Characteristics of the clinical course, pathogenesis and treatment of torsion dystonia in childhood
Markova, E.D.
Zhurnal Nevropatologii i Psikhiatrii Imeni S.S. Korsakova 89(8): 32-35
1989
ISSN/ISBN: 0044-4588 PMID: 2588895 Document Number: 340514
A total of 150 patients, predominantly children, with torsion dystonia++ (TD) were investigated. Their clinical syndromes, pathogenesis and results of treatment were analyzed. Prominent features were interfamilial (true heterogeneity) and intrafamilial clinical polymorphism of the disease. Autosome-dominant++ type of inheritance was found in 15 families and the autosome-recessive in 10. With respect to the clinical syndrome, 2 forms of TD were singled out: dystonic-hyperkinetic++ and rigid. These were different in the neurotransmitter metabolism. Long-term follow-up of the patients (2 to 20 years) proved a high efficiency of treatment with low doses of DOPA-containing drugs in rigid forms of TD.