The entity of young onset primary cervical dystonia
Identifieur interne : 002B35 ( Main/Exploration ); précédent : 002B34; suivant : 002B36The entity of young onset primary cervical dystonia
Auteurs : Vasiliki Koukouni [Royaume-Uni] ; Davide Martino [Royaume-Uni, Italie] ; Gennarina Arabia [Royaume-Uni, Italie] ; Niall P. Quinn [Royaume-Uni] ; Kailash P. Bhatia [Royaume-Uni]Source :
- Movement Disorders [ 0885-3185 ] ; 2007-04-30.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
- Adolescent, Adult, Age of Onset, Botulinum Toxins, Type A (therapeutic use), Child, Child, Preschool, Dystonia, Female, Follow-Up Studies, Humans, Male, Medical History Taking, Movement, Nervous system diseases, Neuromuscular Agents (therapeutic use), Torticollis, Torticollis (drug therapy), Torticollis (physiopathology), primary cervical dystonia, torticollis, young onset.
- MESH :
- chemical , therapeutic use : Botulinum Toxins, Type A, Neuromuscular Agents.
- drug therapy : Torticollis.
- physiopathology : Torticollis.
- Adolescent, Adult, Age of Onset, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Male, Medical History Taking, Movement.
Abstract
Primary cervical dystonia is typically an adult onset condition with symptom onset usually in the fifth and sixth decade. Young onset (<28 years) is uncommon. We report 76 patients with cervical dystonia as a presenting or predominant feature, with disease onset before the age of 28. Male to female ratio was 1.24:1 and the mean onset age was 21 (3–28) years. A family history of tremor and/or dystonia was noted in 26.3%. Depression and anxiety attacks were present in 23.7%.Prior injury or surgery involving the neck was noted in 17.1%. 23 (30.3%) experienced spontaneous partial or complete remissions within the first 5 years of onset, but all relapsed. Cervical dystonia was predominantly rotational torticollis. 30% developed extra‐nuchal dystonia and tremor affecting contiguous parts but in only one there was spread to affect the legs. All 15 patients tested for the DYT1 gene were negative. 74% responded favorably to botulinum toxin injections, whereas none of the 13 patients treated with L‐Dopa preparations had a beneficial response. The distinctive features of this entity are discussed. © 2007 Movement Disorder Society
Url:
DOI: 10.1002/mds.21421
Affiliations:
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Le document en format XML
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Quinn</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Bhatia, Kailash P" sort="Bhatia, Kailash P" uniqKey="Bhatia K" first="Kailash P." last="Bhatia">Kailash P. Bhatia</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="abbrev">Mov. 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="2007-04-30">2007-04-30</date><biblScope unit="vol">22</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="843">843</biblScope><biblScope unit="page" to="847">847</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">2354E6381A19ADE8CFD1F93D31230F4241A45014</idno><idno type="DOI">10.1002/mds.21421</idno><idno type="ArticleID">MDS21421</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>Age of Onset</term><term>Botulinum Toxins, Type A (therapeutic use)</term><term>Child</term><term>Child, Preschool</term><term>Dystonia</term><term>Female</term><term>Follow-Up Studies</term><term>Humans</term><term>Male</term><term>Medical History Taking</term><term>Movement</term><term>Nervous system diseases</term><term>Neuromuscular Agents (therapeutic use)</term><term>Torticollis</term><term>Torticollis (drug therapy)</term><term>Torticollis (physiopathology)</term><term>primary cervical dystonia</term><term>torticollis</term><term>young onset</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Botulinum Toxins, Type A</term><term>Neuromuscular Agents</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Torticollis</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Torticollis</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Age of Onset</term><term>Child</term><term>Child, Preschool</term><term>Female</term><term>Follow-Up Studies</term><term>Humans</term><term>Male</term><term>Medical History Taking</term><term>Movement</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Dystonie</term><term>Système nerveux pathologie</term><term>Torticolis</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Primary cervical dystonia is typically an adult onset condition with symptom onset usually in the fifth and sixth decade. Young onset (<28 years) is uncommon. We report 76 patients with cervical dystonia as a presenting or predominant feature, with disease onset before the age of 28. Male to female ratio was 1.24:1 and the mean onset age was 21 (3–28) years. A family history of tremor and/or dystonia was noted in 26.3%. Depression and anxiety attacks were present in 23.7%.Prior injury or surgery involving the neck was noted in 17.1%. 23 (30.3%) experienced spontaneous partial or complete remissions within the first 5 years of onset, but all relapsed. Cervical dystonia was predominantly rotational torticollis. 30% developed extra‐nuchal dystonia and tremor affecting contiguous parts but in only one there was spread to affect the legs. All 15 patients tested for the DYT1 gene were negative. 74% responded favorably to botulinum toxin injections, whereas none of the 13 patients treated with L‐Dopa preparations had a beneficial response. The distinctive features of this entity are discussed. © 2007 Movement Disorder Society</div></front></TEI><affiliations><list><country><li>Italie</li><li>Royaume-Uni</li></country><region><li>Angleterre</li><li>Grand Londres</li></region><settlement><li>Londres</li></settlement></list><tree><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Koukouni, Vasiliki" sort="Koukouni, Vasiliki" uniqKey="Koukouni V" first="Vasiliki" last="Koukouni">Vasiliki Koukouni</name></region><name sortKey="Arabia, Gennarina" sort="Arabia, Gennarina" uniqKey="Arabia G" first="Gennarina" last="Arabia">Gennarina Arabia</name><name sortKey="Bhatia, Kailash P" sort="Bhatia, Kailash P" uniqKey="Bhatia K" first="Kailash P." last="Bhatia">Kailash P. Bhatia</name><name sortKey="Martino, Davide" sort="Martino, Davide" uniqKey="Martino D" first="Davide" last="Martino">Davide Martino</name><name sortKey="Quinn, Niall P" sort="Quinn, Niall P" uniqKey="Quinn N" first="Niall P." last="Quinn">Niall P. Quinn</name></country><country name="Italie"><noRegion><name sortKey="Martino, Davide" sort="Martino, Davide" uniqKey="Martino D" first="Davide" last="Martino">Davide Martino</name></noRegion><name sortKey="Arabia, Gennarina" sort="Arabia, Gennarina" uniqKey="Arabia G" first="Gennarina" last="Arabia">Gennarina Arabia</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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