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The entity of young onset primary cervical dystonia

Koukouni, V; Martino, D; Arabia, G; Quinn, NP; Bhatia, KP; (2007) The entity of young onset primary cervical dystonia. MOVEMENT DISORD , 22 (6) 843 - 847. 10.1002/mds.21421.

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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. (c) 2007 Movement Disorder Society.

Type: Article
Title: The entity of young onset primary cervical dystonia
DOI: 10.1002/mds.21421
Keywords: primary cervical dystonia, torticollis, young onset, IDIOPATHIC TORSION DYSTONIA, SPASMODIC TORTICOLLIS, ADULT-ONSET, CLINICAL CHARACTERISTICS, FOCAL DYSTONIAS, FAMILY, EXCLUSION, HISTORY, LOCUS, AGE
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences
URI: http://discovery.ucl.ac.uk/id/eprint/71245
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