Magnetic resonance imaging findings of two cases with West syndrome and hypomelanosis of Ito with hemimegalencephaly: a report of two cases


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GÖKÇE E., Beyhan M., Ocak Karatas S. F.

EGYPTIAN JOURNAL OF RADIOLOGY AND NUCLEAR MEDICINE, vol.53, no.1, 2022 (ESCI) identifier identifier

  • Publication Type: Article / Article
  • Volume: 53 Issue: 1
  • Publication Date: 2022
  • Doi Number: 10.1186/s43055-022-00809-w
  • Journal Name: EGYPTIAN JOURNAL OF RADIOLOGY AND NUCLEAR MEDICINE
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus, EMBASE, Directory of Open Access Journals
  • Keywords: Case report, Hemimegalencephaly, Magnetic resonance imaging, West syndrome, Skin pigmentation
  • Bezmialem Vakıf University Affiliated: Yes

Abstract

Background: Hemimegalencephaly is an unusual congenital non-familial malformation of the brain which is characterized by enlargement of the whole or part of one hemisphere due to neural proliferation and dysfunction in the cell migration. The brain stem and cerebellum may also be involved. There are also the common cortical malformation, unusual white matter proliferation, gliosis, and abnormal myelination in hemimegalencephaly. In addition, structural brain abnormalities like atrophy/hypertrophy, demyelination, gliosis, increased thickness of the cortical grey matter, increase signal intensity in the subcortical white matter, abnormal gyral patterns, blurring of the grey-white matter transition, and hamartomatous aspect can be observed on magnetic resonance imaging.