur.nl/en/publications/7556ea36-70e6-48e6-b195-56d43015d630 https://doi.org/10.3174/ajnr.a7551 http://www.scopus.com/inward/record.url?scp=85134361257&partnerID=8YFLogxK |
| Rights: | info:eu-repo/semantics/closedAccess |
| رقم الانضمام: | edsbas.1AB1884F |
| قاعدة البيانات: | BASE |
| DOI: | 10.3174/ajnr.a7551 |
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