The entity of young onset primary cervical dystonia

✍ Scribed by Vasiliki Koukouni; Davide Martino; Gennarina Arabia; Niall P. Quinn; Kailash P. Bhatia

• Publisher: John Wiley and Sons
• Year: 2007
• Tongue: English
• Weight: 60 KB
• Volume: 22
• Category: Article
• ISSN: 0885-3185
• DOI: 10.1002/mds.21421

✦ Synopsis

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