Neurology
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Figures Only
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Correspondence:
Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Correspondence are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Brockmann, K.
Right arrow Articles by Hanefeld, F.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Brockmann, K.
Right arrow Articles by Hanefeld, F.
Related Collections
Right arrow MRS
Right arrow Metabolic disease (inherited)

Neurology 2003;60:819-825
© 2003 American Academy of Neurology

Proton MRS profile of cerebral metabolic abnormalities in Krabbe disease

K. Brockmann, MD, P. Dechent, PhD, B. Wilken, MD, O. Rusch, MD, J. Frahm, PhD and F. Hanefeld, MD

From the Department of Pediatrics and Neuropediatrics (Drs. Brockmann, Wilken, and Hanefeld), Georg-August-University; and Biomedizinische NMR Forschungs GmbH am Max-Planck-Institut für biophysikalische Chemie (Drs. Dechent, Rusch, and Frahm), Göttingen, Germany.

Address correspondence and reprint requests to Dr. Knut Brockmann, Department of Pediatrics and Neuropediatrics, Children’s Hospital, Robert-Koch-Str. 40, 37075 Göttingen, Germany; e-mail: kbrock{at}med.uni-goettingen.de

Background: Krabbe disease (globoid cell leukodystrophy [GLD]) is an autosomal recessive lysosomal disorder affecting the central and peripheral nervous system. The authors performed MRS to characterize metabolic alterations and their regional variation in brain tissue in GLD in vivo.

Methods: Abnormalities of cerebral metabolite concentrations were assessed in seven patients with biochemically proven GLD—four with infantile, two with juvenile, and one with adult subtype—using quantitative localized proton MRS of standardized brain regions.

Results: In infantile GLD, pronounced elevation of both myo-inositol and choline-containing compounds in affected white matter reflected demyelination and glial proliferation. The accompanying decrease of N-acetylaspartate pointed to neuroaxonal loss. Gray matter showed similar, albeit much milder alterations. In juvenile GLD, MRS indicated astrocytosis with minor neuroaxonal damage in white matter. In a patient with adult GLD, results of MRS of affected white matter were close to normal. MRS data are in agreement with histopathologic features of GLD.

Conclusion: Proton MRS provides a powerful tool for assessing metabolic disturbances and the extent of brain damage noninvasively in GLD.




This article has been cited by other articles:


Home page
 J Child NeurolHome page
N. Brunetti-Pierri, M. B. Bhattacharjee, Z. J. Wang, Zili Chu, D. A. Wenger, L. Potocki, J. Hunter, and F. Scaglia
Brain Proton Magnetic Resonance Spectroscopy and Neuromuscular Pathology in a Patient With GM1 Gangliosidosis
J Child Neurol, January 1, 2008; 23(1): 73 - 78.
[Abstract] [PDF]

Home page
 J Child NeurolHome page
C. Hoffmann, B. Ben-Zeev, Y. Anikster, A. Nissenkorn, N. Brand, J. Kuint, and T. Kushnir
Magnetic Resonance Imaging and Magnetic Resonance Spectroscopy in Isolated Sulfite Oxidase Deficiency
J Child Neurol, October 1, 2007; 22(10): 1214 - 1221.
[Abstract] [PDF]

Home page
 RadiologyHome page
J. P. van der Voorn, P. J. W. Pouwels, A. A. M. Hart, J. Serrarens, M. A. A. P. Willemsen, H. P. H. Kremer, F. Barkhof, and M. S. van der Knaap
Childhood White Matter Disorders: Quantitative MR Imaging and Spectroscopy.
Radiology, November 1, 2006; 241(2): 510 - 517.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
H. W. Moser
Peripheral nerve involvement in Krabbe disease: a guide to therapy selection and evaluation.
Neurology, July 25, 2006; 67(2): 201 - 202.
[Full Text] [PDF]

Home page
 NeurologyHome page
T. Linnankivi, N. Lundbom, T. Autti, A. -M. Hakkinen, H. Koillinen, T. Kuusi, T. Lonnqvist, K. Sainio, L. Valanne, T. Aarimaa, et al.
Five new cases of a recently described leukoencephalopathy with high brain lactate
Neurology, August 24, 2004; 63(4): 688 - 692.
[Abstract] [Full Text] [PDF]

Home page
 J Child NeurolHome page
P. B. Barker and A. Horska
Topical Review: Neuroimaging in Leukodystrophies
J Child Neurol, August 1, 2004; 19(8): 559 - 570.
[Abstract] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 2003 by AAN Enterprises, Inc.