Do MRI features distinguish Wilson's disease from other early onset extrapyramidal disorders? An analysis of 100 cases
Identifieur interne : 001D40 ( Main/Exploration ); précédent : 001D39; suivant : 001D41Do MRI features distinguish Wilson's disease from other early onset extrapyramidal disorders? An analysis of 100 cases
Auteurs : L. K. Prashanth [Inde] ; S. Sinha [Inde] ; A. B. Taly [Inde] ; M. K. Vasudev [Inde]Source :
- Movement Disorders [ 0885-3185 ] ; 2010-04-30.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Cuivre.
English descriptors
- KwdEn :
- Adolescent, Adult, Basal Ganglia Diseases (classification), Basal Ganglia Diseases (diagnosis), CPM, Child, Child, Preschool, Copper, Diagnosis, Differential, Extrapyramidal syndrome, Face, Female, Hepatolenticular Degeneration (diagnosis), Humans, MRI, Magnetic Resonance Imaging, Male, Midbrain, Nervous system diseases, Nuclear magnetic resonance imaging, Retrospective Studies, Wilson disease, Wilson's disease, Young Adult, early onset extrapyramidal disorders, face of giant panda, midbrain signal changes.
- MESH :
- classification : Basal Ganglia Diseases.
- diagnosis : Basal Ganglia Diseases, Hepatolenticular Degeneration.
- Adolescent, Adult, Child, Child, Preschool, Diagnosis, Differential, Female, Humans, Magnetic Resonance Imaging, Male, Retrospective Studies, Young Adult.
Abstract
Magnetic resonance imaging (MRI) is frequently used in the evaluation of various extrapyramidal disorders. Among the plethora of MRI features in Wilson's disease (WD), only “face of the giant panda” sign has been recognized to distinguish WD from other early onset extrapyramidal disorders (EOEPD). To ascertain the value of various MRI features in differentiating neuropsychiatric form of WD from other EOEPD. This retrospective analysis included 100 patients (M:F = 56:44) of EOEPD (5–40 years), who had undergone MRI during Jan'03 to Nov'08. Their clinical features were recorded and the following MR sequences were analyzed: T1WI, T2WI, FLAIR. Fifty‐six patients had WD (M:F = 28:30, age at onset: 14 ± 6.8 years) and 44 had other EOEPD (M:F = 27:17, age at onset: 19 ± 9.8 years) that included Huntington's disease‐4, young‐onset Parkinson's disease‐7, mitochondrial disorders‐2, Hallervorden‐Spatz disease‐8, non‐Wilsonian hepatolenticular degeneration‐2, toxic/metabolic disorder‐1, and others‐20. The duration of illness at the time of MRI was comparable (WD: 3.1 ± 4.9 years; Other EOEPD: 2.8 ± 2.4 years). MR signal characteristics varied in topography and severity in both the groups. All the patients of WD had signal abnormalities in MRI, as against 16/44 of the other EOEPD group. The following MR observations were noted exclusively in WD: “Face of giant panda” sign (14.3%), tectal plate hyperintensity (75%), central pontine myelinolysis (CPM)‐like abnormalities (62.5%), and concurrent signal changes in basal ganglia, thalamus, and brainstem (55.3%). Besides “Face of giant panda” sign, hyperintensities in tectal‐plate and central pons (CPM‐like), and simultaneous involvement of basal ganglia, thalamus, and brainstem are virtually pathognomonic of WD. © 2010 Movement Disorder Society
Url:
DOI: 10.1002/mds.22689
Affiliations:
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Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2010-04-30">2010-04-30</date><biblScope unit="vol">25</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="672">672</biblScope><biblScope unit="page" to="678">678</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">0F763A45E2F5D9C8165BFCB3862539E774EEB56B</idno><idno type="DOI">10.1002/mds.22689</idno><idno type="ArticleID">MDS22689</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Basal Ganglia Diseases (classification)</term><term>Basal Ganglia Diseases (diagnosis)</term><term>CPM</term><term>Child</term><term>Child, Preschool</term><term>Copper</term><term>Diagnosis, Differential</term><term>Extrapyramidal syndrome</term><term>Face</term><term>Female</term><term>Hepatolenticular Degeneration (diagnosis)</term><term>Humans</term><term>MRI</term><term>Magnetic Resonance Imaging</term><term>Male</term><term>Midbrain</term><term>Nervous system diseases</term><term>Nuclear magnetic resonance imaging</term><term>Retrospective Studies</term><term>Wilson disease</term><term>Wilson's disease</term><term>Young Adult</term><term>early onset extrapyramidal disorders</term><term>face of giant panda</term><term>midbrain signal changes</term></keywords><keywords scheme="MESH" qualifier="classification" xml:lang="en"><term>Basal Ganglia Diseases</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Basal Ganglia Diseases</term><term>Hepatolenticular Degeneration</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Child</term><term>Child, Preschool</term><term>Diagnosis, Differential</term><term>Female</term><term>Humans</term><term>Magnetic Resonance Imaging</term><term>Male</term><term>Retrospective Studies</term><term>Young Adult</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Cuivre</term><term>Face</term><term>Imagerie RMN</term><term>Maladie de Wilson</term><term>Mésencéphale</term><term>Pathologie du système nerveux</term><term>Syndrome extrapyramidal</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Cuivre</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Magnetic resonance imaging (MRI) is frequently used in the evaluation of various extrapyramidal disorders. Among the plethora of MRI features in Wilson's disease (WD), only “face of the giant panda” sign has been recognized to distinguish WD from other early onset extrapyramidal disorders (EOEPD). To ascertain the value of various MRI features in differentiating neuropsychiatric form of WD from other EOEPD. This retrospective analysis included 100 patients (M:F = 56:44) of EOEPD (5–40 years), who had undergone MRI during Jan'03 to Nov'08. Their clinical features were recorded and the following MR sequences were analyzed: T1WI, T2WI, FLAIR. Fifty‐six patients had WD (M:F = 28:30, age at onset: 14 ± 6.8 years) and 44 had other EOEPD (M:F = 27:17, age at onset: 19 ± 9.8 years) that included Huntington's disease‐4, young‐onset Parkinson's disease‐7, mitochondrial disorders‐2, Hallervorden‐Spatz disease‐8, non‐Wilsonian hepatolenticular degeneration‐2, toxic/metabolic disorder‐1, and others‐20. The duration of illness at the time of MRI was comparable (WD: 3.1 ± 4.9 years; Other EOEPD: 2.8 ± 2.4 years). MR signal characteristics varied in topography and severity in both the groups. All the patients of WD had signal abnormalities in MRI, as against 16/44 of the other EOEPD group. The following MR observations were noted exclusively in WD: “Face of giant panda” sign (14.3%), tectal plate hyperintensity (75%), central pontine myelinolysis (CPM)‐like abnormalities (62.5%), and concurrent signal changes in basal ganglia, thalamus, and brainstem (55.3%). Besides “Face of giant panda” sign, hyperintensities in tectal‐plate and central pons (CPM‐like), and simultaneous involvement of basal ganglia, thalamus, and brainstem are virtually pathognomonic of WD. © 2010 Movement Disorder Society</div></front></TEI><affiliations><list><country><li>Inde</li></country></list><tree><country name="Inde"><noRegion><name sortKey="Prashanth, L K" sort="Prashanth, L K" uniqKey="Prashanth L" first="L. K." last="Prashanth">L. K. Prashanth</name></noRegion><name sortKey="Sinha, S" sort="Sinha, S" uniqKey="Sinha S" first="S." last="Sinha">S. Sinha</name><name sortKey="Taly, A B" sort="Taly, A B" uniqKey="Taly A" first="A. B." last="Taly">A. B. Taly</name><name sortKey="Vasudev, M K" sort="Vasudev, M K" uniqKey="Vasudev M" first="M. K." last="Vasudev">M. K. Vasudev</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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