Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach
Identifieur interne : 000634 ( Main/Exploration ); précédent : 000633; suivant : 000635Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach
Auteurs : Simone Spuler [Allemagne] ; Henriette Krug [Allemagne] ; Christine Klein [Allemagne] ; Isabel Chaure Medialdea [Allemagne] ; Wibke Jakob [Allemagne] ; Georg Ebersbach [Allemagne] ; Doreen Gruber [Allemagne] ; Karl-Titus Hoffmann [Allemagne] ; Thomas Trottenberg [Allemagne] ; Andreas Kupsch [Allemagne]Source :
- Movement Disorders [ 0885-3185 ] ; 2010-04-15.
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Abstract
Extreme forward flexion of the spine, named camptocormia (CC), and head drop syndrome (HD) may be among the most disabling symptoms in Parkinson's disease (PD). This study aims to eludicate the etiology of PD‐associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13–14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the Parkin gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. Aberrant protein aggregation may link PD and CC. © 2009 Movement Disorder Society
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DOI: 10.1002/mds.22913
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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="2010-04-15">2010-04-15</date><biblScope unit="volume">25</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="552">552</biblScope><biblScope unit="page" to="559">559</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">9D421529BE0FE823F02CFE521929343BC7759FC6</idno><idno type="DOI">10.1002/mds.22913</idno><idno type="ArticleID">MDS22913</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Parkinson's disease</term><term>camptocormia</term><term>head drop syndrome</term><term>myopathy</term><term>protein‐surplus myopathy</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Extreme forward flexion of the spine, named camptocormia (CC), and head drop syndrome (HD) may be among the most disabling symptoms in Parkinson's disease (PD). This study aims to eludicate the etiology of PD‐associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13–14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the Parkin gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. Aberrant protein aggregation may link PD and CC. © 2009 Movement Disorder Society</div></front></TEI><affiliations><list><country><li>Allemagne</li></country><region><li>Berlin</li></region><settlement><li>Berlin</li></settlement></list><tree><country name="Allemagne"><region name="Berlin"><name sortKey="Spuler, Simone" sort="Spuler, Simone" uniqKey="Spuler S" first="Simone" last="Spuler">Simone Spuler</name></region><name sortKey="Ebersbach, Georg" sort="Ebersbach, Georg" uniqKey="Ebersbach G" first="Georg" last="Ebersbach">Georg Ebersbach</name><name sortKey="Gruber, Doreen" sort="Gruber, Doreen" uniqKey="Gruber D" first="Doreen" last="Gruber">Doreen Gruber</name><name sortKey="Hoffmann, Karl Itus" sort="Hoffmann, Karl Itus" uniqKey="Hoffmann K" first="Karl-Titus" last="Hoffmann">Karl-Titus Hoffmann</name><name sortKey="Jakob, Wibke" sort="Jakob, Wibke" uniqKey="Jakob W" first="Wibke" last="Jakob">Wibke Jakob</name><name sortKey="Klein, Christine" sort="Klein, Christine" uniqKey="Klein C" first="Christine" last="Klein">Christine Klein</name><name sortKey="Krug, Henriette" sort="Krug, Henriette" uniqKey="Krug H" first="Henriette" last="Krug">Henriette Krug</name><name sortKey="Kupsch, Andreas" sort="Kupsch, Andreas" uniqKey="Kupsch A" first="Andreas" last="Kupsch">Andreas Kupsch</name><name sortKey="Kupsch, Andreas" sort="Kupsch, Andreas" uniqKey="Kupsch A" first="Andreas" last="Kupsch">Andreas Kupsch</name><name sortKey="Medialdea, Isabel Chaure" sort="Medialdea, Isabel Chaure" uniqKey="Medialdea I" first="Isabel Chaure" last="Medialdea">Isabel Chaure Medialdea</name><name sortKey="Spuler, Simone" sort="Spuler, Simone" uniqKey="Spuler S" first="Simone" last="Spuler">Simone Spuler</name><name sortKey="Spuler, Simone" sort="Spuler, Simone" uniqKey="Spuler S" first="Simone" last="Spuler">Simone Spuler</name><name sortKey="Trottenberg, Thomas" sort="Trottenberg, Thomas" uniqKey="Trottenberg T" first="Thomas" last="Trottenberg">Thomas Trottenberg</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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