- Events | Dystonia Medical Research Foundation - Part 3
- Animal models of focal dystonia
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Defective temporal processing of sensory stimuli in DYT1 mutation carriers: a new endophenotype of dystonia? Brain ; Pt 1 : — Sohn YH , Hallett M. Disturbed surround inhibition in focal hand dystonia. Ann Neurol ; 56 : — 9. Abnormal plasticity of sensorimotor circuits extends beyond the affected body part in focal dystonia. J Neurol Neurosurg Psychiatry ; 79 : — Mol Pathol ; 54 : — 8. Functional anatomy of the basal ganglia in X-linked recessive dystonia-parkinsonism.
Ann Neurol ; 58 : 7 — Levodopa-induced dyskinesias and dopamine-dependent stereotypies: a new hypothesis.
Events | Dystonia Medical Research Foundation - Part 3
Trends Neurosci ; 23 10 Suppl : S71 — 7. Functional architecture of basal ganglia circuits: neural substrates of parallel processing. Trends Neurosci ; 13 : — Basal Ganglia disorders associated with imbalances in the striatal striosome and matrix compartments. Front Neuroanat ; 5 : Striatal cholinergic interneurons modulate spike-timing in striosomes and matrix by an amphetamine-sensitive mechanism.
Front Neuroanat ; 11 : Striosome-dendron bouquets highlight a unique striatonigral circuit targeting dopamine-containing neurons. Single nigrostriatal dopaminergic neurons form widely spread and highly dense axonal arborizations in the neostriatum. J Neurosci ; 29 : — Familial dopa-responsive cervical dystonia. Neurology ; 66 : — OpenUrl CrossRef.
Differential involvement of striosome and matrix dopamine systems in a transgenic model of dopa-responsive dystonia. Dopa-responsive dystonia is caused by particular impairment of nigrostriatal dopamine neurons different from those involved in Parkinson disease: evidence observed in studies on Segawa disease. Neuropediatrics ; 44 : 61 — 6. Parkinsonism Relat Disord ; 21 : — Predominant dystonia with marked cerebellar atrophy: a rare phenotype in familial dystonia.
Neurology ; 67 : — Autopsy case of severe generalized dystonia and static ataxia with marked cerebellar atrophy. Neurology ; 85 : — 4. The DYT1 carrier state increases energy demand in the olivocerebellar network. Neuroscience ; : — The neural substrates of rapid-onset Dystonia-Parkinsonism.
Nat Neurosci ; 14 : — Cerebellectomy eliminates the motor syndrome of the genetically dystonic rat. Exp Neurol ; : — Current opinions and areas of consensus on the role of the cerebellum in dystonia. Cerebellum ; 16 : — Unilateral lesions of the globus pallidus: report of four patients presenting with focal or segmental dystonia.
J Neurol Neurosurg Psychiatry ; 69 : — 8. Dystonia after striatopallidal and thalamic stroke: clinicoradiological correlations and pathophysiological mechanisms. J Neurol Neurosurg Psychiatry ; 65 : — 8. Thalamic vo-complex vs pallidal deep brain stimulation for focal hand dystonia. Neurology ; 70 16 Pt 2 : — 1. Carbon M , Eidelberg D.
Animal models of focal dystonia
Abnormal structure-function relationships in hereditary dystonia. Neuroscience ; : — 9. Thalamocortical connectivity correlates with phenotypic variability in dystonia. Cereb Cortex ; 25 : — The cerebellum communicates with the basal ganglia. Nat Neurosci ; 8 : — 3. Striatal dopamine release is triggered by synchronized activity in cholinergic interneurons.
Neuron ; 75 : 58 — Short latency cerebellar modulation of the basal ganglia. Nat Neurosci ; 17 : — Impairment of bidirectional synaptic plasticity in the striatum of a mouse model of DYT1 dystonia: role of endogenous acetylcholine. Brain ; Pt 9 : — Anticholinergic drugs rescue synaptic plasticity in DYT1 dystonia: role of M1 muscarinic receptors.
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Mov Disord ; 29 : — Contribution of tonic vibration reflex to the evaluation and diagnosis of cerebellar disorders. J Neurol Neurosurg Psychiatry ; 45 : — Hemi-dystonia secondary to localised basal ganglia tumour. Individuals with all forms of dystonia are welcome to attend.
Individuals with al forms of dystonia, as well as family and friends, are welcome to attend. Guest Speaker: Dr.
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Ron Pelton OculoPlastic Surgeon[…]. Join us to learn about dystonia and meet with others in the community. Individuals with all forms of dystonia, as well as family and friends, are welcome to attend.
Individuals with all forms 0f dystonia, as well as family and friends, are welcome to attend. Individuals with all forms of dystonia, as well as family and friends are welcome to attend. Friday, October 11, pm —[…]. Cervical dystonia is characterized by involuntary muscle contractions of the neck and abnormal head positions that affect daily life activities and social life of patients.
Patients are usually treated with botulinum toxin injections into affected neck muscles to relief pain and improve control of head postures. In addition, many patients are referred for physical therapy to improve their ability to perform activities of daily living. A recent review on allied health interventions in cervical dystonia showed a lack of randomized controlled intervention studies regarding the effectiveness of physical therapy interventions. The cost- effectiveness of a standardized physical therapy program compared to regular physical therapy, both as add-on treatment to botulinum toxin injections will be determined in a multi-centre, single blinded randomized controlled trial with cervical dystonia patients.
Primary outcomes are disability in daily functioning assessed with the disability subscale of the Toronto Western Spasmodic Torticollis Rating Scale. Secondary outcomes are pain, severity of dystonia, active range of motion of the head, quality of life, anxiety and depression. Data will be collected at baseline, after six months and one year by an independent blind assessor just prior to botulinum toxin injections. For the cost effectiveness, an additional economic evaluation will be performed with the costs per quality adjusted life-year as primary outcome parameter.
Our study will provide new evidence regarding the cost- effectiveness of a standardized, tailored physical therapy program for patients with cervical dystonia. It is widely felt that allied health interventions, including physical therapy, may offer a valuable supplement to the current therapeutic options. A positive outcome will lead to a greater use of the standardized physical therapy program. For the Dutch situation a positive outcome implies that the standardized physical therapy program forms the basis for a national treatment guideline for cervical dystonia.
Cervical Dystonia CD , or torticollis, is a disabling neurological disorder characterized by abnormal positions of the head due to involuntary muscle contractions of the neck [ 1 ]. The posture in CD patients can feature one or a combination of postures: rotation torticollis ; lateral tilting laterocollis ; flexion anterocollis ; extension retrocollis ; and lateral shift. With an estimated prevalence of 5. Pain is experienced in two-thirds to three-quarters of patients and is a major source of disability, which is strongly associated with the presence of muscle contractions and head deviations [ 3 — 6 ].
Decreased self-efficacy, fatigue, anxiety and depression are other factors associated with disability in cervical dystonia [ 7 ]. Treatment options for CD are mainly symptomatic, aiming to reduce involuntary movements, correct abnormal head positions and reduce pain. Currently, the best evidence based treatment option is to inject the dystonic neck muscles with botulinum toxin BTX [ 9 — 12 ]. The effects of BTX fluctuate over time. A peak effect occurs within 2—4 weeks after injections and is followed by a decrease of effect and return of symptoms.
Effect of BTX. Increasing lines indicate a better effect of BTX and less severity of CD, pain and disability to perform daily life tasks. Red lines illustrate the normal effect of BTX, blue dotted lines illustrate the expected additional effect of the PT program. Besides, the evidence for the effects of PT on the ability to perform activities of daily living in CD is very limited.