Evaluation of a programming algorithm for deep brain stimulation in dystonia used in a double-blind, sham-controlled multicenter study.

Background: Programming deep brain stimulation in dystonia is difficult because of the delayed benefits and absence of evidence-based guidelines. Therefore, we evaluated the efficacy of a programming algorithm applied in a double-blind, sham-controlled multicenter study of pallidal deep brain stimulation in dystonia.

Methods: A standardized monopolar review to identify the contact with the best acute antidystonic effect was applied in 40 patients, who were then programmed 0.

Increased basal-ganglia activation performing a non-dystonia-related task in focal dystonia.

Background And Purpose: We tried to determine whether altered sensorimotor cortex and basal-ganglia activation in blepharospasm (BSP) and cervical dystonia (CD) are restricted to areas directly responsible for the innervation of dystonic muscles, or whether impairment in focal dystonia reaches beyond these direct associations supporting a more global disturbance of sensory and motor control in focal dystonia.

Methods: Twenty patients with focal dystonia (11 BSP, 9 CD) and 14 healthy controls were investigated with functional magnetic resonance imaging (fMRI) performing a simple grip force forearm contraction task.

Results: BSP and CD patients and healthy controls showed similar activation in the pre-motor, primary motor and primary sensory cortex, whilst basal-ganglia activation was increased in BSP and CD with related activation patterns compared with controls.

Pallidal deep brain stimulation improves quality of life in segmental and generalized dystonia: results from a prospective, randomized sham-controlled trial.

As part of the first randomized, sham-stimulation controlled trial on deep brain stimulation (DBS) in primary segmental or generalized dystonia, health-related quality of life (HRQoL) was assessed by SF-36. After the 3-month sham-controlled phase, significant HRQoL improvement occurred only in the active-stimulation group. The open-label extension phase resulted in a significant improvement in all SF-36 domains following 6 months of neurostimulation.

Pallidal deep-brain stimulation in primary generalized or segmental dystonia.

Background: Neurostimulation of the internal globus pallidus has been shown to be effective in reducing symptoms of primary dystonia. We compared this surgical treatment with sham stimulation in a randomized, controlled clinical trial.

Methods: Forty patients with primary segmental or generalized dystonia received an implanted device for deep-brain stimulation and were randomly assigned to receive either neurostimulation or sham stimulation for 3 months.

Mutation at the SCA17 locus is not a common cause of primary dystonia.

Spinocerebellar ataxia type 17 (SCA17) is a dominant progressive neurodegenerative disorder, caused by a triplet repeat expansion within the TATA-binding protein. As well as ataxia and dementia, Parkinsonism and dystonia are common in SCA17. In some pedigrees focal dystonia in the absence of ataxia has been described as a main clinical feature.

Lack of mutations in the epsilon-sarcoglycan gene in patients with different subtypes of primary dystonias.

Primary dystonias represent a clinically and genetically heterogeneous group of movement disorders. Mutations in the epsilon-sarcoglycan (SGCE) gene have been found recently to cause myoclonus-dystonia (MD). Considerable clinical variation of SGCE mutation carriers leads to the hypothesis that mutations in the SGCE gene might also be relevant for other subtypes of dystonias.

Botulinum toxin A in the prophylactic treatment of migraine--a randomized, double-blind, placebo-controlled study.

Botulinum toxin A has been suggested to be effective in the prophylactic treatment of migraine. However, only very few randomized, double-blind, placebo-controlled studies are available. We designed such a study with a specific focus on different injection sites.

Fracture of the odontoid process complicating tardive dystonia.

We report on a 57-year-old woman with frequent and powerful retrocollis. The involuntary movements combined with rheumatoid arthritis and osteoporosis led to a fracture of the axial odontoid process. Neurologists with a focus on movement disorders should screen for diseases putting the patient at risk for spinal lesions and consider early treatment.

Orthopedic and neurological complications of cervical dystonia--review of the literature.

Cervical dystonia is the most frequent form of focal dystonia. Further, cervical dystonia can occur as a feature of segmental or generalized dystonias and cerebral palsy. Treatment with botulinum toxin to relieve pain and improve functional and psychological outcome is effective, but expensive.

Frequency and phenotypic variability of the GAG deletion of the DYT1 gene in an unselected group of patients with dystonia.

Background: Dystonia is a clinically and genetically heterogeneous movement disorder characterized by sustained muscle contractions affecting one or more sites of the body, frequently causing twisting and repetitive movements or abnormal postures. A 3-base pair (GAG) deletion in the DYT1 gene is held responsible for most cases of early-onset primary generalized dystonia in the Ashkenazi Jewish population as well as in non-Jewish patients.

Objectives: To investigate the prevalence of the GAG deletion in the DYT1 gene and the phenotypic variability in the general population by testing patients with different subtypes of dystonia from 4 different movement disorder outpatient clinics in Germany.

Treatment of headache with botulinum toxin A--a review according to evidence-based medicine criteria.

The aim of this review is to evaluate the studies available from reference systems and published congress contributions on the prophylactic treatment of idiopathic and cervicogenic headache with botulinum toxin A, and to classify these studies according to evidence-based medicine (EBM) criteria. The studies were analysed with respect to the study design, the number of patients enrolled, the efficacy parameters, and the significance of results. We used the following classification of EBM.

Hemispheric dominance in the processing of J.S. Bach fugues: a transcranial Doppler sonography (TCD) study with musicians.

Although the majority of studies on musical processing in musicians observed a left hemisphere dominance which has usually been explained by a proficient analytical strategy used by these subjects, the findings are still inconsistent. Changes in hemispheric activity induced by listening to music (J. S.

Cerebral hemodynamics during electrically induced seizures.

Electroconvulsive therapy (ECT) is an appropriate clinical model to investigate blood flow during seizures. In this study cerebral blood flow velocity (CBFV) was measured during 40 ECTs in 10 patients by means of transcranial Doppler sonography. EEG was recorded continuously.

Localized proton NMR spectroscopy in the striatum of patients with idiopathic spasmodic torticollis.

Single voxel proton spectroscopy was used to study brain metabolism in idiopathic spasmodic torticollis. Peak metabolite ratios in long echo time (135 ms) spectra were evaluated in the basal ganglia of 16 patients (29-65 years). Eight of them were untreated; the other eight were examined 4-6 weeks after administration of botulinum toxin type A.