Alagille syndrome associated with angiographic moyamoya

We describe the case of a 13-year-old boy with Alagille syndrome in whom intracranial imaging was performed following a seizure. The MRI and MRA revealed changes of angiographic moyamoya within both the anterior and posterior circulation. This very rare manifestation of the systemic vasculopathy in...

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Bibliographic Details
Published in:Child's nervous system 2002-04, Vol.18 (3-4), p.186-190
Main Authors: CONNOR, S. E. J, HEWES, D, BALL, C, JAROSZ, J. M
Format: Article
Language:English
Subjects:
Alagille Syndrome - complications
Biological and medical sciences
Carotid Stenosis - etiology
Humans
Infant
Magnetic Resonance Angiography
Magnetic Resonance Imaging
Male
Medical sciences
Moyamoya Disease - complications
Moyamoya Disease - diagnosis
Moyamoya Disease - etiology
Neurology
Seizures - etiology
Vascular diseases and vascular malformations of the nervous system
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Summary:We describe the case of a 13-year-old boy with Alagille syndrome in whom intracranial imaging was performed following a seizure. The MRI and MRA revealed changes of angiographic moyamoya within both the anterior and posterior circulation. This very rare manifestation of the systemic vasculopathy in Alagille syndrome has not been previously documented in a patient without a focal neurological deficit. We discuss the potential role of routine intracranial imaging in patients with Alagille syndrome.
ISSN:0256-7040
1433-0350
DOI:10.1007/s00381-001-0518-3