Dystonia Idiopathic Torsion DiseaseWe report two siblings who are suffering from a dystonic syndrome, clinically indistinguishable from idiopathic torsion dystonia (dystonia musculorum deformans) but with cranial computerized tomographic scan findings of basal ganglia lesions, similar to that reported in Wilson's disease An estimate of the mean degree of generalization of the disease at different times from onset was calculated and a curve of the estimated temporal evolution was drawn DMD and Oppenheim disease are terms now used for childhood- and adolescent-onset dystonia due to the DYT1 gene. With the recent mapping of genes for idiopathic torsion dystonia and identification of a gene for early onset dystonia, the description primary, or idiopathic, dystonia has evolved; it now may be viewed as secondary to or symptomatic of an identified cause. Symptoms typically start in one part of the body, usually in an arm or leg, and eventually spread to the rest of the body within about 5 years. The rate of evolution was not influenced by gender or familiarity Although secondary forms of dystonia are frequently associated with structural lesions of the basal ganglia and thalamus, no consistent histologic or biochemical findings are noted in primary torsion dystonia Besides motor control difficulties, defective sensory processing is described . Current models of basal ganglia circuitry have been adapted and suggesting dysfunction at the basal ganglia level The present statistical method of estimating the evolution in different patient groups should be useful especially for the evaluation of therapeutic interventions. Idiopathic torsion dystonia (ITD) is characterized by involuntary, repetitive, sustained muscle contractions or postures involving one or more sites of the body Initially believed to be a manifestation of hysteria, idiopathic torsion dystonia gradually became established as a neurologic entity with a genetic basis. A form of dystonia known as early-onset torsion dystonia (also called generalized torsion dystonia) begins in childhood around the age of 12. In order to take advantage of all data regarding the evolution of the disease, independently of the length of follow-up, we have used a life table method of analysis. The course of the disease seemed more deleterious in non-Ashkenazic Jews. Abnormal brain networks have been described in different functional imaging studies; this is substantial evidence implicating dysfunction in dopaminergic pathways in the pathophysiology of primary torsion dystonia (Eidelberg, 1998; Perlmutter, 2004). The prevalence in the Ashkenazi Jewish population is approximately five to ten times higher, due to a founder mutation. Treatment options include botulinum toxin injections for focal symptoms, pharmacological therapy such as anticholinergics (most commonly trihexyphenydil) for generalized dystonia and surgical approaches such as deep brain stimulation of the internal globus pallidus in severe cases. Causes of Dystonia Idiopathic Torsion DiseaseSome common causes of Dystonia Idiopathic Torsion Disease :
Symptoms of Dystonia Idiopathic Torsion DiseaseSome common symptoms of Dystonia Idiopathic Torsion Disease :
Treatment of Dystonia Idiopathic Torsion Disease
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