Reversible Diffuse White Matter Lesion in Alagille Syndrome - 02/08/11
, Sachiko Shimozato, MD a, Kenjiro Kosaki, MD a, Suketaka Momoshima, MD b, Takao Takahashi, MD a
Communications should be addressed to: Dr. Takenouchi; Department of Pediatrics; Keio University School of Medicine; 35 Shinanomachi; Shinjuku, Tokyo 1608582, Japan.Abstract |
A boy with genetically confirmed Alagille syndrome was incidentally found to manifest striking diffuse hyperintensity of the white matter on T2-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.
Le texte complet de cet article est disponible en PDF.Plan
Vol 45 - N° 1
P. 54-56 - juillet 2011 Retour au numéro
Article précédent
- A Novel GPR56 Mutation Causes Bilateral Frontoparietal Polymicrogyria
- Rong Luo, Hye Min Yang, Zhaohui Jin, Dicky J.J. Halley, Bernard S. Chang, Lesley MacPherson, Louise Brueton, Xianhua Piao
- MRI Features of 4 Female Patients With Pyruvate Dehydrogenase E1 alpha Deficiency
- Nicholas Ah Mew, Johanna B. Loewenstein, Nadja Kadom, Uta Lichter-Konecki, Andrea L. Gropman, Jodie M. Martin, Adeline Vanderver
Bienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.
Déjà abonné à cette revue ?