Quantitative T2 Measurements in Juvenile and Late Infantile Neuronal Ceroid Lipofuscinosis
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O r i g i n a l A rt i c l e
Quantitative T2 Measurements in Juvenile and Late Infantile Neuronal Ceroid Lipofuscinosis A. Paniagua Bravo · N. D . Forkert · A. Schulz · U. Löbel ·J. Fiehler · X. Ding · J. Sedlacik · M. Rosenkranz ·E. Goebell
Received: 17 August 2012 / Accepted: 29 November 2012 / Published online: 23 December 2012 © Springer-Verlag Berlin Heidelberg 2012
Abstract Purpose The two most prevalent forms of neuronal ceroid lipofuscinosis (NCL) are the juvenile form (Batten disease, CLN3) and late infantile form (Jansky–Bielschowsky disease, CLN2). The aim of this study was to compare quantitative T2-values of brain tissue in CLN2 and CLN3 patients with reference values from age-matched normal subjects. Methods Twenty-three CLN2 (n = 6) and CLN3 (n = 17) patients (m:f = 11:12) underwent MRI examination including a multiecho T2 sequence. Quantitative T2-values were measured in six defined regions of interest (ROIs) in the calculated quantitative T2 maps within the white matter (WM) and gray matter (GM). The extracted quantitative T2-values were compared with reference values from
healthy children and young adults. Informed consent was obtained from the patients or their parents for all patients. Results Statistical analysis revealed elevated quantitative T2-values in nearly all ROIs placed in the WM of the CLN2 patients. In contrast to this finding, no significant differences were found for the quantitative T2-values of the CLN3 patients compared to the age-matched healthy controls in any of the defined WM ROIs. Both groups exhibited no significant alterations of the quantitative T2values in the GM ROIs compared to the healthy subjects. Conclusion Alterations of quantitative T2-values in the cerebral WM may not be a reliable sign to confirm the diagnosis in CLN3 patients but could prove valuable for diagnosis confirmation, follow-up examinations, and longitudinal monitoring of the disease progression in CLN2 patients.
E. Goebell, MD () · A. Paniagua Bravo, MD · U. Löbel, MD · J. Fiehler, MD · J. Sedlacik, PhD Department of Neuroradiology, University Medical Center Hamburg-Eppendorf (UKE), Martinistrasse 52, 20246 Hamburg, Germany e-mail: [email protected]
Keywords Neuronal ceroid lipofuscinosis · T2 relaxometry · Neurodegeneration · CLN2 · CLN3 · Quantitative T2 measurement · Neurodegeneration in children
N. D. Forkert Institute for Computational Neuroscience, University Medical Center Hamburg-Eppendorf (UKE), Martinistrasse 52, 20246 Hamburg, Germany
Quantitative T2-Messungen bei Patienten mit juveniler und spät infantiler neuronaler Lipofuscinose
A. Schulz, MD Department of Paediatrics, University Medical Center HamburgEppendorf (UKE), Martinistrasse 52, 20246 Hamburg, Germany X. Ding, MD, PhD Department of Neuroradiology, Hannover Medical School, Carl-Neuberg-Str. 1, 30625 Hannover, Germany M. Rosenkranz, MD Department of Neurology, University Medical Center HamburgEppendorf (UKE), Martinistrasse 52, 20246 Hamburg, Germany
Zusammenfassung Einleitung Die beiden häufigsten Formen der neuronal
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