The evaluation of dystonia requires a reliable rating scale. The widely used Fahn-Marsden Scale (F-M) has not been sufficiently tested across multiple centers and investigators. The Dystonia Study Group developed the Unified Dystonia Rating Scale (UDRS) and a Global Dystonia Rating Scale (GDS) to serve as instruments to assess dystonia severity. In this study, 25 dystonia experts evaluated the ...

Genes causing primary dystonia are rare. Recently, pathogenic mutations in the anoctamin 3 gene (ANO3) have been identified to cause autosomal dominant craniocervical dystonia and have been assigned to the dystonia locus dystonia-24 (DYT24). Here, we expand on the phenotypic spectrum of DYT24 and provide demonstrative videos. Moreover, tremor recordings were performed, and back-averaged electro...

The neurobiological basis of psychogenic movement disorders remains poorly understood and the management of these conditions difficult. Functional neuroimaging studies have provided some insight into the pathophysiology of disorders implicating particularly the prefrontal cortex, but there are no studies on psychogenic dystonia, and comparisons with findings in organic counterparts are rare. To...

Dystonia is a movement disorder defined by sustained muscle contractions, causing twisting and repetitive movements and abnormal postures. To understand the abnormalities in pallidal discharge in dystonia, we have analyzed the spontaneous activity of 453 neurons sampled from the internal or external pallidum (GPi or GPe) of 22 patients with dystonia, 140 neurons from 11 patients with Parkinson'...

Dystonia is a functionally disabling movement disorder characterized by abnormal movements and postures. Although substantial recent progress has been made in identifying genetic factors, the pathophysiology of the disease remains a mystery. A provocative suggestion gaining broader acceptance is that some aspect of neural plasticity may be abnormal. There is also evidence that, at least in some...

We recently showed a beneficial effect of selective dorsal rhizotomy (SDR) on daily care and comfort in nonwalking children with severe bilateral spasticity. However, despite careful selection, some patients showed dystonia after the intervention, in which cases caregivers tended to be less satisfied with the result.The aim of this study is to identify risk factors for dystonia after SDR in chi...

BACKGROUND AND PURPOSE Traditionally dystonia has been considered a disorder of basal ganglia dysfunction. However, recent research has advocated a more complex neuroanatomical network. In particular, there is increasing interest in the pathophysiological role of the cerebellum. Patients with cervical and focal hand dystonia have impaired cerebellar associative learning using the paradigm eyebl...

OBJECTIVE To quantitatively assess control of balance under static and dynamic conditions in patients with tonic and phasic cervical dystonia. METHODS Ten patients with purely tonic cervical dystonia with fixed postural deviation and 20 patients with cervical dystonia with phasic head movements were investigated at least 3 months after botulinum toxin injections. Seventeen age matched volunte...

Complex regional pain syndrome (CRPS) is a syndrome usually localized in the extremities, mostly occurring after a preceding trauma or operation. Dystonia is present in a minority of CRPS patients, but, when present, leads to severe disability. Various pathological factors have been postulated to present in CRPS-dystonia, such as involvement of the sympathetic system, reorganization of the cent...

OBJECTIVES To analyse the lateralising value of unilateral manual automatism (UMA), its relation to contralateral dystonia and the hand by which the UMA was performed. METHODS In this retrospective study, we reviewed video recordings of 141 patients (mean age 34.1+/-10) who had consecutively undergone presurgical evaluations with ictal video-EEG recordings and high-resolution MRI, had had epi...