Literature DB >> 33811550

Clinical report of a brain magnetic resonance imaging finding in Noonan syndrome.

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.
© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.

Entities:  

Keywords:  DSC-PWI; MRI; Noonan syndrome; RASopathy; UBO

Mesh:

Substances:

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


  2 in total

1.  Neurofibromatosis bright objects in children with neurofibromatosis type 1: a proliferative potential?

Authors:  P D Griffiths; S Blaser; W Mukonoweshuro; D Armstrong; G Milo-Mason; S Cheung
Journal:  Pediatrics       Date:  1999-10       Impact factor: 7.124

2.  Expanding the SHOC2 mutation associated phenotype of Noonan syndrome with loose anagen hair: structural brain anomalies and myelofibrosis.

Authors:  Karen W Gripp; Dina J Zand; Laurie Demmer; Carol E Anderson; William B Dobyns; Elaine H Zackai; Elizabeth Denenberg; Kim Jenny; Deborah L Stabley; Katia Sol-Church
Journal:  Am J Med Genet A       Date:  2013-08-05       Impact factor: 2.802

  2 in total
  1 in total

1.  MRI and MR neurography features of a patient with Noonan syndrome associated with diffuse thickening of peripheral and cranial nerves.

Authors:  Diogo Goulart Corrêa; Luiz Celso Hygino da Cruz
Journal:  Childs Nerv Syst       Date:  2022-09-29       Impact factor: 1.532

  1 in total

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