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Tay's syndrome: MRI
Authors:L. Porto  R. Weis  C. Schulz  P. Reichel  H. Lanfermann  F. E. Zanella
Affiliation:(1) Institut of Neuroradiology, Johann Wolfgang Goethe-University, Schleusenweg 2–16, 60 528 Frankfurt/Main, Germany e-mail: Stalmann.Porto@t-online.de Tel.: 49-69-63 01 54 62 Fax: 49-69-63 01 71 76, DE;(2) Neuropediatric Department, Johann Wolfgang Goethe-University, Frankfurt/Main, Germany, DE;(3) Dermatology Department, Johann Wolfgang Goethe-University, Frankfurt/Main, Germany, DE
Abstract:Tay's syndrome is a trichothiodystrophy associated with congenital ichthyosis. We report the findings on MRI and spectroscopy in a young girl with sparse, short, ruffled hair, dry skin and delayed milestones. T2-weighted images showed prominent diffuse confluent increase in signal symmetrically in all the supratentorial white matter. These findings are similar to those in a previously described case, and consistent with dysmyelination. Spectroscopy showed increased myoinositol and decreased choline. Received: 7 March 2000 Accepted: 13 April 2000
Keywords:Brittle hair  Tay's syndrome  Dysmyelination  Magnetic resonance imaging  Magnetic resonance spectroscopy
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