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The entity of young onset primary cervical dystonia
Author(s) -
Koukouni Vasiliki,
Martino Davide,
Arabia Gennarina,
Quinn Niall P.,
Bhatia Kailash P.
Publication year - 2007
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.21421
Subject(s) - cervical dystonia , dystonia , medicine , torticollis , botulinum toxin , spasmodic torticollis , age of onset , depression (economics) , pediatrics , focal dystonia , anxiety , surgery , disease , psychiatry , economics , macroeconomics
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