The evaluation of hyperkineses in hepatocerebral dystrophy (Wilson-Konovalov disease) from the position of the theory of muscle spindles

• Published in: Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova Vol. 115; no. 4; p. 54
• Main Author: Starikov, A S
• Format: Journal Article
• Language: Russian
• Published: Russia (Federation) 2015
• Subjects: Electromyography; Female; Globus Pallidus - physiopathology; Hepatolenticular Degeneration - complications; Humans; Hyperkinesis - etiology; Hyperkinesis - physiopathology; Male; Motor Neurons, Gamma - physiology; Muscle Spindles - physiopathology; Tremor - physiopathology
• DOI: 10.17116/jnevro20151154154-60
• ISSN: 1997-7298

To obtain evidence for the possibility of considering hyperkineses in hepatocerebtal dystrophy from the position of the theory of muscle spindles. We examined 27 patients: rigid-arrhythmic-hyperkinetic form was diagnosed in 2 patients, trembling-rigid in 8, trembling in 16 and extrapyramidal-cortical in 1. Electromyography of different muscles in resting state and functional loadings taking into account surgical intervention was the main method of the study. An analysis of electrophysiological results based on hyperkinesis variant (torsion dystonic, choreoathetoid etc) revealed a role of the striatal pallidal system in the anomalous control of static and dynamic γ-motorneurons and involvement of spinal reflexes in forced movements. This hypothesis may help to deeply understand the genesis of extrapyramidal dyskinesia and more reasonably select a stereotaxic target in surgical treatment.