Idiopathic cervical dystonia: Clinical characteristics

Authors

  • Jane Chan,

    1. Saul R. Korey Department of Neurology, Albert Einstein College of Medicine, Bronx
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  • Mitchell F. Brin,

    1. Dystonia Clinical Research Center, Neurological Institute, Department of Neurology, Columbia University, New York, New York, U.S.A.
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  • Dr. Stanley Fahn

    Corresponding author
    1. Dystonia Clinical Research Center, Neurological Institute, Department of Neurology, Columbia University, New York, New York, U.S.A.
    • Neurological Institute, 710 West 168th Street, New York, NY 10032, U.S.A.
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Abstract

We reviewed detailed clinical features of 266 patients with idiopathic cervical dystonia, commonly called spasmodic torticollis. Mean age at onset (41 years), female-to-male ratio (1.9:1), clustering of onset between ages 30 and 59 (70%), familial history of dystonia (12%), and remissions (9.8%) were similar to those found in previous studies. In contrast to the single prior large clinical study of this disorder, no predominance of right-handers or significant thyroid disease was found. Pain, which occurred in 75% of patients and contributed to disability score (p <0.01), distinguishes this syndrome from all other focal dystonias. Pain was also strongly associated with constant (vs. intermittent) head turning, severity of head turning, and presence of spasm. Eighty-three percent of patients had deviation of the head of >75% of the time when sitting with the head unsupported (constant head deviation at rest). Of the 97% who had head turning, 81% also had head tilting in various combinations. The 23% with hand tremor had an older age at onset (mean, 46 vs. 41 years; p <0.05). An earlier age at onset (p <0.05) was seen in patients with a family history of dystonia (mean, 36 years), with trauma shortly preceding symptoms (mean, 36 years), with a change in the direction of head turning (mean, 30 years), and with remissions (mean, 33 years). Jerky movements or forced transient spasms of the head occurred in 62% of the patients, and these patients would be the ones for whom the designation “spasmodic torticollis” could logically apply. Therefore, because the cervical dystonia in 38% of patients is not spasmodic, we propose that the term “spasmodic torticollis” is not a completely appropriate designation for this condition. We suggest labeling the condition either idiopathic cervical dystonia or idiopathic dystonic torticollis, preferably the former for reasons explained in the text.

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