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