Reversible diffuse white matter lesion in alagille syndrome

Toshiki Takenouchi, Sachiko Shimozato, Kenjiro Kosaki, Suketaka Momoshima, Takao Takahashi

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)


A boy with genetically confirmed Alagille syndrome was incidentally found to manifest striking diffuse hyperintensity of the white matter on T 2-weighted cranial magnetic resonance images. He never exhibited signs of hepatic encephalopathy. For his progressive liver failure, he underwent a live-donor liver transplant at age 2 years, which unexpectedly resulted in a near-complete resolution of the diffuse white matter lesion. Reversible white matter lesions attributed to cerebral edema were reported in adult patients with liver cirrhosis, but not in the pediatric population. The diffuse reversible white matter lesion in the present case demonstrated T2 hyperintensity, coupled with restricted diffusion confirmed by apparent diffusion coefficient, and was suggestive of etiologies such as ischemia or cytotoxic edema rather than vasogenic edema.

Original languageEnglish
Pages (from-to)54-56
Number of pages3
JournalPediatric Neurology
Issue number1
Publication statusPublished - 2011 Jul

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Developmental Neuroscience
  • Clinical Neurology


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