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Quantitative t2 measurements in juvenile and late infantile neuronal ceroid lipofuscinosis.

Standard

Quantitative t2 measurements in juvenile and late infantile neuronal ceroid lipofuscinosis. / Bravo, Alvaro Paniagua; Forkert, N D; Schulz, A; Löbel, U; Fiehler, J; Ding, X; Sedlacik, J; Rosenkranz, M; Goebell, E.

in: CLIN NEURORADIOL, Jahrgang 23, Nr. 3, 3, 2013, S. 189-196.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Bravo, AP, Forkert, ND, Schulz, A, Löbel, U, Fiehler, J, Ding, X, Sedlacik, J, Rosenkranz, M & Goebell, E 2013, 'Quantitative t2 measurements in juvenile and late infantile neuronal ceroid lipofuscinosis.', CLIN NEURORADIOL, Jg. 23, Nr. 3, 3, S. 189-196. https://doi.org/10.1007/s00062-012-0189-3

APA

Bravo, A. P., Forkert, N. D., Schulz, A., Löbel, U., Fiehler, J., Ding, X., Sedlacik, J., Rosenkranz, M., & Goebell, E. (2013). Quantitative t2 measurements in juvenile and late infantile neuronal ceroid lipofuscinosis. CLIN NEURORADIOL, 23(3), 189-196. [3]. https://doi.org/10.1007/s00062-012-0189-3

Vancouver

Bravo AP, Forkert ND, Schulz A, Löbel U, Fiehler J, Ding X et al. Quantitative t2 measurements in juvenile and late infantile neuronal ceroid lipofuscinosis. CLIN NEURORADIOL. 2013;23(3):189-196. 3. https://doi.org/10.1007/s00062-012-0189-3

Bibtex

@article{5dc597cb96014d8cb0673bae072d6753,
title = "Quantitative t2 measurements in juvenile and late infantile neuronal ceroid lipofuscinosis.",
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 T2-values 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.",
keywords = "Adolescent, Aminopeptidases, Brain, Brain Diseases, Child, Dipeptidyl-Peptidases and Tripeptidyl-Peptidases, Female, Humans, Image Interpretation, Computer-Assisted, Magnetic Resonance Imaging, Male, Membrane Glycoproteins, Molecular Chaperones, Nerve Fibers, Myelinated, Neuronal Ceroid-Lipofuscinoses, Reproducibility of Results, Sensitivity and Specificity, Serine Proteases, Young Adult",
author = "Bravo, {Alvaro Paniagua} and Forkert, {N D} and A Schulz and U L{\"o}bel and J Fiehler and X Ding and J Sedlacik and M Rosenkranz and E Goebell",
year = "2013",
doi = "10.1007/s00062-012-0189-3",
language = "English",
volume = "23",
pages = "189--196",
journal = "CLIN NEURORADIOL",
issn = "1869-1439",
publisher = "Springer Heidelberg",
number = "3",

}

RIS

TY - JOUR

T1 - Quantitative t2 measurements in juvenile and late infantile neuronal ceroid lipofuscinosis.

AU - Bravo, Alvaro Paniagua

AU - Forkert, N D

AU - Schulz, A

AU - Löbel, U

AU - Fiehler, J

AU - Ding, X

AU - Sedlacik, J

AU - Rosenkranz, M

AU - Goebell, E

PY - 2013

Y1 - 2013

N2 - 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 T2-values 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.

AB - 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 T2-values 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.

KW - Adolescent

KW - Aminopeptidases

KW - Brain

KW - Brain Diseases

KW - Child

KW - Dipeptidyl-Peptidases and Tripeptidyl-Peptidases

KW - Female

KW - Humans

KW - Image Interpretation, Computer-Assisted

KW - Magnetic Resonance Imaging

KW - Male

KW - Membrane Glycoproteins

KW - Molecular Chaperones

KW - Nerve Fibers, Myelinated

KW - Neuronal Ceroid-Lipofuscinoses

KW - Reproducibility of Results

KW - Sensitivity and Specificity

KW - Serine Proteases

KW - Young Adult

U2 - 10.1007/s00062-012-0189-3

DO - 10.1007/s00062-012-0189-3

M3 - SCORING: Journal article

C2 - 23263384

VL - 23

SP - 189

EP - 196

JO - CLIN NEURORADIOL

JF - CLIN NEURORADIOL

SN - 1869-1439

IS - 3

M1 - 3

ER -