| Literature DB >> 33811550 |
Alessandra D'Amico1, Maria Brunella Cipullo2, Mariateresa Falco3, Lorenzo Ugga1, Daniela Melis3.
Abstract
Noonan syndrome (NS) is an autosomal dominant disease caused by aberrant up-regulated signaling through RAS GTPase. It is characterized by facial dysmorphisms, short stature, congenital heart defects, malformations of rib cage bones, bleeding problems, learning difficulties, or mild intellectual disability. Additional intracranial findings in NS patients include tumors, midline anomalies, and malformations of cortical development. In this report, we present the case of a young female patient, with a known diagnosis of Noonan syndrome that in complete well being developed two brain lesions, in the right nucleus pallidus and in the left cerebellar hemisphere respectively, whose location and signal on MRI looked similar to neurofibromatosis type 1 unidentified bright objects (UBOs), and whose spectroscopic characteristics excluded neoplasms.Entities:
Keywords: DSC-PWI; MRI; Noonan syndrome; RASopathy; UBO
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Year: 2021 PMID: 33811550 DOI: 10.1007/s00381-021-05149-0
Source DB: PubMed Journal: Childs Nerv Syst ISSN: 0256-7040 Impact factor: 1.475