Biomaterial Database

[The evaluation of hyperkineses in hepatocerebral dystrophy (Wilson-Konovalov disease) from the position of the theory of muscle spindles]. 2015

A S Starikov

Esenin Ryazan State University, Ryazan.

OBJECTIVE To obtain evidence for the possibility of considering hyperkineses in hepatocerebtal dystrophy from the position of the theory of muscle spindles. METHODS 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. CONCLUSIONS 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.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D009047	Motor Neurons, Gamma	Motor neurons which activate the contractile regions of intrafusal SKELETAL MUSCLE FIBERS, thus adjusting the sensitivity of the MUSCLE SPINDLES to stretch. Gamma motor neurons may be "static" or "dynamic" according to which aspect of responsiveness (or which fiber types) they regulate. The alpha and gamma motor neurons are often activated together (alpha gamma coactivation) which allows the spindles to contribute to the control of movement trajectories despite changes in muscle length.	Neurons, Fusimotor,Neurons, Gamma Motor,Gamma Motorneurons,Motor Neurons, Gamma-Efferent,Fusimotor Neuron,Fusimotor Neurons,Gamma Motor Neuron,Gamma Motor Neurons,Gamma Motorneuron,Gamma-Efferent Motor Neuron,Gamma-Efferent Motor Neurons,Motor Neuron, Gamma,Motor Neuron, Gamma-Efferent,Motor Neurons, Gamma Efferent,Motorneuron, Gamma,Motorneurons, Gamma,Neuron, Fusimotor,Neuron, Gamma Motor,Neuron, Gamma-Efferent Motor,Neurons, Gamma-Efferent Motor
D009470	Muscle Spindles	Skeletal muscle structures that function as the MECHANORECEPTORS responsible for the stretch or myotactic reflex (REFLEX, STRETCH). They are composed of a bundle of encapsulated SKELETAL MUSCLE FIBERS, i.e., the intrafusal fibers (nuclear bag 1 fibers, nuclear bag 2 fibers, and nuclear chain fibers) innervated by SENSORY NEURONS.	Muscle Stretch Receptors,Neuromuscular Spindles,Receptors, Stretch, Muscle,Stretch Receptors, Muscle,Muscle Spindle,Muscle Stretch Receptor,Neuromuscular Spindle,Receptor, Muscle Stretch,Receptors, Muscle Stretch,Spindle, Muscle,Spindle, Neuromuscular,Spindles, Muscle,Spindles, Neuromuscular,Stretch Receptor, Muscle
D004576	Electromyography	Recording of the changes in electric potential of muscle by means of surface or needle electrodes.	Electromyogram,Surface Electromyography,Electromyograms,Electromyographies,Electromyographies, Surface,Electromyography, Surface,Surface Electromyographies
D005260	Female		Females
D005917	Globus Pallidus	The representation of the phylogenetically oldest part of the corpus striatum called the paleostriatum. It forms the smaller, more medial part of the lentiform nucleus.	Paleostriatum,Pallidum,Pallidums
D006527	Hepatolenticular Degeneration	A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.	Cerebral Pseudosclerosis,Neurohepatic Degeneration,Pseudosclerosis,Wilson Disease,Copper Storage Disease,Hepatic Form of Wilson Disease,Hepato-Neurologic Wilson Disease,Hepatocerebral Degeneration,Hepatolenticular Degeneration Syndrome,Kinnier-Wilson Disease,Progressive Lenticular Degeneration,Westphal-Strumpell Syndrome,Wilson Disease, Hepatic Form,Wilson's Disease,Cerebral Pseudoscleroses,Copper Storage Diseases,Degeneration Syndrome, Hepatolenticular,Degeneration Syndromes, Hepatolenticular,Degeneration, Hepatocerebral,Degeneration, Hepatolenticular,Degeneration, Neurohepatic,Degeneration, Progressive Lenticular,Degenerations, Hepatocerebral,Degenerations, Neurohepatic,Disease, Copper Storage,Diseases, Copper Storage,Diseases, Hepato-Neurologic Wilson,Diseases, Kinnier-Wilson,Hepato Neurologic Wilson Disease,Hepato-Neurologic Wilson Diseases,Hepatocerebral Degenerations,Hepatolenticular Degeneration Syndromes,Kinnier Wilson Disease,Kinnier-Wilson Diseases,Lenticular Degeneration, Progressive,Neurohepatic Degenerations,Pseudoscleroses, Cerebral,Pseudosclerosis, Cerebral,Storage Disease, Copper,Storage Diseases, Copper,Syndrome, Hepatolenticular Degeneration,Syndromes, Hepatolenticular Degeneration,Westphal Strumpell Syndrome,Westphal-Strumpell Syndromes,Wilson Disease, Hepato-Neurologic,Wilson Diseases, Hepato-Neurologic,Wilsons Disease
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006948	Hyperkinesis	Excessive movement of muscles of the body as a whole, which may be associated with organic or psychological disorders.	Hyperactivity, Motor,Hyperkinesia,Hyperkinesia, Generalized,Hyperkinetic Movements,Generalized Hyperkinesia,Generalized Hyperkinesias,Hyperkinesias, Generalized,Hyperkinetic Movement,Motor Hyperactivity,Movement, Hyperkinetic,Movements, Hyperkinetic
D014202	Tremor	Cyclical movement of a body part that can represent either a physiologic process or a manifestation of disease. Intention or action tremor, a common manifestation of CEREBELLAR DISEASES, is aggravated by movement. In contrast, resting tremor is maximal when there is no attempt at voluntary movement, and occurs as a relatively frequent manifestation of PARKINSON DISEASE.	Action Tremor,Intention Tremor,Resting Tremor,Coarse Tremor,Continuous Tremor,Darkness Tremor,Fine Tremor,Intermittent Tremor,Involuntary Quiver,Massive Tremor,Passive Tremor,Persistent Tremor,Pill Rolling Tremor,Rest Tremor,Saturnine Tremor,Senile Tremor,Static Tremor,Tremor, Limb,Tremor, Muscle,Tremor, Neonatal,Tremor, Nerve,Tremor, Perioral,Tremor, Semirhythmic,Action Tremors,Coarse Tremors,Continuous Tremors,Darkness Tremors,Fine Tremors,Intention Tremors,Intermittent Tremors,Involuntary Quivers,Limb Tremor,Limb Tremors,Massive Tremors,Muscle Tremor,Muscle Tremors,Neonatal Tremor,Neonatal Tremors,Nerve Tremor,Nerve Tremors,Passive Tremors,Perioral Tremor,Perioral Tremors,Persistent Tremors,Pill Rolling Tremors,Quiver, Involuntary,Rest Tremors,Resting Tremors,Saturnine Tremors,Semirhythmic Tremor,Semirhythmic Tremors,Senile Tremors,Static Tremors,Tremor, Action,Tremor, Coarse,Tremor, Continuous,Tremor, Darkness,Tremor, Fine,Tremor, Intention,Tremor, Intermittent,Tremor, Massive,Tremor, Passive,Tremor, Persistent,Tremor, Pill Rolling,Tremor, Rest,Tremor, Resting,Tremor, Saturnine,Tremor, Senile,Tremor, Static,Tremors