Alexander's disease : magnetic resonance imaging and proton magnetic resonance spectroscopy : report of three cases

Alexander's disease : magnetic resonance imaging and ...

Bibliographic description

title:	Alexander's disease : magnetic resonance imaging and proton magnetic resonance spectroscopy : report of three cases
alternative title:	Choroba Alexandra w badaniu rezonansu magnetycznego i spektroskopii protonowej HMRS : opis trzech przypadków
author:	Jurkiewicz Elżbieta, Pakufa-Kościesza Iwona, Mierzewska Hanna, Bekiesińska-Figatowska Monika, Salomons Gajja S, Kmieć Tomasz, Walecki Jerzy
Journal title:	Polish Journal of Radiology
volume:	71
issue:	1
date of publication:	2006
pages:	93-99
article ID:	445743
ISSN:	1733-134X
eISSN:	1899-0967
URL:	http://archiwum.inforadiologia.pl/download/index/idArt/445743.html
date accessed:	2019-08-29
notes:	Bibliogr. s. 99
language:	Polish
Journal language:	Polish
abstract in english:	Background: Alexander's disease is a rare genetic leukodystrophy connected with mutation of the GFAP gene. Infantile, juvenile, and adult subtypes are described. Case reports: We analyzed MR images in three and HMRS in two cases of Alexander's disease. The examinations were performed with a 1.5T scanner in the SE, FSE, and FLAIR sequences in T1,T2 W I before and after gadolinium injection. Single voxel HMRS was performed. M RI showed extensive abnormal signal in the white matter of the frontal lobes, in external capsules, basal ganglia, posterior limbs of the internal capsules, and the hilus of dentate nuclei. Focal contrast enhancement was seen near the frontal horns. HMRS revealed increased Cho/Cr and mI/Cr ratios and decreased NAA/Cr ratio. The presence of lactate was also observed. Conclusions: MR is useful in imaging typical forms of Alexander's disease. Genetic investigation is necessary for definitive diagnosis. HMRS demonstrates metabolic abnormalities of white matter.
keywords in english:	Alexander's disease, magnetic resonance imaging MRI, proton spectroscopy HMRS
type:	journal article
subtype:	academic paper

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