The entity of young onset primary cervical dystonia

Mov Disord. 2007 Apr 30;22(6):843-7. doi: 10.1002/mds.21421.

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.

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset*
  • Botulinum Toxins, Type A / therapeutic use
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Medical History Taking
  • Movement
  • Neuromuscular Agents / therapeutic use
  • Torticollis / drug therapy
  • Torticollis / physiopathology*

Substances

  • Neuromuscular Agents
  • Botulinum Toxins, Type A