Ataxia is an ailment that is a failure inContinuity of motor operations of different muscles against the background of a lack of muscle weakness. Simply put, ataxia is often an observed motor dysfunction, a neuromuscular defect, often of a genetic nature. In addition, it can originate due to serious interruptions with vitamin B12, leading to cerebral stroke. The motor acts with the considered sickness become distorted, clumsy, their consistency and order are violated, imbalance of balance is observed in motor operations and in standing position. In the limbs, strength can be maintained or slightly reduced.
Mismatch of coordination of motorOperations is manifested in the deterioration of precise and smooth execution of movements. The described state is caused by pathological processes leading to degeneration of the cerebellum or destruction of the afferent pathways.
Cerebellar ataxia can be generatedToxic intoxications caused by the use of certain pharmacopoeial drugs, alcohol-containing liquids or drugs, infectious processes and conditions after the transfer of infections (eg, HIV), inflammatory phenomena (multiple sclerosis), vitamin deficiency, endocrine disruptions (hypothyroidism), tumor and neurodegenerative processes, structural pathologies (Eg, arteriovenous malformations).
Sensitive ataxia is caused by destruction:
- posterior areas of white matter, which is a component of the brain (ascending brain pathways), more often caused by trauma;
- Peripheral nerve structures provoked by axon degeneration (axonopathy), segmental demyelination, secondary degenerative process (decay of nerve endings);
- posterior roots due to compression or trauma;
Medial loop (anatomical componentMiddle brain), which is an element of the conducting path, transporting impulses to the parietal segment and the cerebellum, which allow us to determine the position of the trunk or its elements in space;
- the thalamus, which plays an important role in the regulation of reflexes.
Often, this form of the described disease leads to destruction of the parietal segment from the contralateral margin.
Sensitive ataxia can often beIs provoked by the following diseases: spinal dryness, funicular myelosis, manifested by the breakdown of the brain (lateral and posterior) cordons, resulting from a prolonged B12 deficiency, certain varieties of polyneuropathies, vascular pathologies, and tumor processes.
This form of ataxia is also found in Friedreich's disease, which is the rarest form of the disease, the etiological factor of which is heredity.
Vestibular ataxia is generated by degenerativeProcesses affecting the cortical center, the vestibular nerve, nuclei, and the labyrinth of the brain. The variant of the disorder to be analyzed is observed with ear ailments, Menier's syndrome (an increase in the endolymph in the inner ear), stem encephalitis, and tumor processes.
Spinocerebellar ataxia is classified as a diseaseHereditary genesis. It is born when the structural elements of the nervous system are destroyed. The destruction in question is characterized by an escalation, that is, after a time they are aggravated. The most common is Friedreich's ataxia.
Often, with the form of violation describedThere is atrophy of the cerebellum, as a result of which Pierre-Marie enters the cerebellar ataxia, transmitted by the dominant inheritance mechanism. Simply put, the violation occurs due to the transfer from the parent of the abnormal gene. It is possible to single out a number of factors of exogenous character, which are considered so-called catalysts, which provoke the debut of the disease and worsen its course. Such triggers include various infectious processes (typhoid, brucellosis, pyelonephritis, dysentery, bacterial pneumonia), trauma, pregnancy, intoxication.
The frontal form of the described pathology appearsCause of destruction of the cortex of the frontal segment or occipiton-temporal zone. Often occurs due to the transfer of strokes, with trauma, tumor processes that result from the brain, abscesses, multiple sclerosis,
Symptoms of ataxia
In order to understand, ataxia, what it is,It is recommended to refer to the etymology of this concept, which means "disorder" if translated from Greek. The term being analyzed is a frequently occurring motor disturbance caused by a failure in the transmission of impulses. The malaise is manifested in the mismatch of motor operations against the background of a lack of muscle weakness. The described disorder is not a separately isolated nosological form, but rather a consequence or manifestation of any dysfunction of the nervous system.
The pathology considered leads to a lossMotor acts of consistency, consistency. They are made inaccurate, clumsy, walking often suffers. In some situations, speech disorders can occur.
On the static type of the pathology analyzedA breach of stability only in standing position. When a coordination failure occurs during the product of motor acts, they speak of the dynamic form of the described violation.
Ataxia of the cerebellum is manifested in a decreaseMuscular tonus of the atactic limb. When violations of motor operations due to the defeat of the cerebellum manifestation is not limited to a certain muscle, a muscle group or a separate movement. This variation of ataxia is characterized by a widespread character. It has a specific symptomatology: defects in standing and gait, coordination in the limbs, delayed speech with sporadic pronunciation of words, intentional trembling, vibrational spontaneous movement of eyeballs, reduced muscle tone.
Sensitive ataxia during examination is manifestedThe following symptomatology: coordination due to vision control, muscular-articular susceptibility disorder, vibration sensitivity, loss of stability in Romberg's position with blinked eyes, decrease or shutdown of tendon reflexes, instability of gait.
A specific feature of this variation of the diseaseIs a feeling of walking, as if on cotton wool. In order to compensate for motor defects, patients try to always look under their feet, strongly bend and raise their lower limbs high, dropping them on the floor surface with force.
The main manifestation of Friedreich ataxia isUnstable, awkward gait. Steps in patients are sweeping. They step, legs wide apart, deviating from the center. As the disease escalates, coordination disorders spread to the face, upper limbs, and chest muscles. Mimicry changes, and speech becomes more slow, jerky. Plexus and tendon reflexes are absent or highly minimized. Often there is a worsening of hearing.
Pierre-Marie cerebellar ataxia is aProgressive disease with chronic leakage. Its key symptom is neuromuscular motor dysfunction, not limited to individual motor acts or a group of muscles. This variation of ataxia is distinguished by such specific manifestations: disorders of an approach and statics, trembling of the body and
Limbs, muscular twitching, delayed speech, spontaneous stable vibrational movements of the eyeballs, a change in the handwriting, a decrease in the tonus of the musculature.
Vestibular ataxia has the following specificSymptoms: conditionality of the severity of symptoms from motor acts and corners and head movements, deep disturbances in standing and walking disorders without speech disorders.
In addition, this variation of ataxia is inherent in the presence of: - horizontal nystagmus (spontaneous vibrational horizontal movements of eyeballs, characterized by high frequency);
- sharp dizziness, present even in a lying position, often accompanied by nausea;
- propensity to fall, there is a shaky approach and a deviation in the direction of the side of the lesion (such deviations are also noted standing or sitting);
- rarely worsening of hearing.
Coordination of motor acts of hands atThe considered variation of ataxia is preserved. The symptomatology, which is multiplied by turns of the trunk, head or eyes, forces the patient to perform these motor operations slowly and try to avoid them. Violation of coordination compensates for visual control, and therefore
, Closing his eyes, does not feel confident. There are no disorders of the joint-muscular susceptibility. If there is dizziness when the eyes are closed in a lying position, then often a violation of falling asleep.
Also, vegetative reactions are often noted: facial hyperemia or pallor, sensation
, Increased heart rate, pulse lability,Increased sweating. When the cortical form of the disease, there are motor dysfunctions, expressed in violation of the gait. This feature is similar to the manifestations of the destruction of the cerebellum. The patient develops instability, uncertainty of gait. In the process of walking, the body is tilted back (Henner's symptom), the location of the feet on one line, often "tangled" in the limbs.
Spinocerebellar ataxia is characterized byA variety of manifestations, the key of which is coordination dysfunction. Also for the ailment are motor disorders of the eyes, expressed in sharp, jerky movements of the eyes during the movement of the gaze. Often there are violations of swallowing, speech disorders, a decrease in the functioning of the hearing aid, failures in acts of defecation and urination, paralysis of the limbs, pathological reflexes are observed.
Types of ataxia
Clinical medicine distinguishes several variations of ataxia: sensitive, frontal, cerebellar, vestibular, functional and hereditary, such as Friedreich, Pierre-Marie, and Louis-Bar ataxia.
The sensitive form is caused by the defeat of deepVarieties of sensitivity. With the analyzed form, there is a malfunction of the joint-muscular susceptibility, which takes information about the placement of the subject's trunk in space, failures in the vibration sensitivity, disturbances in sensation of pressure and weight. There is a "stamping gait", expressed in the excessive bending of the limbs and an excessively strong approach. A sick individual often loses the idea of placing the elements of his own body in space, does not feel the direction of the motor acts. Subjects suffering from this type of pathology try to compensate for motor dysfunctions through vision: during walking they always look under their feet, which helps to reduce the feeling of ataxia. However, with the closed eyes, the patients can not move.
The key differential-diagnosticThe criterion of the described ataxia variety is considered to be the appearance of a symptomatology or a significant increase in manifestations in the absence of control by sight, for example, with the eyes closed, in the dark.
This form can be found in the upperLimbs, which is manifested by pseudoathetosis, which is a slow vermiform movement of the hands with loss of visual control. Pseudoathetosis and the sensitive form of ataxia are always accompanied in the extremities of the disorder of deep sensitivity.
The cerebellar variation of ataxia originates,Occurs in the degenerative processes of the cerebellum. A characteristic criterion for diagnosing is the inability of an individual to stand in Romberg's position smoothly. Patients fall in the direction of the affected segment of the cerebellum. Patients suffering from this variation of the disease, when walking, stagger, widely arrange the limbs. Their movements are characterized by sweeping, slowness and awkwardness. In contrast to the sensitive variety of the pathology under consideration, with this variation of ataxia, a strong relationship with vision is not observed. In this case, the patients are slow, stretched, and the handwriting becomes uneven and sweeping. Rarely from the destruction of the cerebellum there is a decrease in the tone of the muscles and malfunctions of the tendon reflexes. The described variation of ataxia is often a sign of multiple sclerosis, various encephalitis, malignant tumor processes.
The vestibular variety of ataxia appearsDue to the defeat of some structures of the vestibular apparatus. A key sign is system dizziness: the subject sees that the surrounding objects move in one direction. Due to head turns, dizziness becomes worse. Often this form is accompanied by nausea, a horizontal nystagmus. It can be a sign of stem encephalitis, a number of ailments of the ear, serious tumor processes of the ventricles of the brain.
At inspection the individual is not able to passA couple of steps straight with your eyes closed. It will always deviate from the given direction. His way will be like the outlines of a star. If the patient tries to get up and take several steps, he often falls in the direction of the affected labyrinth, also stimulation of the vestibular apparatus and turning of the head often affect the course of the fall.
Frontal ataxia often accompanies such ailments,As strokes, brain tumor processes, hematomas, encephalitis. The walk of an individual suffering from the form of pathology in question is made "drunk". When walking, the subject deviates in the direction opposite to the site of destruction. With severe degenerations of the frontal segment, astasia-abasia appears, which is manifested in the inability to walk or stand without outside help in the absence of paralysis of the limbs. Often this variant is accompanied by other signs of destruction of the frontal zone (a change in the psyche, an olfactory disorder) or the temporo-occipital segment (partial blindness, auditory
On the ongoing events).
The functional form of ataxia is observed
Hysterical genesis. It is characterized by a gait that changes in dynamics and is not inherent in the clinical picture of the above ataxia species. It is combined with the symptoms of a hysterical neurosis.
Friedreich ataxia is progressiveAilment of hereditary genesis. It combines the symptoms of cerebellar ataxia and the sensitive. This type of male gender is more likely to suffer. The debut of the ailment falls on the period of childishness and during the course of existence it intensifies. Disorders are first observed in the lower limbs, gradually grabbing arms and trunk. Also, visual defects are noted, the joint-muscular sense is seriously disturbed. Often the muscle tone is lowered, tendon reflexes are weakened, nystagmus is found, often - hearing loss. In addition, it is possible to reduce the intellectual function gradually down to the stage
. In addition to neurologic symptoms, uIndividuals suffering from Friedreich ataxia, kyphoscoliosis (deformity of the spinal column), myocardial dystrophy (structural changes in the myocardium) is detected. Therefore through time there is a dyspnea, pristupoobnye cardiac algies, tachycardia.
Pierre-Marie ataxia has a genetic etiology andIs shown in the young age period. With the considered variation of the pathology being analyzed, there is a decrease in strength in the muscles of the extremities, muscle tone is elevated, and tendon reflexes are strengthened. Quite often develop oculomotor disorders, a decrease in visual acuity, due to the destruction of the optic nerve, there is a limitation of the field of vision, there are systematic depressive moods,
Louis-Bara ataxia is also a geneticPathology, combining the destruction of the nervous system, dermis, eyes and internal organs. He makes his debut in the early childhood stage. Can be detected at birth. She is symptomatic of progressive ataxia of the cerebellum and a high predisposition to infectious diseases. There is a decrease in reflexes, monotony of speech. Also, there is often a delay in growth and a lag in intellectual development. A specific criterion for this variation of the disease is telangiectasia (an expansion of small capillaries of the dermis), expressed by small reddish oblong speckles on the epidermis, filled with a network of minute and visible under the skin capillaries.
Treatment of ataxia
In order to determine the therapeutic course,It is necessary to first understand what is ataxia in humans. The essence of this pathology is a serious mismatch of motor acts and coordination defect, not due to muscle weakness. The described pathological symptomatology can be observed on the limbs or touch the entire body. In addition, with ataxia, speech defects and a number of vegetative dysfunctions are not uncommon.
This ailment is a syndrome, localCharacter, expanding through time throughout the human body, giving rise to other pathologies. Therefore, choosing a therapeutic strategy, it is important to direct it to eradicate the essence of the problem that caused ataxia.
What is ataxia in man - should be understood in order to competently combat it.
Ataxia is a complex of defects leading to randomness of the patient's motor acts. Individuals suffering from the described destruction, are not able to control their own movements.
Treatment of ataxia is a complex process,Since the corrective effect should be directed not so much at eradicating symptoms and reducing symptoms as on eliminating the etiologic factor. In order to achieve this, it is often necessary to resort to an operative intervention, for example, to eliminate a cyst or a tumor process.
Before choosing a treatment strategy, a doctorTo establish the form of the ailment in question, since each species determines the specificity of therapy and implies various corrective methods, since the etiological factor in different ataxia species is not the same. For example, when Louis-Bar ataxia is observed a specific symptom - telangiectasia, which has no effective methods of correction. Here only competent care, correction of immunodeficiency and support of relatives are possible.
If intoxication processes gave rise to ataxia,Then the first thing to do is to eliminate the cause of poisoning and nourish the body with vitamin preparations. In disorders of the vestibular analyzer, the use of antibacterial agents and hormones is practiced.
In Friedreich ataxia, a large role in the relaxation of symptoms is played by agents that can support the functioning of mitochondria (Riboflavin, succinic acid, vitamin E).
Symptomatic therapy includes the followingAlgorithms. First of all, general strengthening measures are carried out, including vitamin therapy and the appointment of anticholinesterase drugs, a specially developed system of exercises aimed at strengthening the musculature and reducing the phenomena of discoordination. Also shown the appointment of vitamin B drugs group, Cerebrolysin, immunoglobulins, ATP, Riboflavin.
The prognosis of ataxia, having hereditary genesis,Maloblagopriyatny because there is a progression of nervous disorders and mental dysfunction. Decreased ability to work. Symptomatic therapy can prevent repeated infectious ailments and intoxication processes, which will allow patients to live to the elderly.