Biomaterial Database

Natural course of idiopathic torsion dystonia among Jews. 1994

N Zilber, and R Inzelberg, and E Kahana, and A D Korczyn

Centre de Recherche Français de Jérusalem, UMR CNRS-DGRCST, Israel.

The natural history of 51 idiopathic torsion dystonia cases identified in a nationwide survey in Israel was studied. 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. 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. This analysis confirmed the heterogeneity of the disease course. The most rapid deterioration occurred in patients with juvenile onset in the lower limbs, particularly in the first 2 years following the onset. The rate of evolution was not influenced by gender or familiarity. The course of the disease seemed more deleterious in non-Ashkenazic Jews. The present statistical method of estimating the evolution in different patient groups should be useful especially for the evaluation of therapeutic interventions.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007557	Israel	A country in the Middle East, bordering the Mediterranean Sea, between Egypt and Lebanon. The capital is Jerusalem.
D007585	Jews	An ethnic group with historical ties to the land of ISRAEL and the religion of JUDAISM.	Jew
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D009460	Neurologic Examination	Assessment of sensory and motor responses and reflexes that is used to determine impairment of the nervous system.	Examination, Neurologic,Neurological Examination,Examination, Neurological,Examinations, Neurologic,Examinations, Neurological,Neurologic Examinations,Neurological Examinations
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D003430	Cross-Sectional Studies	Studies in which the presence or absence of disease or other health-related variables are determined in each member of the study population or in a representative sample at one particular time. This contrasts with LONGITUDINAL STUDIES which are followed over a period of time.	Disease Frequency Surveys,Prevalence Studies,Analysis, Cross-Sectional,Cross Sectional Analysis,Cross-Sectional Survey,Surveys, Disease Frequency,Analyses, Cross Sectional,Analyses, Cross-Sectional,Analysis, Cross Sectional,Cross Sectional Analyses,Cross Sectional Studies,Cross Sectional Survey,Cross-Sectional Analyses,Cross-Sectional Analysis,Cross-Sectional Study,Cross-Sectional Surveys,Disease Frequency Survey,Prevalence Study,Studies, Cross-Sectional,Studies, Prevalence,Study, Cross-Sectional,Study, Prevalence,Survey, Cross-Sectional,Survey, Disease Frequency,Surveys, Cross-Sectional
D004422	Dystonia Musculorum Deformans	A condition characterized by focal DYSTONIA that progresses to involuntary spasmodic contractions of the muscles of the legs, trunk, arms, and face. The hands are often spared, however, sustained axial and limb contractions may lead to a state where the body is grossly contorted. Onset is usually in the first or second decade. Familial patterns of inheritance, primarily autosomal dominant with incomplete penetrance, have been identified. (Adams et al., Principles of Neurology, 6th ed, p1078)	Childhood Torsion Disease,Idiopathic Torsion Dystonia,Oppenheim-Ziehen Disease,Progressive Torsion Spasm,Torsion Dystonia,Dystonia Deformans Musculorum,Dystonia Deformans Progressiva,Torsion Disease of Childhood,Dystonia, Idiopathic Torsion,Dystonias, Idiopathic Torsion,Dystonias, Torsion,Idiopathic Torsion Dystonias,Oppenheim Ziehen Disease,Spasm, Progressive Torsion,Torsion Disease, Childhood,Torsion Dystonia, Idiopathic,Torsion Spasm, Progressive