Background : Deep brain stimulation (DBS) of the globuspallidusinternus (GPi) is recommended as a promising technique for the management of the primary generalized dystonia (PGD) with DYT1 gene mutation. We present the first report of DBS results in Iranian patients with DYT1 positive PGD.   Methods : Nine patients who suffered from severely disabling DYT1 positive PGD consecutively were recr...

Some patients with dystonic movements and postures not known to be caused by environmental or degenerative disorders can be segregated from classical-appearing idiopathic torsion dystonia on the basis of distinctive clinical and pharmacologic features. Many of them should be considered within the family of dystonia, as clinical variants of idiopathic torsion dystonia, while others are better cl...

OBJECTIVE To investigate the effect of deep brain stimulation (DBS) on reducing dystonia and disability in adults with cerebral palsy (CP) and to compare the therapeutic outcomes between primary dystonia patients and CP patients over two years after bilateral pallidal DBS. METHODS Five patients with primary dystonia and seven CP patients with dystonia were recruited. All subjects received DBS...

Background: Dystonia is common in children with acquired and inherited neurological disorders. Status dystonicus (SD) the most severe form of dystonia that can lead to life-threatening complications if not treated promptly. We identified a local provider knowledge gap acute management dystonia, leading uncertainty delays care. To our knowledge, no in-hospital clinical pathway exists for ward-ba...

Why dystonia, generalized or focal, occurs is unknown. Increasing evidence suggests that generalized dystonia has a strong genetic component. Further, that in families with generalized dystonia, the rate of focal dystonia in other family members is increased.1 My own observations of the reverse; that is, families with focal dystonia having members with generalized dystonia have failed to yield ...

The relation between age at dystonia onset and sex was investigated in 264 patients with cranial-cervical dystonia and 56 patients with upper limb dystonia. In cranial-cervical dystonia, women had a significantly greater age at the onset of dystonia than men. The association was independent of duration of disease and distance of referral, but it was no longer detectable after adjustment for edu...

Dystonia have many subtypes, and is classified as focal, segmental and generalized. As for focal dystonia, spasmodic torticollis (cervical dystonia) and writer's cramp are most common. Cervical dystonia is mainly treated effectively with selective peripheral denervation, and task specific focal dystonia of the hand (writer's cramp) is effectively alleviated by stereotactic ventro-oral thalamoto...

Extrapyramidal disorders are a rare presentation of multiple sclerosis. One such is paroxysmal hemidystonia. Dystonia is, in the strict sense, result damage to various sites central nervous system. In one published cases series, lesions causing dystonia occurred heterogeneous locations, most commonly basal ganglia, followed by thalamus, brainstem, and white matter. series 62 patients with thala...

Oromandibular dystonia (OMD) refers to involuntary spasms of masticatory, lingual, and pharyngeal muscles. Phenomenologically, there are seven types of OMD: jaw-closing dystonia (JCD); jaw-opening dystonia (JOD); jaw-deviation dystonia (JDD); lip and perioral dystonia; lingual dystonia; pharyngeal dystonia; and combination OMD (1). OMD may be seen in isolation (focal dystonia), as part of a mor...

There is substantial variability in the responsiveness of dystonia patients to deep brain stimulation (DBS), presumably due to the multiple causes of dystonia. This article presents the results of an analysis of the combined published results of individual patient outcomes following DBS for all types of dystonia. From 157 papers reporting clinical outcomes of DBS for dystonia, individual quanti...