Parkinson's disease and other movement disorders 2000. Pavia, Italy, May 28-30, 2000. Abstracts.
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The term dystonia was introduced by Oppenheim and Vogt in 1911 to describe the relatively slow, sustained, frequently forceful contorting movements involving striatal muscles. Dystonia is characteristically seen in childhood ("primary dystonia"), but also occurs in a variety of other disorders of the CNS ("secondary dystonia"). In the case of childhood dystonia (dystonia musculorum deformans) symptoms usually occur in young patients in which case the illness is usually inherited as either autosomal dominant or recessive. In cases of adult onset dystonia, genetic factors are less likely (Eldrige, 1970). Although dystonic movement disorders have been presumed to originate from dysfunction of the basal ganglia, examination of the brains of patients who have died with idiopathic or hereditary dystonia musculorum deformans have revealed no consistent neuropathological or neurochemical abnormalities (Zeman, 1970). Furthermore, therapy of value in the treatment of other basal ganglia disorders, in these conditions, has not been helpful (Fahn, 1982). The following paper will discuss evidence implicating deranged hypothalamic neuropeptidergic functions in dystonia. We have developed a hypothesis, which reviews and incorporates published data, in which we discuss the role of deranged hypothalamic neuropeptidergic function in the pathnophysiology of idiopathic dystonia.
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On the basis of the examination covering 30 young children (aged 2 months to 3.5 years), three variants of motor psychogenic disorders were specified. Group I included elementary, stereotypic motor phenomena expressed as special poses, rocking, jack-knifing, finger sucking and other stereotypes of motor acts. Group II was characterized by more complicated motor and psychomotor disorders in the form of various manifestations of motor infantilism. Group III comprised children with neurotic motor monosymptomatic and ritual-behavioural obsessive actions. The above disorders are assessed from the evolutional-dynamic point of view.
Fifty-two patients with reactive states were studied for specific features of their motor disorders. The clinical follow-up data were supplemented by electromyographic examinations. The motor pathology was shown to be characterized by polymorphism and marked atypical nature. It was revealed that under conditions of considerable functional disintegration of the cerebral motor systems, the motor pathology observed was similar to that seen with organic brain diseases.
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