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Craniocervical dystonia: clinical and pathophysiological features


A. Berardelli, Department of Neurological Sciences and Neuromed Institute, Sapienza University of Rome, Viale dell’Università 30, 00185 Rome, Italy (tel.: +39 0649914700; fax: +39 0649914700; e-mail:


Blepharospasm, oromandibular, lingual, laryngeal and cervical dystonia are common forms of adult-onset dystonia. Each condition may appear in isolation or manifest along with other forms of craniocervical dystonia. Although the various craniocervical dystonias typically present with involuntary muscle spasms causing abnormal postures, they differ for some clinical features. Neurophysiologic and neuroimaging studies have shown a number of motor and sensory abnormalities at cortical and subcortical levels, probably reflecting a dysfunction in the basal ganglia–thalamo-cortical circuits. The best treatment for craniocervical dystonia is botulinum toxin injected into the overactive muscles.