Dynamic ataxia. Types and forms: cerebellar

A pathology that manifests itself as impaired motor skills is called ataxia. It occurs as a result of the development of neoplasms or serious diseases of the brain and spinal cord. There are several types of ataxia, depending on the location of the lesion. Setting the latter is prerequisite effective treatment.

general information

The term “ataxia” has Greek roots and literally translates as “disorder.” The disease he describes is quite common and manifests itself as a lack of coordination and balance. When it occurs, a person experiences inconsistency in interaction various groups

muscles. Moreover, the strength of his limbs is either completely preserved or decreases slightly. At the same time, the lesions entail vagueness and clumsiness of movements not only while walking, but also in a normal standing position. Due to the fact that the body has several sections nervous system , ensuring balance and coordination, when ataxia occurs, the doctor, first of all, conducts an examination to exclude malfunctions in one of them. That is, in the work of the cerebellum, frontal lobe cortex, vestibular apparatus, conductors of deep joint-muscular sensitivity, occipital and temporal lobe

brain.Note! Ataxias are pathologies that entail uncoordination of movements. In other words, a person with such a diagnosis is impaired: speech, walking, fine motor skills

, swallowing, eye movements. Her motor activity may become unsmooth, intermittent, or even more difficult.

Kinds

• First of all, hereditary and acquired ataxia is diagnosed. There is another classification - depending on the nature of the lesions. According to it, pathology can be:
• static, when balance is disturbed (it is difficult for a person to stand calmly);
• dynamic, when there is a disturbance in movements, for example, when walking;

static-dynamic, when signs of both types appear.

• Depending on the location of the lesion, ataxia is distinguished: Sensitive. It appears when muscle-joint sensitivity is impaired. The latter is possible with damage to the posterior columns of the spinal cord, peripheral nerves, thalamus, parietal lobe cortex, development of neurosyphilis or various. The disease manifests itself as instability and an unusual gait, when a person bends his legs excessively at the knees or hip joints, stamps his foot pronouncedly. Distinctive feature The disease is an increase in the symptoms of the disease at the moment when the patient closes his eyes.
• Cerebellar. Damage to the cerebellum can provoke both the development of dynamic ataxia, diagnosed when problems arise in the hemispheres, and statodynamic ataxia. The latter is observed against the background of pathologies of the cerebellar vermis and is manifested by gait disturbances, instability. The causes of the disease can be either brain tumors or vascular diseases cerebellar structures. In addition to everything, with cerebellar ataxia, handwriting and speech disorders may develop.
• Vestibular. This ataxia is diagnosed when there is a malfunction of the vestibular apparatus. Along with unsteadiness in this case, a person feels constant dizziness, nausea up to vomiting, horizontal nystagmus. The cause of the development of the disease is most often ear diseases, especially Meniere's disease. Distinctive feature Such ataxia has a pronounced dependence on head movements. When making sharp turns, the symptoms intensify.
• Cortical. Pathology occurs when a tumor appears frontal lobes, for abscesses, circulatory disorders in the brain, encephalitis. Its main symptoms are incoordination of movements, disturbance of gait, sometimes loss of the ability to walk and stand, pronounced deviation of the body back, braiding of the limbs, the appearance of a “fox gait” when the patient puts his feet in one line. Despite the fact that vision does not affect the severity of symptoms, the latter are often accompanied by impairments of hearing, smell, memory, and the appearance of hallucinations.

brain. Doctors also distinguish psychogenic and intrapsychic ataxia. If they develop, a person experiences a split in mental functions. This type of disease can be recognized by its elaborate movements when walking: the patient walks without straightening or crossing his legs.

Ataxias, which are hereditary in nature, are also divided into several types, namely:

• Ataxia Pierre Marie– transmitted from parents and is characterized by the appearance of symptoms of cerebellar ataxia, cerebellar hypoplasia, and atrophy of the cerebral pons. At risk are people aged 30–35 years. Pathology is suspected in cases of disturbances in facial expressions, speech, difficulties in walking, loss of the ability to quickly move the arms, involuntary contractions of the muscles of the fingers and limbs, ptosis - drooping of the upper eyelid, the appearance of depression, fear.
• Friedreich's ataxia. Pathology that occurs in consanguineous marriage. It manifests itself mainly as gait disturbances, when a person places his legs too wide when walking. As the disease progresses, there is a violation of hand coordination and facial expressions, when involuntary contraction of the facial muscles occurs, speech slows down, hearing impairment, and decreased reflexes. If the patient is not provided with timely assistance, he develops cardiovascular diseases, and the risk of fractures and joint dislocations increases. The pathology is often accompanied hormonal disorders, diabetes mellitus, sexual dysfunctions.
• Louis-Bar syndrome. Hereditary pathology, the signs of which appear even in early childhood When a child reaches the age of 9, he loses the ability to walk. Along with this, he has mental retardation, a tendency to acute infectious diseases - hypoplasia thymus. Often against this background a malignant tumor arises, which aggravates the course of the disease, making its prognosis unfavorable.

Causes of ataxia

The causes of pathology are:

brain.Acute forms of ataxia occur, as a rule, against the background of sickle cell anemia.

Symptoms of ataxia

It is worth noting that each form of the disease manifests itself differently. Meanwhile, most pathologies are characterized by the following symptoms:

Diagnostics

Diagnosis of ataxia consists, first of all, in identifying its form. To do this, the specialist collects anamnesis, conducts a general examination, and sends the patient for laboratory testing. instrumental studies.

When collecting anamnesis, the doctor asks about diseases suffered and used to treat them. medicines, heredity. At general examination he evaluates muscle tone, quality of vision, hearing, reflexes, and also conducts coordination tests - finger-nose and knee-heel.

In addition, he may refer the patient to:

• and and - they allow you to exclude or confirm the presence of metabolic failures, as well as identify signs of poisoning or inflammation;
• – the procedure helps to diagnose atrophic processes in the upper part of the skull;
• electroencephalography - a method designed to assess the electrical activity of individual areas of the brain;
• and the spinal cord - the results of this study help to look layer by layer at the structure of the brain tissue, identifying possible pustules, neoplasms, and hemorrhages;
• spinal tap with mandatory analysis cerebrospinal fluid;
• genetic tests to detect mutations;
• DNA diagnostics if there are hereditary pathologies.

Important!To make an accurate diagnosis, it is extremely important to contact an experienced specialist and go through all necessary examinations. IN in rare cases The pathology may resemble multiple sclerosis in its symptoms, thereby misleading doctors.

Treatment of ataxia

Treatment of ataxia is predominantly symptomatic. Neurologist prescribes vitamin complexes, including those that contain , and drugs aimed at general strengthening body, increasing immunity and maintaining motor activity . Additionally, normalization agents may be prescribed blood pressure or improving brain microcirculation.

When an infection is detected, it is used. At multiple sclerosis possible use hormonal drugs and plasmapheresis.

brain. The key to success in treating ataxia is timely detection reasons for the development of pathology and its elimination. This is why tumors are removed surgically.

When diagnosing poisoning, supporting solutions are administered. Additionally, to strengthen the body, the doctor may recommend physical exercise aimed at increasing muscle tone. Canes, walkers or other devices can also help ease the patient's condition.

Forecast

Early detection of the disease and following all the recommendations of a specialist allows a person to maintain working capacity for as long as possible. At the same time, the lack of qualified medical care can result in destruction of the neuropsychic system and a constant deterioration in the patient’s well-being. As a result - unfavorable outcome both for ability to work and for life is practically guaranteed.

Vestibular ataxia is a type of ataxia that results in dysfunction of the vestibular system. The vestibular system consists of the inner ear canals, which contain fluid.

They sense head movements and help with balance and spatial orientation. Vestibular ataxia is a consequence of a disorder inner ear.

Signals from the inner ear are unable to reach the cerebellum and brain stem when a person has vestibular ataxia. A person with vestibular ataxia experiences a loss of balance while maintaining strength. The patient often experiences dizziness, a feeling that everything around him is spinning. Shows loss of balance or involuntary eye movement.

In unilateral or acute cases the anomaly is asymmetrical, the patient experiences nausea, vomiting, and dizziness. In slow chronic bilateral cases it is symmetrical, the person only feels an imbalance or instability.

There are many types of ataxia. In this article we will discuss some of the most common types, causes and available methods treatment.

Fast Facts

Ataxia is caused wide range factors.

• Symptoms include poor coordination, slurred speech, tremors, and hearing problems.
• Diagnosis is complex and often requires a number of studies.
• It is not always curable, but symptoms can often be relieved.

It belongs to a group of disorders that affect coordination, speech, and balance. Makes it difficult to swallow and walk.

Some people are born with it, others develop the syndrome slowly over time. For some, it is the result of another condition such as stroke, multiple sclerosis, brain tumor, head injury, or overconsumption alcohol.

It gets worse or stabilizes over time. It partly depends on the cause.

Types

The following are some of the most common types of ataxia:

Cerebellar ataxia

The cerebellum is responsible for sensory perception, coordination and motor control.

Caused by dysfunction of the cerebellum, an area of ​​the brain involved in the assimilation of sensory perception, coordination and motor control.

Cerebellar ataxia provokes neurological problems, such as:

The extent of symptoms depends on which parts of the cerebellum are damaged, and whether there is damage on one side (unilateral) or on both sides (bilateral).

If the vestibular system is affected, balance control of the face and eyes will be impaired. The person stands with their feet wide apart to gain better balance and avoid rocking back and forth.

Even when the patient's eyes are open, balance when bringing the legs together is difficult. If the cerebellum is affected, the patient will have an unusual gait with uneven steps, and stuttering begins and stops. Spinocerebellum regulates body position and limb movement.

If the deep structures of the brain are affected, the person will have problems with voluntary movements. The head, eyes, limbs, and torso may tremble when walking. Speech is slurred, with changes in rhythm and volume.

Sensory ataxia

Appears due to loss of proprioception. Proprioception is the sense of the relative position of adjacent parts of the body. Indicates whether the body is moving with the required force and provides feedback regarding the position of its parts relative to each other.

A patient with sensory ataxia typically has an unsteady stomping gait, with the heel striking hard as it hits the ground with each step. Postural instability worsens in low light conditions. If the doctor asks you to stand up eyes closed and feet together, instability will worsen. This is because the loss of proprioception makes a person much more dependent on visual input.

It is difficult for him to perform smoothly coordinated movements of the limbs, torso, pharynx, larynx, and eyes.

Cerebral ataxias

Early onset cerebellar ataxia usually occurs between the ages of 4 and 26 years. Late appears after the patient has reached 20. Late is characterized by less severe symptoms compared to early.

Ataxia is a motor disorder not accompanied by paralysis, characteristic features which are rhythm disorders and Translated from Greek, the word ataxia means “chaotic” and “disorganized.”

With this diagnosis, movements become disproportionate, awkward, inaccurate, walking often suffers, and in some cases even speech. Let's consider what types of ataxia are distinguished and what are their characteristic features.

Friedreich's ataxia

Friedreich's hereditary ataxia is a genetically determined neurological disease that is progressive. Initial manifestations the disease becomes noticeable in the first few decades of life.

First, handwriting and gait disturbances appear. For children, however, it is difficult to identify handwriting disorders due to the fact that it has not yet been fully formed. As for gait, the patient needs support; he constantly sways. All leg movements are more jerky than progressive.

Over time, the inability to stand (astasia) and even walk (abasia) develops. However, the latter is a characteristic manifestation of the rapidly progressing course of the disease and its final stages.

Changes in the spine are observed, which is especially important for adolescents in whom the process of its formation has not yet been completed. After a few years, the patient develops diabetes due to malfunctions of the pancreas. A little later due to dystrophic changes hypogonadism occurs in the gonads. On late stages Ataxia is accompanied by visual impairment, which occurs as a result of dystrophic changes in the visual and In addition, dementia develops due to damage to neurons in the brain.

Cerebellar lesion

Cerebellar ataxia is a disorder of motor coordination that develops when the main organ responsible for it is damaged. In some cases, minor changes develop, and in others, more serious and severe ones.

The development of such ataxia occurs due to the impact various areas cerebellum pathological process. Often cerebellar ataxia diagnosed with encephalitis, vascular diseases cerebellum, multiple sclerosis, malignant tumors brain, intoxication, as well as certain ailments of genetic origin. There are 2 types of cerebellar ataxia - static and dynamic.

Static nature of cerebellar ataxia

It is expressed in static ataxia due to a reduction in muscle tone. During this process, it becomes difficult for the patient long time stay in one position, he also has a slight impairment of motor coordination. The person moves with very wide and staggering steps, as if he is in a state of intoxication. When severe course Due to the disease, the patient is unable to sit and stand independently, since, not even having the strength to hold his head up, he constantly falls. Static ataxia in severe form deprives the patient of the ability to independently maintain balance. It is worth noting that motor coordination is not affected by whether the patient is with his eyes open or with his eyes closed.

The dynamic nature of cerebellar ataxia

Dynamic ataxia develops when the cerebellar hemispheres are involved in pathological process. With this type of disease, coordination disorders are observed exclusively during physical movement. The smoothness and precision of movements are lost, they become sweeping and awkward. Discoordination and slower movements are observed on the affected side. Dynamic ataxia is characterized by hypermetry (excessiveness, opposite movements), adiadochokinesis, misses, as well as intention tremor and speech disorders (patients speak slowly, dividing words into syllables).

In a standing position and when walking, the patient deviates to the side corresponding to the damaged cerebellar hemisphere. The patient's handwriting changes: it becomes uneven, sweeping, with large letters. A decline is possible tendon reflexes.

Sensitive ataxia

This ataxia is a movement disorder in which changes in gait occur due to loss of sensation in the legs resulting from damage to the peripheral nerves, medial lemniscus, dorsal columns, or dorsal roots of the spinal cord. The patient does not feel the position of his legs, and therefore experiences difficulties both when walking and when standing. As a rule, he stands with his legs spread wide apart and can only maintain his balance when open eyes, if you close them, the person will begin to stagger and, most likely, fall ( positive symptom Romberg). When walking, patients also spread their legs wide and raise them much higher than necessary, and also sway impulsively, first and then back. Their steps have different lengths, and their feet make clapping sounds when they touch the floor. When walking, the patient usually uses a stick for support and slightly bends the torso at the hip-femoral joints. Gait disturbances are aggravated. Often, patients lose stability, sway and fall when washing, because when they close their eyes, they temporarily lose visual control.

Spinocerebellar ataxia

This term refers to a variety of movement disorders, mostly resulting from ischemic damage to the central nervous system in the perinatal period or hypoxia. The severity of gait changes can vary and depend on the severity and nature of the lesion. Thus, mild limited lesions can cause Babinski's symptom, increased tendon reflexes and not be accompanied by pronounced change gait Larger and more severe lesions usually result in bilateral hemiparesis. There are changes in gait and posture characteristic of paraparesis.

Causes movement disorders, leading to changes in gait. In patients this occurs in the extremities, which are accompanied by grimaces on the face or rotational movements of the neck. As a rule, the legs are extended and the arms are bent, however, this asymmetry of the limbs can become noticeable only with careful observation of the patient. So, for example, one arm may be pronated and extended, while the other is supinated and flexed. Asymmetrical position of the limbs often occurs when the head is turned in different directions.

Diagnosis of ataxia

To establish a diagnosis, the following diagnostic methods are used:

• MRI of the brain;
• electroencephalography of the brain;
• DNA diagnostics;
• electromyography.

In addition to any of the indicated methods, it is necessary to take a blood test and undergo an examination by specialists such as a neurologist, psychiatrist and ophthalmologist.

Treatment of ataxia

Ataxia is serious illness, requiring timely action. Treatment carried out by a neurologist is predominantly symptomatic and includes the following areas.

1. General restorative therapy (anticholinesterase drugs, Cerebrolysin, ATP, B vitamins).
2. Physiotherapy aimed at preventing various kinds complications ( muscle atrophy and contractures, for example), improving walking and coordination, maintaining physical fitness.

A special gymnastic complex of physical therapy exercises, the purpose of which is to reduce incoordination and strengthen muscles. At in a radical way treatment (surgery for cerebellar tumors, for example) can be expected to be partial or full recovery or, at a minimum, stopping further progression.

In Friedreich's ataxia, if we take into account the pathogenesis of the disease, drugs aimed at maintaining mitochondrial functions (Riboflavin, vitamin E, coenzyme Q10, succinic acid) can play a huge role.

Prognosis for the disease

Forecast hereditary diseases is quite unfavorable. Over time, especially with inaction, neuropsychic disorders only progress. People diagnosed with ataxia, the symptoms of which become more pronounced with age, usually have a significantly reduced ability to work.

However, thanks to symptomatic treatment, as well as the prevention of intoxication, injury and infectious diseases, patients live to an old age.

Prevention

Conduct preventive actions specifically for ataxia is not possible. First of all, it is necessary to prevent possible appearance and the development of acute infectious diseases (sinusitis, otitis, pneumonia, for example), which can provoke ataxia.

Consanguineous marriages should be avoided. In addition, it should be remembered that there is a high probability of transmitting hereditary ataxia from parent to child, and therefore patients are often recommended to refuse the birth of their own babies and adopt someone else’s child.

Ataxia is a serious neurological disorder that requires immediate treatment. That is why the earlier this disease was identified, the more favorable the prognosis for the patient will be.

Ataxia is a motor disorder in which a person cannot coordinate movements normally. Patients experience some loss of limb strength and find it difficult to maintain balance while standing or moving. From the outside, the movements seem awkward, a person cannot do them accurately, continuity and consistency are disrupted.

Ataxia: how is it?

Ataxia is a condition associated with the inability to accurately control movements. Normally, coordination is realized through the activity of muscle structures: synergists, antagonists. For a normal sequence of contractions, the coordination system has three control mechanisms: through the cerebellum, receptors and impulses of the vestibular apparatus. The cerebellum is a central organ; its connections and systems are most important for correct coordination. Receptors are necessary to assess how stretched muscles, joint capsules, and tendons are. Through receptors, at each moment of time, information about the state of tissues is supplied to the coordinating center. Finally, impulses are necessary to assess the position of the organism in space.

Ataxia is a pathological condition in which the functioning of one of three points or several at once is disrupted. The classification of cases is based on an assessment of the performance of different elements of the coordination system.

What is?

Main types of disease:

• sensitive;
• vestibular;
• cerebellar;
• cortical.

Possible hereditary ataxia. These are diseases of Friedreich, Pierre-Marie, Louis-Bar.

Where did the trouble come from?

The causes of ataxia are various bruises and blows, injuries to the skull and brain. The ability to coordinate movements may be impaired if fluid accumulates in the brain cavities, if there is a malformation of the brain or cranium, as well as impaired blood flow. Ataxia may occur due to encephalitis or paralysis, cancer or abscess. If in childhood the patient suffers from epilepsy, the risk of developing ataxia is assessed as above average.

More about types

Sensitive type ataxia syndrome can be observed if the integrity and functionality of the peripheral nodes or posterior brain stem, parietal medulla, posterior nerves and tubercle responsible for vision are impaired.

The cerebellar form is observed if the health and integrity of the vermis of this organ, legs, and hemispheres are impaired. More often this is observed against the background of sclerotic processes and encephalitis.

The vestibular type of ataxia is observed if the functioning and integrity of the vestibular apparatus (any of its parts) is disrupted. The brainstem nuclei, the cortex in the temporal part of the brain, the labyrinth of the brain, or the nerve responsible for the functioning of the system may be affected.

The cortical form is possible when the functioning of the frontal lobe is disrupted.

Hereditary ataxia can be transmitted by one of two mechanisms: autosomal recessive, autosomal dominant.

How to notice?

Symptoms of ataxia depend on the form of the disease. In particular, with sensitive legs, primarily the muscles and joints suffer. The patient loses stability, bends his legs excessively when walking, and feels as if he is walking on cotton wool. Reflexively, such a person constantly looks at his feet while moving to compensate for the lack of activity. musculoskeletal system. If you close your eyes, the symptoms will become more pronounced. If the damage to the brain areas is very severe, the patient loses the ability to move in principle.

Symptoms of cerebellar ataxia include unsteadiness when walking, unduly wide leg spacing, and sweeping movements. The patient is awkward, and when walking he falls to one side - this shows which hemisphere of the cerebellum is affected. Problems with coordination do not depend on visual control of the situation; symptoms persist equally if the eyes are open and closed. The patient speaks slowly and writes loosely. Muscle tone decreases, tendon reflexes are impaired.

Manifestations: what else are possible?

If the vestibular form develops, it can be noticed by frequent urges to nausea and vomiting. The patient feels dizzy, this sensation becomes stronger if you turn your head, even with a smooth movement.

Cortical ataxia expresses itself as instability during movement, most pronounced when turning, disturbances in the perception of smells, as well as mental disorders. The patient's grasping reflex disappears.

Hereditary forms

Pierre-Marie's ataxia is close to cerebellar in its manifestations. More often, the first manifestations of the disease are observed at the age of about 35 years - gait is disturbed, difficulties with facial expressions are observed. It is difficult for the patient to move his arms and speak, tendon reflexes increase, and muscle strength lower limbs weakens. Involuntary short-term convulsions are possible. Vision declines, intelligence deteriorates. Many suffer from depression.

Friedreich's disease is accompanied by dysfunction of the Gaulle, Clarke, and spinal systems. The gait becomes awkward, the person moves uncertainly, sweepingly, while the legs are spread unreasonably wide. The patient deviates from the center in different directions. Facial expressions gradually suffer, speech deteriorates, reflexes of certain groups of tendons decrease, and hearing weakens. If the disease is severe, the skeleton changes and the heart suffers.

Louis-Bar syndrome usually occurs in children. The disease is characterized high speed progress, and by the age of ten the patient can no longer move. The functioning of the nerves of the skull is disrupted, intelligence lags behind, and immunity decreases. Patients with this syndrome are characterized by frequent bronchitis, pneumonia, and runny nose.

How can I check the status?

If you suspect cerebellar ataxia, vestibular or any other of those described above, you need to consult a doctor for a full diagnosis. Only after a final diagnosis has been formulated can a treatment program be chosen.

If ataxia is suspected, the patient is referred to MRI to assess the state of the brain, electroencephalography and electromyography. If a hereditary form is suspected, DNA diagnostics is necessary. Based on the results of this type of indirect analysis, doctors determine how high the probability of inheriting the pathogen within the family is. The patient is also prescribed MRI angiography. If there are brain tumors, this method helps to determine them as accurately as possible.

To identify hereditary, static, cerebellar ataxia and any other form, a series of tests is done additional research. The patient is examined by an ophthalmologist, a neurologist, and a psychiatrist. IN laboratory tests metabolic problems can be identified.

How to fight?

Treatment of ataxia is possible only in a clinical setting. It is impossible to cope with this disease on your own - it does not matter in what form or type the disorder develops. Treatment control rests with the neurologist. The main idea of ​​the therapeutic course is to eliminate the disease that led to ataxia. If it is a neoplasm, it is removed; if there is a hemorrhage, the damaged tissue is removed. In some cases, removal of the abscess and stabilization of pressure in the circulatory system, decreased pressure in the posterior fossa of the skull.

Treatment of ataxia involves practice gymnastic exercises, a complex of which is developed based on the patient’s condition. The main task of gymnastics is to strengthen muscle tissue and reduce coordination problems. The patient is discharged restoratives, vitamins, ATP.

How to treat ataxia in Louis-Bar syndrome? In addition to the measures described above, the patient is prescribed drugs to eliminate immunodeficiency. A course of immunoglobulin is prescribed. For Friedreich's disease, medications are indicated to correct the performance of mitochondria.

What if you don't treat it?

With ataxia, a person cannot move normally, so the progress of the pathology becomes the reason for assigning the status of a disabled person. There is a risk of death. With ataxia, patients suffer from hand tremors, severe and frequent dizziness, it is impossible to move independently, swallow, and the ability to defecate is impaired. Over time, there is insufficient work respiratory system, hearts in chronic form, immune status decreases. The patient is characterized by frequent infections.

Obvious complications are not observed in 100% of cases. If you strictly follow the doctor’s recommendations and take medications prescribed by a specialist, and take measures to correct the signs of the disease, your quality of life will remain at the same level. Patients receiving adequate therapy live to an advanced age.

Dangers and likelihood of getting sick

The predominant percentage of patients are individuals predisposed to ataxia due to a genetic factor. It is highly likely that treatment for ataxia (cerebellar, sensitive or other type) will be required for people who have had a brain infection, suffer from epilepsy and malignant neoplasms. The likelihood of ataxia is higher in the presence of malformations of the skull, brain, and blood flow problems.

To minimize the birth of people suffering from ataxia, with poor heredity, it is necessary to take a very responsible approach to the issue of procreation. In some cases, the doctor may recommend that you abstain from having children altogether. This is especially true if you already have children with ataxia due to genetics.

Prevention of ataxia involves avoiding marriages between close relatives, treating any infectious foci and controlling blood pressure, as well as maintaining a normal daily routine, proper nutrition. Sports that carry a risk of TBI should be avoided.

Cerebellar form: features

With this form of the disease, the patient cannot coordinate movements, speech becomes chanted, and trembling of the arms, legs, and head is bothersome. The disease can affect both children and adults. To clarify the diagnosis, a physical examination and instrumental studies are necessary. Ataxia cannot be completely eliminated. At rapid development The prognosis for the disease is negative. The exception is cerebellar ataxia, which is explained by infection.

More often the disease is detected in a hereditary form; a much smaller percentage of cases are acquired. Cerebellar ataxia can be provoked by a lack of vitamin B12, head injury, neoplasm, virus or infection, sclerosis, cerebral palsy and similar pathological conditions, stroke, poisoning with poisons, metals. Statistics show that the acquired form is more often observed after a stroke or injury. Hereditary may be explained gene mutation. Currently no detailed explanation causes of processes.

Types and forms: cerebellar

Hereditary disease It can be congenital and not prone to progress, autosomal recessive and recessive, in which cerebellar insufficiency gradually progresses. There is the Batten form, a congenital form in which the child’s development slows down, but in the future the patient manages to adapt. A late format of cerebellar ataxia is Pierre-Marie's disease. Mostly diagnosed at the age of 25 years and older.

Cerebellar ataxia can be acute (against the background of viral invasion, infection), subacute, provoked by a neoplasm, sclerosis, chronic, prone to progress, and paroxysmal-episodic. To clarify which form needs to be dealt with in a particular case, the doctor prescribes tests and studies.

Specifics of manifestation of the cerebellar form

Clinical picture the progressive form of the disease is specific, so establish accurate diagnosis usually easy. The disease can be assumed based on general symptoms, the patient’s behavior, the postures he takes. When observing a person, you get the feeling that he is trying to balance, for which he spreads his arms to the sides. The patient tends to avoid turning his body and head, and voluntarily falls from a slight push when trying to move his legs, and does not even realize this. The limbs are tense, the gait is similar to that of a drunk, the body is straightened and thrown back.

As cerebellar ataxia progresses, it results in an inability to correct movements. It is impossible to touch the tip of the nose. The kidneys and speech change, the face looks like a mask, the muscles are toned all the time, the back, neck, legs and arms hurt. Possible convulsions, nystagmus, strabismus. Some patients experience weakened vision, hearing, and difficulty swallowing.

Cerebellar form: congenital

You can suspect a disease in a child if the efforts the baby puts into making movements are disproportionate to the action. The patient is unstable, development is slow, he begins to crawl and walk later than his peers. Nystagmus is observed, words are pronounced in syllables, clearly separated from each other. The development of speech and psyche is slowed down.

The listed symptoms may indicate not only cerebellar ataxia, but also some other pathological conditions associated with brain function. To clarify the diagnosis, it is necessary to show the child to the doctor.

What to do?

With cerebellar ataxia, the main goal of treatment is to slow down negative processes. The Friedreich form, like other congenital forms, cannot be treated. The use of radical, conservative approaches helps preserve the patient’s quality of life for as long as possible. Medicines are prescribed, relatives are taught the rules of caring for those in need.

Treatment of ataxia involves the use of nootropics, stimulants of blood flow in the brain, drugs for seizures, substances that reduce muscle tone, muscle relaxants and betagestin drugs.

The patient is prescribed massage, gymnastics, occupational therapy, physiotherapy, speech therapy classes, psychotherapy. The doctor will recommend what items will help you adapt to life - canes, beds, and other household items.

Strict follow-up medical recommendations helps slow down the progression of the condition, but it is impossible to completely get rid of congenital cerebellar ataxia. The prognosis is determined by the reasons pathological condition, form of manifestation, age of the patient, vital signs of the body.

Ataxia (incoordination) is a violation of the consistency of action (coordination) of various muscles, which is manifested by a disorder of purposeful movements and static functions. Ataxia is a fairly common motor disorder, with limb strength being completely preserved or quite slightly reduced. Movements become awkward, imprecise, there is a disorder in their sequence and continuity, while walking and standing, balance is disturbed.

To adequately perform absolutely any movement, joint coordinated work is required. large number muscle groups. The movement itself is provided by mechanisms that regulate the choice of appropriate muscle groups, the duration and strength of individual muscle contractions and the sequence of their inclusion directly in the motor act itself. In the absence of coordination of antagonistic and synergistic muscles, a change in the quality of movement occurs with a loss of its proportionality and accuracy. Movements become intermittent, lose their smoothness, become awkward and excessive. The transition between movements (for example, flexion-extension) is significantly more difficult. This incoordination of movements is called locomotor (dynamic) ataxia.

Clinically distinguished the following types ataxia:

→ Cortical (frontal) ataxia – the cortex of the frontal or occipitotemporal region is affected

→ Vestibular ataxia – the vestibular apparatus is affected

→ Cerebellar ataxia – the cerebellum is affected

→ Posterior columnar (sensitive) ataxia – the conduction of deep muscle sensitivity is impaired

Frontal ataxia

This disorder is observed in the case of damage to the frontal lobes of the brain due to damage to the pontofrontal-cerebellar connections. The disease manifests itself most strongly in the limb contralateral to the lesion (leg); characteristic disorders balance, sometimes reaching the degree of astasia - abasia, there is a decrease in intelligence, memory and criticism. Ataxia may develop as a consequence of the following: neurological diseases: abscesses and brain tumors, funicular myelosis, multiple sclerosis, polyneuropathy, idiopathic and hereditary cerebellar ataxias, traumatic brain injuries, stroke, etc. In addition, this genetic disorder can develop with chronic or acute alcohol intoxication, vitamin E deficiency, solvent poisoning, etc. salts of heavy metals, e, when taking certain medicines(diphenin, carbamazepine, etc.)

Vestibular ataxia

This disorder develops when the peripheral vestibular apparatus and manifests itself as balance disturbances in sitting, standing and walking. Vestibular ataxia is manifested by dizziness (a feeling of rotating surrounding objects), which intensifies when moving the head; in some cases, nausea and vomiting are observed, muscle-joint sensation is preserved, there are no disorders in the limbs

Cerebellar ataxia

As the name suggests, this disorder occurs with lesions of the cerebellum, as well as its adductor tracts, and is manifested by ataxia when standing (expressed in widely spaced legs) and walking, dysmetria when performing any precise movements, dysdiadochokinesis (a person cannot quickly change one movement to another ), tremor in the limbs, asynergia (inconsistency of movements). In the cerebellar form of the disease, changes in handwriting (macrography, unevenness), speech in colloquial patterns (“chopped speech”), muscle hypotonia, nystagmus. Damage to the cerebellar vermis leads to ataxia of the trunk, damage to the cerebellar hemisphere leads to ataxia of the homolateral limbs

Sensitive ataxia

This condition develops in case of damage to conductors or receptors of muscle-articular sensitivity at any site in the dorsal roots, brain stem, peripheral nerves, thalamus and posterior columns of the spinal cord. Sensitive ataxia is manifested by disturbances in gait, balance, damage to the limbs (when performing precise movements a person misses), muscle-joint sensation decreases; when closing the eyes, the degree of sensitive ataxia increases significantly (Romberg test). In the case of damage to the posterior columns of the spinal cord, ataxia is manifested by characteristic muscular hyporeflexia and hypotonia; when walking, the patient disproportionately flexes and straightens his legs, while hitting the floor quite hard with his heel (“stamping” gait)

Friedreich's ataxia

This type of disease develops with a frequency of about 5 people per 100,000 population. Most often, the disease develops between 10 and 15 years of age (no later than 25 years of age). Due to damage to the spinal cord and cerebellum, progressive sensory and cerebellar ataxia is characteristic. Skeletal abnormalities (high arches, kyphoscoliosis), mild central paresis of the limbs, and damage to the heart muscle are often observed. For Friedreich's ataxia, the degree of progression varies, but most patients die before they reach thirty years of age

Hereditary idiopathic cerebellar ataxia

This type of ataxia develops in people over 25 years of age, and its genesis is unclear. First type of this disease manifests itself as progressive cerebellar ataxia. The second type is characterized by a combination of ataxia with central paresis, symptoms of damage to the autonomic nervous system (orthostatic hypotension) and basal ganglia

Congenital ataxia

This condition is associated with cerebellar aplasia and is often combined with other congenital anomalies development. The main syndrome is non-progressive cerebellar ataxia. Often in the congenital form there is good development independent walking, motor skills, learning and self-care abilities

Diagnosis of ataxia most often does not cause difficulties and is based on the presence of typical clinical manifestations. The diagnosis is confirmed by the results of an immunological study of cerebrospinal fluid and blood. In addition, CT and MRI of the head are indicated to confirm the diagnosis. If a hereditary nature is suspected, genetic testing is indicated.

The rapidly growing symptoms of the disease are most often caused by a brain abscess, tumor, chronic alcohol intoxication or multiple sclerosis. Acute development ataxia in most cases is caused acute disorders cerebral circulation in the cerebellum (infarction, hemorrhage, etc.). Episodic ataxia most often develops as a result of taking certain medications (diphenin, carbamazepine), with multiple sclerosis, and episodic hereditary ataxia. Slowly progressive ataxia develops in idiopathic and hereditary degenerative forms of the disease and multiple sclerosis.

Treatment of ataxia in most cases is symptomatic and is based on treatment of the underlying disease. Idiopathic degenerative and hereditary forms are not subject to treatment. In addition, important importance in treatment is given to social rehabilitation patient and therapeutic gymnastics complexes.

The prognosis for ataxia is unfavorable. The patient experiences a gradual progression of various neuropsychiatric disorders. In most cases, working capacity is noticeably reduced. And even despite this, in the case of preventing intoxication, injuries, repeated infectious diseases and timely implementation of adequate symptomatic treatment, patients can successfully live to old age.

Prevention of ataxia is to exclude any possibility of consanguineous marriages. In addition, families with diagnosed patients hereditary ataxia, it is not recommended to have children.