J Korean Child Neurol Soc.
2009 Nov;17(2):221-225.
A Case of Neurofibromatosis Type I with Moyamoya Syndrome and Ganglioneuroma in Lung
- Affiliations
-
- 1Department of Pediatrics, College of Medicine, Chungbuk National University Cheongju, Korea. wskim@chungbuk.ac.kr
Abstract
- Neurofibromatosis type I is one of the most common neurocutaneous syndrome which is inherited by autosomal dominant manner, characterized by cafe au-lait spots, axillary freckling, Lisch nodules in iris, multiple neurofibromas and bone involvement with pseudoarthrosis, bowing of the long bone. And Moyamoya disease is a specific disease characterized by progressive idiopathic stenosis and eventual occlusion of the large cerebral arteries at the circle of Willis. In response to the stenosis, an abnormal network of small collateral vessels develops, creating the "puff of smoke". Intracranial lesions associated with Neurofibromatosis type I include optic glioma, sphenoid wing dysplasia, "unidentified bright objects" and cerebrovascular lesions such as Moyamoya syndrome and aneurysm. Moyamoya syndrome is an uncommon association of neurofibromatosis type 1 and lung mass has not frequently been found with neurofibromatosis and moyamoya syndrome. We report a case with Neurofibromatosis type I with Moyamoya syndrome and ganglioneuroma in lung with reviewing literatures.
Keyword
- Full Text Links
-
- Actions
-
Cited
- CITED
-
- Close
- Share
-
- Similar articles
-
- Moyamoya Syndrome: A Window of Moyamoya Disease
- A Case of Neurofibromatosis Associated with Moyamoya Disease
- Concomitant moyamoya syndrome and infratentorial arteriovenous malformation in a neurofibromatosis type 1 patient: a case report
- A Case of Moyamoya Disease with Neurofibromatosis Type I
- A Case of Moyamoya Syndrome Diagnosed by Ophthalmic Examination in a Patient with Moyamoya Disease
