Brain, Vol 121, Issue 4 547-560, Copyright © 1998 by Oxford University Press
WT Dauer, RE Burke, P Greene and S Fahn
Idiopathic cervical dystonia (ICD) is the most common form of adult- onset
focal dystonia. Previously, disagreement existed about whether ICD was a
psychiatric illness, but the disorder is now viewed as a neurological
illness and large clinical series have clarified the clinical features of
the disease. At the time of diagnosis, extracervical dystonia is found in
approximately 20% of patients, and there may be a concomitant head or hand
tremor. Importantly, adult- onset ICD does not become generalized, although
there may be segmental spread and pain may increase independently of the
dystonia. While 10- 20% of patients may experience remission, nearly all
patients relapse within 5 years and are left with persistent disease. The
aetiology of ICD is unknown, but there has been much progress in clarifying
the genetic abnormality in families with inherited adult-onset cervical
dystonia; linkage to chromosome 18p has been demonstrated in one family,
and the DYT1 locus has been excluded in two other families. Painful trauma
may be involved in the pathogenesis of ICD. Painful stimuli are received
and processed by the basal ganglia, and the synaptic changes provoked by
pain may lead to the abnormal physiology underlying dystonia. Consistent
with this idea are experiments which demonstrate that altered sensory input
leads to plasticity of the motor cortex, and those that explore the 'tonic
vibration reflex' in patients with dystonia. Another theory suggests that a
primary vestibular abnormality is responsible for ICD. Botulinum toxin is
the most effective treatment for ICD. Roughly 75% of patients improve, and
a response is generally seen within the first week. However, many questions
remain regarding the optimal technique of administration. The development
of neutralizing antibodies occurs in at least 5-10% of patients, and
appears to be related both to dosage and to the interval between
treatments. Side-effects are generally mild and result from the action of
the toxin in the periphery. If the response to botulinum toxin is not
adequate, anticholinergics, benzodiazepines, baclofen and other medications
are used as adjunctive therapy. Surgical therapies are available for the
treatment of ICD but are reserved for patients refractory to conservative
measures.
REVIEWS
Current concepts on the clinical features, aetiology and management of idiopathic cervical dystonia
Center for Parkinson's Disease and Other Movement Disorders, Neurological Institute of New York, Columbia Presbyterian Medical Center, USA.
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