Hyperkinesis is excessive violent motor acts that occur against the will of the patient. They include a variety of clinical forms: tics, myoclonia, chorea, ballism, tremor, torsion dystonia, facial para- and hemispasm, akathisia, athetosis. Clinically diagnosed, additionally prescribed EEG, ENMG, MRI, CT, duplex scanning, cerebral vascular ultrasound, blood test. Conservative therapy requires an individual selection of a pharmaceutical from the following groups: cholinolytics, neuroleptics, valproates, benzodiazepines, DOPA drugs. In resistant cases, stereotactic destruction of extrapyramidal subcortical centers is possible.
G25 Other extrapyramidal and motor disorders
Translated from Greek, “hyperkinesis” means “overdrive”, which accurately reflects the excessive nature of pathological motor activity. Hyperkinesis has been known for a long time, described in the Middle Ages, often referred to in literary sources as the “dance of St. Vitus”. Since it was not possible to identify morphological changes in brain tissues, until the middle of the twentieth century hyperkinesis was considered a manifestation of neurotic syndrome. The development of neurochemistry allowed us to assume the connection of pathology with the imbalance of neurotransmitters, to take the first steps in studying the mechanism of motor disorders. Hyperkinesis can appear at any age, is equally common among the male and female population, and is an integral part of many neurological diseases.
Hyperkinetic syndrome occurs due to genetic disorders, organic brain damage, intoxication, infections, injuries, degenerative processes, drug therapy by individual groups of pharmaceuticals. In accordance with the etiology in clinical neurology , the following hyperkineses are distinguished:
- Primary – are a consequence of idiopathic degenerative processes in the central nervous system, have a hereditary character. Hyperkinesis is distinguished, developing as a result of selective damage to subcortical structures (essential tremor), and hyperkinesis in multisystem lesions: Wilson’s disease, olivopontocerebellar degenerations.
- Secondary – manifest in the structure of the underlying pathology caused by traumatic brain injury, brain tumor, toxic lesion (alcoholism, thyrotoxicosis, CO2 poisoning), infection (encephalitis, rheumatism), cerebral hemodynamic disorder (dyscirculatory encephalopathy, ischemic stroke). They may be a side effect of therapy with psychostimulants, carbamazepine, neuroleptics and MAO inhibitors, overdose of dopaminergic drugs.
- Psychogenic – associated with a chronic or acute traumatic situation, mental disorders (hysterical neurosis, manic-depressive psychosis, generalized anxiety disorder). They belong to rare forms.
Hyperkinesis is a consequence of the dysfunction of the extrapyramidal system, the subcortical centers of which are the striatum, caudate, red and lentil-shaped nuclei. The integration structures of the system are the cerebral cortex, cerebellum, thalamic nuclei, reticular formation, motor nuclei of the trunk. The connecting function is performed by extrapyramidal pathways. The main role of the extrapyramidal system – regulation of voluntary movements – is carried out along the descending tracts going to the motor neurons of the spinal cord. The effect of these factors leads to a violation of the described mechanisms, which causes the appearance of uncontrolled excessive movements. A certain pathogenetic role is played by a malfunction in the neurotransmitter system that ensures the interaction of various extrapyramidal structures.
Hyperkinesis is classified according to the level of damage to the extrapyramidal system, pace, motor pattern, time and nature of occurrence. In clinical practice, for the differential diagnosis of hyperkinetic syndrome, the separation of hyperkinesis according to four main criteria is of decisive importance.
By localization of pathological changes:
- With a dominant lesion of subcortical formations: athetosis, chorea, ballism, torsion dystonia. Characterized by a lack of rhythm, variability, complexity of movements, muscular dystonia.
- With predominant disorders at the level of the brain stem: tremor, tics, myoclonia, facial hemispasm, myorhythmias. They differ in the rhythmicity, simplicity and stereotype of the motor pattern.
- With dysfunction of cortical-subcortical structures: Hunt’s dissinergia, myoclonic epilepsy. Generalization of hyperkinesis, the presence of epileptic paroxysms is typical.
By the speed of involuntary movements:
- Rapid hyperkinesis: myoclonia, chorea, tics, ballism, tremor. Combined with a decrease in muscle tone.
- Slow hyperkinesis: athetosis, torsion dystonia. There is an increase in tone.
By type of occurrence:
- Spontaneous – arising independently of any factors.
- Promotional – provoked by arbitrary motor acts, a certain pose.
- Reflex – appearing in response to external influences (touching, tapping).
- Induced – committed partially at the will of the patient. To a certain extent, they can be restrained by the patient.
- Permanent: tremor, athetosis. They disappear only in a dream.
- Paroxysmal – appear occasionally in the form of time-limited paroxysms. For example, attacks of myoclonia, tics.
The main manifestation of the disease is motor acts developing against the patient’s will, characterized as violent. Hyperkinesis involves movements described by patients as “occurring due to an irresistible desire to perform them.” In most cases, excessive movements are combined with symptoms typical of causal pathology.
Tremor – rhythmic low- and high-amplitude oscillations caused by alternating contraction of antagonist muscles. It can cover various parts of the body, strengthen at rest or in motion. It accompanies cerebellar ataxia, Parkinson’s disease, Guillain-Barre syndrome, atherosclerotic encephalopathy.
Tics are jerky, low–amplitude arrhythmic hyperkinesis, capturing individual muscles, partially suppressed by the will of the patient. Winking, blinking, twitching of the corner of the mouth, shoulder area, head turns are more often observed. The tick of the speech apparatus is manifested by the pronunciation of individual sounds.
Myoclonia – disorderly contractions of individual bundles of muscle fibers. When spreading to a group of muscles, a sharp involuntary movement, a jerky change in the position of the body cause. Arrhythmic fascicular twitching that does not lead to a motor act is called myokymia, rhythmic twitching of a single muscle is called myorhythmias. The combination of myoclonic phenomena with epileptic paroxysms forms the clinic of myoclonic epilepsy.
Chorea – arrhythmic impulsive hyperkinesis, often of large amplitude. The basic symptom of minor chorea, Huntington’s chorea. Arbitrary movements are difficult. Typically, the onset of hyperkinesis in the distal extremities.
Ballism is a sharp involuntary rotation of the shoulder (hip), leading to a throwing movement of the upper (lower) limb. It is more often unilateral in nature – hemiballism. The connection of hyperkinesis with the lesion of the Lewis nucleus has been established.
Blepharospasm is a spastic closing of the eyelids as a result of hypertonicity of the circular muscle of the eye. It is observed in Gallervorden-Spatz disease, facial hemispasm, ophthalmic diseases.
Oromandibular dystonia is the forcible closing of the jaws and opening of the mouth due to involuntary contraction of the corresponding muscles. Provoked by chewing, talking, laughing.
Writing spasm is a spastic contraction of the muscles of the hand, provoked by writing. It is of a professional nature. Possible myoclonia, tremor of the affected hand. Family cases of the disease were noted.
Athetosis – worm-like slow movements of fingers, hands, feet, forearms, shins, facial muscles, which are the result of asynchronously occurring hypertonicity of the muscles of agonists and antagonists. It is characteristic of perinatal CNS lesion.
Torsion dystonia is slow generalized hyperkinesis with characteristic twisted body postures. More often it has a genetic condition, less often it has a secondary character.
Facial hemispasm – hyperkinesis starts with blepharospasm, captures the entire facial muscles of half of the face. A similar bilateral lesion is called facial paraspasm.
Akathisia is a motor anxiety. The lack of motor activity causes severe discomfort in patients, which encourages them to constantly make movements. Sometimes it manifests itself against the background of secondary parkinsonism, tremor, treatment with antidepressants, neuroleptics, DOPA pharmaceuticals.
Hyperkinesis is recognized based on a characteristic clinical picture. The type of hyperkinesis, concomitant symptoms, assessment of neurological status allow us to judge the level of damage to the extrapyramidal system. Additional studies are needed to confirm/refute the secondary genesis of hyperkinetic syndrome. The survey plan includes:
- Neurologist’s examination. A detailed study of the hyperkinetic pattern, identification of concomitant neurological deficit, assessment of the mental and intellectual sphere is carried out.
- Electroencephalography. The analysis of the bioelectric activity of the brain is especially relevant for myoclonia, allows you to diagnose epilepsy.
- Electroneuromyography. The study makes it possible to differentiate hyperkinesis from muscle pathology, neuromuscular transmission disorders.
- MRI, CT of the brain. They are carried out when organic pathology is suspected, they help to identify tumors, ischemic foci, cerebral hematomas, degenerative processes, inflammatory changes. In order to avoid radiation exposure, MRI of the brain is prescribed for children.
- Study of cerebral blood flow. It is carried out with the help of ultrasound of the vessels of the head, duplex scanning, MRI of cerebral vessels. It is shown under the assumption of the vascular genesis of hyperkinesis.
- Blood test. Helps to diagnose hyperkinesis of dysmetabolic, toxic etiology. Patients younger than 50 years of age are recommended to determine the level of ceruloplasmin to exclude hepatolenticular degeneration.
- Consultation of a geneticist. It is necessary in the diagnosis of hereditary diseases. It includes the compilation of a family tree to determine the nature of the inheritance of pathology.
Differential diagnosis is carried out between various diseases, the clinical picture of which includes hyperkinesis. An important point is the exclusion of the psychogenic nature of violent movements. Psychogenic hyperkinesis is characterized by inconstancy, sudden long-term remissions, polymorphism and variability of the hyperkinetic pattern, absence of muscular dystonia, positive response to placebo and resistance to standard treatment methods.
Therapy is mainly medicamental, carried out in parallel with the treatment of a causal disease. Additionally, physiotherapy techniques, hydrotherapy, physical therapy, reflexotherapy are used. The choice of a drug that stops hyperkinesis and the selection of dosage is carried out individually, sometimes it requires a long period of time. Among the antihyperkinetic agents , the following groups of pharmaceuticals are distinguished:
- Cholinolytics (trihexyphenidyl) – weaken the action of acetylcholine involved in the transmission of excitation. Moderate efficacy is noted in tremor, writing spasm, torsion dystonia.
- DOPA (levodopa) drugs – improve dopamine metabolism. They are used for torsion dystonia.
- Neuroleptics (haloperidol) – stop excessive dopaminergic activity. Effective against blepharospasm, chorea, ballism, facial paraspasm, athetosis, torsion dystonia.
- Valproates – improve gabaergic processes in the central nervous system. They are used in the treatment of myoclonia, hemispasm, tics.
- Benzodiazepines (clonazepam) – have a muscle relaxant, anticonvulsant effect. Indications: myoclonia, tremor, tics, chorea.
- Botulinum toxin preparations are injected locally into muscles subject to tonic contractions. Block the transmission of arousal to muscle fibers. They are used for blepharospasm, hemi-, paraspasm.
In cases of hyperkinesis resistance to pharmacotherapy, surgical treatment is possible. In 90% of patients with facial hemispasm, neurosurgical decompression of the facial nerve on the affected side is effective. Pronounced hyperkinesis, generalized tic, torsion dystonia are indications for stereotactic pallidotomy. A new way of treating hyperkinesis is deep stimulation of cerebral structures – electrostimulation of the ventrolateral nucleus of the thalamus.
Prognosis and prevention
Hyperkinesis is not life-threatening for the patient. However, their demonstrative nature often forms a negative attitude among others, which affects the psychological state of the patient, leads to social maladaptation. Pronounced hyperkinesis, which complicates voluntary movements and self-care, invalidizes the patient. The general prognosis of the disease depends on the causal pathology. In most cases, treatment allows you to control pathological motor activity, significantly improve the quality of life of the patient. Specific preventive measures have not been developed. Prevention and timely treatment of perinatal, traumatic, hemodynamic, toxic, infectious brain lesions helps to prevent the development of hyperkinesis.