Predictive Factors of Outcome in Primary Cervical Dystonia Following Pallidal Deep Brain Stimulation

Surgical Movement Disorders, Department of Neurology, University of California, San Francisco, California, USA.
Movement Disorders (Impact Factor: 5.68). 09/2013; 28(10). DOI: 10.1002/mds.25560
Source: PubMed


Improvement after bilateral globus pallidus internus deep brain stimulation (DBS) in primary generalized dystonia has been negatively associated with disease duration and age, but no predictive factors have been identified in primary cervical dystonia (CD).
Patients treated with bilateral globus pallidus internus DBS for primary CD from 2 DBS centers with preoperative and postoperative Toronto Western Spasmodic Torticollis Rating Scales (TWSTRS) were studied retrospectively to explore possible predictors of response.
Patients showed significantly improved TWSTRS total and severity scores (n = 28, mean 55.6% and 50.8%, respectively, both P < .001). Patients with lateral shift at baseline had less improvement in TWSTRS severity subscores (P = .02). No correlations between outcomes and disease duration, age at dystonia onset or surgery, baseline scores, or other included variables were found.
Although this is the largest study supporting efficacy of bilateral pallidal DBS in primary CD, no major clinical predictive outcomes of surgical benefit were identified. © 2013 Movement Disorder Society.

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Available from: Leslie C Markun, Sep 19, 2014
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    • "Physiotherapy based treatments aimed at retraining posture or stretching dystonic muscles are also largely ineffective [5] [6]. More recently, deep brain stimulation targeting the globus pallidus of the basal ganglia has shown promising results [7] [8] [9] [10]. Efforts to discover effective non-invasive treatments have led researchers to non-invasive brain stimulation (NBS). "
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    ABSTRACT: Primary isolated dystonia is a hyperkinetic movement disorder whereby involuntary muscle contractions cause twisted and abnormal postures. Dystonia of the cervical spine and upper limb may present as sustained muscle contractions or task-specific activity when using the hand or upper limb. There is little understanding of the pathophysiology underlying dystonia and this presents a challenge for clinicians and researchers alike. Emerging evidence that the cerebellum is involved in the pathophysiology of dystonia using network models presents the intriguing concept that the cerebellum could provide a novel target for non-invasive brain stimulation. Non-invasive stimulation to increase cerebellar excitability improved aspects of handwriting and circle drawing in a small cohort of people with focal hand and cervical dystonia. Mechanisms underlying the improvement in function are unknown, but putative pathways may involve the red nucleus and/or the cervical propriospinal system. Furthermore, recent understanding that the cerebellum has both motor and cognitive functions suggests that non-invasive cerebellar stimulation may improve both motor and non-motor aspects of dystonia. We propose a combination of motor and non-motor tasks that challenge cerebellar function may be combined with cerebellar non-invasive brain stimulation in the treatment of focal dystonia. Better understanding of how the cerebellum contributes to dystonia may be gained by using network models such as our putative circuits involving red nucleus and/or the cervical propriospinal system. Finally, novel treatment interventions encompassing both motor and non-motor functions of the cerebellum may prove effective for neurological disorders that exhibit cerebellar dysfunction.
    12/2013; DOI:10.2478/s13380-013-0143-0
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    ABSTRACT: Isolated focal dystonia is a neurological disorder that manifests as repetitive involuntary spasms and/or aberrant postures of the affected body part. Craniocervical dystonia involves muscles of the eye, jaw, larynx, or neck. The pathophysiology is unclear, and effective therapies are limited. One mechanism for increased muscle activity in craniocervical dystonia is loss of inhibition involving the trigeminal sensory nuclear complex (TSNC). The TSNC is tightly integrated into functionally connected regions subserving sensorimotor control of the neck and face. It mediates both excitatory and inhibitory reflexes of the jaw, face, and neck. These reflexes are often aberrant in craniocervical dystonia, leading to our hypothesis that the TSNC may play a central role in these particular focal dystonias. In this review, we present a hypothetical extended brain network model that includes the TSNC in describing the pathophysiology of craniocervical dystonia. Our model suggests the TSNC may become hyperexcitable due to loss of tonic inhibition by functionally connected motor nuclei such as the motor cortex, basal ganglia, and cerebellum. Disordered sensory input from trigeminal nerve afferents, such as aberrant feedback from dystonic muscles, may continue to potentiate brainstem circuits subserving craniocervical muscle control. We suggest that potentiation of the TSNC may also contribute to disordered sensorimotor control of face and neck muscles via ascending and cortical descending projections. Better understanding of the role of the TSNC within the extended neural network contributing to the pathophysiology of craniocervical dystonia may facilitate the development of new therapies such as noninvasive brain stimulation.
    The Journal of Neuroscience : The Official Journal of the Society for Neuroscience 11/2013; 33(47):18358-18367. DOI:10.1523/JNEUROSCI.3544-13.2013 · 6.34 Impact Factor
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    ABSTRACT: Treatment of dystonia refractory to oral medications or botulinum toxin injections includes the use of deep brain stimulation (DBS). Expectations should be established based on patient-related factors, including type of dystonia, genetic cause, target symptoms, age at the time of surgery, disease duration, or the presence of fixed skeletal deformities. Premorbid conditions such as psychiatric illness and cognitive impairment should be considered. Target selection is an emerging issue in DBS for dystonia. Although efficacy has been established for targeting the globus pallidus internus for dystonia, other brain targets such as the subthalamic nucleus, thalamus, or cortex may be promising alternatives.
    Neurosurgery clinics of North America 01/2014; 25(1):59-75. DOI:10.1016/ · 1.44 Impact Factor
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