Reversible diffuse white matter lesion in alagille syndrome

Research output: Contribution to journalArticle

Abstract

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
Volume45
Issue number1
DOIs
Publication statusPublished - 2011 Jul

Fingerprint

Alagille Syndrome
Edema
Hepatic Encephalopathy
Brain Edema
Liver Failure
Liver Cirrhosis
Magnetic Resonance Spectroscopy
Ischemia
Tissue Donors
Pediatrics
Transplants
White Matter
Liver
Population

ASJC Scopus subject areas

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

Cite this

Reversible diffuse white matter lesion in alagille syndrome. / Takenouchi, Toshiki; Shimozato, Sachiko; Kosaki, Kenjiro; Momoshima, Suketaka; Takahashi, Takao.

In: Pediatric Neurology, Vol. 45, No. 1, 07.2011, p. 54-56.

Research output: Contribution to journalArticle

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