Botulinum Toxin Seroype A for Cervical Dystonia—An Assessment

Botulinum Toxin Seroype A for Cervical Dystonia—An Assessment

Brashear Allison
Published: US Neurology - Volume 4 Issue 2
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Diagnosis of Cervical Dystonia
Dystonia involves a lack of synergy of antagonist and agonist muscles, resulting in irregular and unco-ordinated movements.1 Clinically, focal dystonia occurs when the involuntary movements are isolated to one body part such as a limb (writer’s cramp), the larynx (spasmodic dysyphonia), or the eyes (blepharospasm). Cervical dystonia (CD), a form of focal dystonia involving the neck muscles, presents with abnormal postures of the neck. While typical CD presents with tilt (laterocollis), turn (torticollis), and/or pulling forward (anterocollis) or backward (retrocollis), any combination of these may be seen. In addition, some patients present with superimposed tremor and/or pain. While the involuntary movement is always part of CD presentation, in some patients pain can be the prominent complaint.2

The incidence of CD is reported to be 1/250,000 people,3 but the numbers are often thought to be higher due to delays in referral and an inability to accurately diagnosis CD. In a European study, the prevalence of primary dystonia in patients over 50 years of age was 732 per 100,000,4 suggesting that the true prevalence of primary dystonia may be significantly higher than the published rates. A 2008 study in an ethnically diverse population identified 66 incident cases of CD from 8.2 million person-years of observation. The minimum estimation of the incidence of CD in this population is 0.80 per 100,000 person-years. The incidence was higher in Caucasian individuals and 2.5 times higher in women than in men.5

The cellular pathology of CD is unknown, but functional magnetic resonance imaging (fMRI) of patients with CD demonstrated increased activation in the caudate nucleus, putamen, and thalamus.6 Recent work in non-human primate studies revealed that lesions in brain stem areas involved in the control of muscular tone with gamma-aminobutyric acid (GABA)-ergic manipulations in various basal ganglia nuclei or thalamus also lead to dystonia, suggesting that the illness is associated with aberrant sensory representations interfering with motor control.7 Patients with dystonia may often manifest a sensory trick, such as a touch or movement that temporarily alleviates the CD symptoms. These clinical findings support the fMRI discoveries suggesting the interaction of sensory and motor pathways in the pathophysiology of dystonia.

The diagnosis of CD is based solely on clinical signs and symptoms. While there is one commercial genetic test for dystonia (DYT-1), for almost all cases of isolated CD there is no diagnostic test.8 A recent screening tool was developed as part of a larger study of familial dystonia: the Beth Israel Dystonia Screen (BIDS). A computer-assisted telephone interview was tested on 193 individuals from 16 families with CD and cranial dystonia and correlated with a videotape assessment as the gold standard. The BIDS correctly identified patients with CD as having a positive response to at least two of five screening questions and a sensitivity for CD of 100% and a specificity of 92%.9 However, despite the success of the BIDS there remains no diagnostic imaging or blood test to confirm the clinical suspicion. The diagnosis of CD remains clinical and may often result in patients making recurrent trips to specialists for confirmation. In a recent large study the duration of the period between showing symptoms and diagnosis was 3.9 and 5.3 years for women and men, respectively.5

In most cases CD is idiopathic, but in rare patients CD may be present in cases with a genetic, traumatic, or iatrogenic cause. Idiopathic CD typically peaks in later years, but an early age at onset is not uncommon. Patients may report subtle symptoms over years and family members may report a gradual increase in symptoms over time. In older literature, a remission rate of 10–120% was reported, but persistent remission in patients has not been reported since the advent of botulinum toxin (BoNT) treatment. The ability of patients to transiently moderate their symptoms with sensory tricks such as using a tooth pick or closing one eye suggests a diagnosis of CD.

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