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Ataxia cerebellar vestibular frontal sensitive. Possible types of ataxia: causes and manifestations during their development

Ataxia, which translated from Greek means disorder, is considered as a disease associated with an imbalance of voluntary movements and a violation of their coordination. The accuracy of each movement of the muscles of the limbs is the result of the coordinated work of several structures of the nervous system, which transmit impulses to each other in the direction to the muscles of the limbs from the brain and in the opposite direction from the muscle fibers to the brain, which analyzes the correctness of the movement performed. There is a certain sequence in this chain: brain (cerebral cortex, cerebellum) > semicircular canals of the labyrinth of the inner ear (vestibular apparatus) > posterior columns of the spinal cord and peripheral nerves extending from it > nerve fibers penetrating muscle tissue. When the functioning of at least one link of this chain is disrupted, the phenomenon of ataxia occurs. And depending on where exactly the failure occurred, cerebellar, sensitive, frontal, and labyrinthine ataxia are distinguished.

Cerebellar ataxia– this is the result of a violation of the structure and/or functions of the cerebellum;
Frontal (cortical) ataxia– this is the result of a violation of the frontal-cerebellar pathways for processing and transmitting impulses;
Sensitive ataxia– this is the result of damage to the posterior columns of the spinal cord and peripheral nerves extending from it;
Labyrinthine ataxia- this is the result of deviations in the functioning of the vestibular analyzer.

For each of types of ataxia Its clinical manifestations are characteristic:

Symptoms of cerebellar ataxia manifest themselves in the form of hypermetry, when the distance to the object that needs to be taken is poorly calculated, the hand travels a path much greater than necessary and misses. Another sign of cerebellar ataxia is scanned speech, when a person speaks, sometimes loudly, sometimes quietly and sharply “throwing out” words, rather than pronouncing them smoothly, often dividing words into syllables. With cerebellar ataxia, the gait changes: a person walks, swaying and weaving, like a drunk. Problems begin with rising from a “lying” position, when it is impossible to stand up without the help of your hands, because your legs rise instead of your torso. With cerebellar ataxia, the dynamics of complex movements are disrupted, and tremor of the hands appears when they approach the target.

For frontal ataxia(cortical) motor functions and coordination of movements are impaired on the side opposite to the brain hemisphere in which the disorder occurred. Frontal ataxia manifests itself in the form of instability of the body when turning, bending or turning the body. A person is virtually unable to move without relying on any objects. In addition, other functions of the nervous system are disrupted, the sense of smell disappears, and the psyche degrades (a grasping reflex appears).

Symptoms of sensitive ataxia are reflected in a person’s gait when, when walking, he greatly increases the amplitude of the leg swing and walks, throwing his legs to the sides. When in a “standing” position with legs closed and eyes closed, the person’s torso begins to sway in different directions.

With labyrinthine ataxia horizontal nystagmus appears (constant rapid movement of the eyes from side to side), dizziness and many symptoms characteristic of a malfunction of the vestibular analyzer.

Causes of ataxia, if they do not have a genetic origin (or other anomalies in the development of the body), then they are associated with a number of past or in the process of developing diseases, with a lack of vitamins, with injuries, with intoxication of the body with drugs or chemical harmful substances, with infections, with neoplasms located in places where they interfere with the smooth functioning of the nervous system, stroke, with hyperthermia (or sunstroke).

Cause of cerebellar ataxia may become tumors, multiple sclerosis, toxic effects and vitamin deficiencies.

Cause of frontal ataxia There may be injuries, tumors, inflammatory processes.

Cause of sensitive ataxia most often there are injuries or tumors of the spinal cord and its branches, a lack of vitamin B12.

Cause of labyrinthine ataxia chronic ear diseases, encephalitis, Meniere's disease, and multiple sclerosis become common.

Treatment of ataxia– this is the hard work of a sick person and the correct therapy of a neurologist. Therapy is carried out with anticholinesterase drugs, cerebrolysin, ATP, vitamin therapy with drugs containing group B. Sometimes drugs are prescribed to correct immunity. As a rule, it is not ataxia itself that is treated, but the diseases that contribute to its development.

If the cause of ataxia is an operable tumor or an operable aneurysm, then surgical treatment is performed to remove the traumatic factor.

Special sets of exercises aimed at training coordination of movements, skiing, and Nordic walking play a major role in the treatment of ataxia.

The prognosis for treatment of ataxia depends on whether ataxia is hereditary or an abnormal phenomenon, and what specific diseases it is caused by.

How often we walk, perform many actions and don’t even think about how we manage to reproduce these movements with such ease and accuracy. It's all about a very complex mechanism in which various parts of the central nervous system are involved. Most people do not think that there may be any problems or difficulties with the usual maintenance of balance. However, there are a number of diseases that make it difficult to walk straight, stand, or even fully perform standard finger movements. In some patients with these symptoms, doctors diagnose ataxia.

What is ataxia in adults, pregnant women and children

Ataxia is a lack of coordination of movements. The name of the disease comes from the Greek word ataxia - disorder. Patients with this pathology may indeed experience chaotic movements both when walking and when trying to move their fingers, etc. The person begins to complain about the inability to maintain balance and the appearance of awkwardness and inaccuracy while performing any actions. Ataxia can develop at any age, including in young children. In pregnant women, the severity of the disease in some cases may increase, and then additional examinations and more careful monitoring of the functioning of the heart and respiratory system are required.

Coordination is a very delicate process that depends on the coordinated work of certain parts of the central nervous system: the cerebellum, the cortex of the temporal and frontal lobes, the vestibular apparatus and conductors of deep muscle sensitivity. If at least one link of this chain is damaged, a person experiences various disturbances in the coordination of movements of body parts.

Patients with ataxia have poor coordination of movements and sometimes find it difficult to maintain their body in a standing position.

In people with ataxia, there is a divergence in the actions of different muscles, which leads to the impossibility of full coordination. This causes many problems in everyday life, sometimes it becomes almost impossible to go outside and generally exist independently. Sometimes even strength in the upper and lower extremities decreases.

Video about coordination, ataxia and methods of its treatment

Classification of ataxias

Currently, there are several types of ataxia. They differ in causes and symptoms:

Sensitive (posterior columnar) ataxia appears with various disorders in the conductors of deep muscle sensitivity.
Cerebellar ataxia is the most common. It can develop due to various disorders, including genetic ones that are inherited. Cerebellar ataxia Pierre-Marie, benign ataxia Westphal-Leiden, ataxia-telangiectasia (Louis-Bar syndrome) are distinguished.
Vestibular ataxia begins due to damage to one of the departments of the apparatus of the same name.
Cortical, or frontal, ataxia develops with disorders in the temporal and frontal cortex of the brain.
Friedreich's familial ataxia progresses due to mixed cerebellar-sensory lesions.
Spinocerebellar ataxia is a hereditary disease in which multiple degenerative processes occur in the cerebellum, cortex, white matter and many other parts of the brain.
Hysterical (psychogenic) ataxia manifests itself in pretentious and unusual ways of walking. This is a separate species that is not associated with actual damage to brain structures.

There is also a classification according to the types of coordination disorders. If a person has difficulty maintaining balance while standing, they speak of static ataxia. When problems occur during movements and walking, dynamic ataxia is diagnosed.

Very often, ataxia develops due to degenerative processes in the cerebellum

Symptoms and causes of ataxia

Each type of ataxia must be considered separately, since the types of the disease differ greatly in the causes of its occurrence and the symptoms shown. Sometimes doctors need to conduct many examinations and examinations in order to accurately establish the localization of pathological processes and determine the type of ataxia.

Sensitive (posterior columnar) ataxia

This type of ataxia appears due to disturbances in the posterior columns of the spinal cord and nerves, the cortex in the parietal part of the brain. The conductors of deep muscle sensitivity are affected. Patients cease to fully feel and control muscles and joints, and the sense of mass, pressure and position of the body in space is also impaired. It is worth understanding that sensitive ataxia is not a separate disease and it manifests itself as one of the symptoms in all kinds of neurological diseases. The cause of this type of disorder can be benign and malignant neoplasms in the spinal cord, neurosyphilis, injuries and fractures of the spine, multiple sclerosis. In some cases, sensory ataxia may occur after unsuccessful brain surgery.

The symptoms in patients are very obvious; disturbances in coordination of movements are visible to the naked eye. A person cannot walk normally, he bends his knees too much or, conversely, weakly, and sometimes even tries to walk on straight legs. Since it becomes difficult for the patient to feel himself in space, he begins to strongly knock his heels on the floor when moving, because he does not realize the real distance to the surface and his own body weight. Doctors call this gait “stamping.” The patients themselves say that they feel as if they are walking on a soft surface and falling through. To begin to control their gait, they have to constantly look at their feet. As soon as you look away, coordination disappears again. It also becomes difficult to take care of yourself at home, and fine motor skills are impaired. When the patient is at rest, his fingers may move involuntarily and sharply. In some cases, disorders may affect only the upper or only the lower extremities.

Cerebellar ataxia is one of the most common forms of coordination disorder. When a person sits or walks, he or she is found to lean towards the affected area of the cerebellum. If the patient falls to any side and even backwards, then this is a characteristic symptom of a disorder in the cerebellar vermis. People complain about the inability to maintain a normal gait, because they stop assessing the correctness of their movements and feel how they move their legs. Patients feel very insecure, stagger, and place their feet wide apart. Visual control practically does not help maintain balance. Muscle tone may be significantly reduced, especially on the side where the lesions occurred in the cerebellum. In addition to gait disturbances, deviations in the pronunciation of words are also noted. Patients draw out syllables and pronounce phrases slowly. Also a characteristic symptom is sweeping and uneven handwriting.

Patients with cerebellar ataxia often have a writing disorder and it becomes difficult to draw geometric shapes

Cerebellar ataxia can be a consequence of brain injury and a consequence of surgery. Also very often, such a disorder occurs in various types of encephalitis, multiple sclerosis, tumors in the brain, and damage to blood vessels in the spinal cord and cerebellum. Alcoholism and drug addiction can also be causes, in which the entire human body is exposed to serious toxic poisoning.

With cerebellar ataxia, the gait becomes uncertain, the patient places his legs wide apart

Cerebellar ataxia is classified according to the speed of its flow. The disease can be acute (symptoms appear in one day), subacute (symptoms increase over several weeks), chronic (ever progressive) and episodic.

Cerebellar ataxia of Pierre-Marie

This type of ataxia is hereditary. It occurs in a chronic form and constantly progresses. Usually the disease makes itself felt around the age of twenty, most often after thirty. Patients experience degenerative processes in the cerebellum and its conducting tissues. This disease is transmitted in an autosomal dominant manner. This means that children of both sexes can get sick if the defective gene is passed on from at least one of the parents.

With an autosomal dominant type of development, the defective gene can be transmitted from any parent, the chance of having a sick child is 50%

Often, the appearance of Pierre-Marie ataxia can be triggered by head trauma, certain infectious diseases (typhoid and typhus, dysentery, brucellosis, etc.) and even pregnancy. The symptoms of this pathology are very similar to ordinary cerebellar ataxia; they begin with slight disturbances in gait and strange shooting pains in the lower back and lower extremities. Subsequently, trembling of the hands occurs, and the facial muscles begin to contract involuntarily.

A characteristic symptom is visual impairment due to degenerative processes in the optic nerve. Some people begin to gradually develop drooping of the upper eyelid, and the visual field decreases. Patients also experience statistical ataxia, often experience depressive states and even a decrease in intelligence.

Cerebellar ataxia acute Leiden-Westphal

This type of ataxia occurs most often in young children after infectious diseases. This complication appears very quickly, and the course of the disease occurs in acute or subacute form. About two weeks after suffering from influenza, typhus, and some other diseases, the child begins to experience the first signs of damage to the cerebellar structures. Children lose control of coordination when standing and walking. The movements become very sweeping and disproportionate, but babies have difficulty feeling these changes. Also a common symptom is asynergia, in which it becomes impossible to correctly combine muscle movements.

When trying to sit without using his hands, a patient with cerebellar lesions begins to lift his legs up

Ataxia-telangiectasia (Louis-Bar syndrome)

This type of cerebellar ataxia is also hereditary. This disease manifests itself very early, with the first signs appearing in young children around the age of several months. In medicine, Louis-Bar syndrome is classified as a special subtype - phakomatosis - due to genetically determined degenerative processes in the nervous system and skin disorders. It is transmitted in an autosomal recessive manner, can be inherited from any parent and manifests itself in children of both sexes. For the disease to occur, mom and dad must be carriers of the defective gene. Fortunately, this is extremely rare and ataxia-telangiectasia occurs in only one child out of 40 thousand births.

Louis-Bar syndrome is transmitted according to an autosomal recessive mode of inheritance; in children, the disease can manifest itself only if both parents were carriers of the diseased gene

Degenerative processes in the cerebellum and some other parts of the brain are combined with reduced immunity due to a lack of immunoglobulins A and E. Because of this, children often suffer from all kinds of infectious diseases and suffer from cancer, which usually affects the lymphatic system. A characteristic symptom, in combination with ataxia, is the appearance of spider veins (telangiectasia) of varying sizes throughout the body and even on the whites of the eyes.

With Louis-Bar syndrome, patients develop spider veins all over the body, face and even on the whites of the eyes

Vestibular ataxia

The vestibular apparatus is responsible for human coordination and proper movement. Patients feel as if they were circling in one place around their axis for a long time. They stagger, do not maintain their body position well, their eyes twitch involuntarily and quickly, they become dizzy, and may feel nauseous. A characteristic sign is an increase in symptoms when turning the head, eyes and body. It is because of this that patients try to move as carefully, carefully and slowly as possible in order to have time to control changes in the body in space.

This type of ataxia can begin due to damage to any part of the vestibular apparatus, but most often, disorders of the hair cells are detected in the inner ear. These injuries can occur due to otitis media, ear trauma, or tumor formations. The vestibular nerve is also sometimes affected due to various infections and even the use of medications.

The vestibular apparatus has a very complex structure and is responsible for coordination of movements and a sense of self in space.

Cortical or frontal ataxia

Cortical ataxia begins due to lesions in the frontal lobe of the brain. The symptoms are similar to disorders of the cerebellar structures. Some, in addition to uncertainty when walking, experience astasia, in which it is impossible to stand, and abasia, when the patient is unable to walk. Visual control does not help maintain motor coordination. Characteristic symptoms are also revealed that indicate damage to the cortex in the frontal lobes: changes in the psyche, disturbances in the sense of smell, and a decrease in the grasping reflex. The cause of this type of ataxia is various inflammatory diseases, encephalitis, neoplasms in the brain and circulatory disorders.

Spinocerebellar ataxias

There is a whole complex of spinocerebellar ataxias, which are hereditary diseases. Currently, doctors identify more than twenty different species. All of them are transmitted in an autosomal dominant manner, and with each new generation the symptoms and severity of the disease become more pronounced, especially if the defective gene was inherited from the father.

Despite the differences in different types of spinocerebellar ataxias, they all have a similar mechanism of development. Due to an increase in the amount of glutamine in proteins involved in the metabolism of nervous tissue, their structure changes, which leads to disease. The age of first manifestations of the disease varies depending on the type of disease. In some cases, the first symptoms are detected in the preschool years, and in others - after thirty years. Manifestations of ataxia are standard: loss of coordination, deterioration of vision, handwriting, abnormalities in the functioning of internal organs.

Psychogenic or hysterical ataxia

This type is very different from others; it is not associated with organic disorders in the central nervous system. Due to mental disorders, a person’s gait, facial expressions, and pronunciation of words change. The patient begins to perceive himself worse in space. Very often, hysterical ataxia develops in patients with schizophrenia.

Patients with psychogenic ataxia often experience straight-legged walking.

Familial Friedreich's ataxia

This type of ataxia is hereditary and is transmitted in an autosomal recessive manner, very often in consanguineous marriages. Due to a mutation in the gene that encodes the protein frataxin, which transports iron from mitochondria, permanent degenerative damage to the nervous system occurs. The lesion in Friedreich's ataxia is of a mixed nature, cerebellar-sensitive; disturbances in the columns of the spinal cord gradually increase, especially in the Gaulle bundles. The first signs of the disease usually begin to appear before the age of twenty-five.

Friedreich's ataxia can occur in both boys and girls. A distinctive feature is the fact that this disease has not been identified in any person of the Negroid race.

With Friedreich's ataxia, there is a curvature of the foot

The symptoms are similar to other cerebellar ataxias: patients move unsteadily, stagger from side to side. As the disease progresses, it becomes difficult to coordinate the work of the upper and lower extremities, facial muscles and chest. Many people suffering from pathology develop hearing loss. Over time, the following disorders develop:

interruptions in the functioning of the heart, rapid pulse, shortness of breath appear;
kyphoscoliosis, in which deformation of the spine occurs in different planes;
violation of the structure of the foot, it changes its shape, becomes curved;
diabetes;
decreased production of sex hormones;
atrophy of the upper and lower extremities;
dementia;
infantilism.

This type of disease is one of the most common types of ataxia. Occurs in approximately 3–7 people out of one hundred thousand people.

Video about Friedreich's familial ataxia

Diagnosis and differential diagnosis

At the first signs of ataxia, you should consult a neurologist. For further consultation, he can refer you to a geneticist, oncologist, traumatologist, endocrinologist, otolaryngologist and various other specialists.

To study disorders of the vestibular apparatus, the following procedures may be prescribed:

stabilography, in which the patient’s stability is analyzed using an oscilloscope;
vestibulometry is a set of techniques that allows you to evaluate the functioning of the vestibular apparatus;
Electronystagmography, which records eye movements to determine the cause of dizziness and identify disorders of the inner ear.

During vestibulometry, the patient follows the target, and doctors evaluate the accuracy of eye movements and reaction speed

There are also many diagnostic methods that will help clarify the diagnosis and indicate with great accuracy the localization of the pathological process in the central nervous system:

Magnetic resonance imaging (MRI) is the most modern and accurate method. With its help, you can take layer-by-layer images of any organ. In case of ataxia, they help to identify oncological tumors, degenerative processes, developmental anomalies and other abnormalities.
Computed tomography (CT) is a modern radiation method for obtaining layer-by-layer images of internal organs. Contrasting with special liquids can also be performed.
Multislice computed tomography (MSCT) is a very fast scanning method using special sensors that record X-rays passing through the patient's head. Using this type of diagnostics, it is possible to identify tumor formations, inflammatory processes, hemorrhages, and assess the rate of blood circulation.
Dopplerography of cerebral vessels is performed using ultrasound. The rate of blood circulation, vascular patency, intracranial pressure, etc. are assessed.
Ultrasound diagnostics of the brain helps to detect the growth or reduction of cerebellar tissue.
ECG and ultrasound of the heart are necessary for the development of degenerative processes in the heart muscle in the presence of chest pain, rhythm disturbances, etc.

The following tests may also be prescribed:

general blood test;
study of the level of immunoglobulins in the blood (IgA, IgE, IgG);
PCR (polymerase chain reaction, the method is based on repeated doubling of a certain section of DNA using enzymes under artificial conditions) to identify pathological microorganisms;
lumbar puncture (a procedure in which cerebrospinal fluid is taken from the spinal canal using a special needle) to study the cerebrospinal fluid;
DNA diagnostics for detecting hereditary diseases.

To diagnose cerebellar damage, doctors perform a test for asynergia (impaired ability to perform combined movements). To do this, the patient is asked to perform simple movements in which the following disturbances in the combination of muscle action are visible:

when walking, the body leans back, the person falls on his back;
If you begin to tilt your head while standing, your knees will not bend and the patient will lose balance.

During the asynergia test, discrepancies in muscle action are detected in patients with damaged cerebellum

Differential diagnosis must be carried out with various brain tumors, Randu-Osler-Weber disease, Hippel-Lindau disease, funicular myelosis, neurosyphilis, hereditary vitamin E deficiency, multiple sclerosis, Parkinson's disease and many other diseases.

Treatment

The treatment tactics for ataxia depend on its type and the stage of damage to the brain structures. In the initial stages, you can get by with pharmacological drugs; they help slow down degenerative processes. In more advanced cases, the doctor may recommend surgical treatment to the patient.

Drug therapy

In case of ataxia, pharmacological drugs help to stop pathological processes:

Antibacterial treatment is prescribed for infectious lesions (Tetracycline, Ampicillin, Bilmicin).
Vasoactive drugs are necessary for vascular disorders (Parmidin, Trental, Mexicor).
B vitamins are needed to maintain the functioning of the nervous system (Neuromultivitis).
To improve metabolic processes in nerve tissues, the administration of ATP and anticholinesterase drugs (Galantamine, Proserin) is indicated.
Antidepressants are prescribed for a depressed emotional state (Amitriptyline, Citalopram).
Sedatives are necessary in the presence of psychomotor agitation (Magnesium sulfate, Valerian tincture).
Nootropic drugs are prescribed to improve brain function (Phesam, Piracetam).
Metabolic drugs are necessary for Friedreich's ataxia (antioxidants, succinic acid, Riboflavin L-carnitine).
Neuroprotectors are needed to maintain the activity of the nervous system (Pyritinol, Meclofenoxate).
Medicines are prescribed that improve metabolism in the heart (Inosine, Trimetazidine).
Cholinomimetics are necessary to improve the transmission of nerve impulses in neurons (Gliatilin).
Immunostimulants are needed to maintain resistance to infections in patients with Louis-Bar syndrome (Immunoglobulin).

Surgical treatment

Patients do not always require surgical intervention. However, in some situations, conservative treatment does not give the desired results and doctors strongly recommend resorting to surgery:

If tumors, especially malignant ones, are detected, surgical removal is indicated for some patients. Only a neurosurgeon can determine the operability or inoperability of a neoplasm.
If hair cells are damaged, the patient is recommended for cochlear implantation; it helps restore hearing and partially improve coordination.
Middle ear lavage is prescribed for acute and chronic otitis media that have led to vestibular ataxia. Using a special syringe, a liquid containing antibiotics, corticosteroids and other agents is injected into the patient's ear canal.
Sanitation surgery of the middle ear is indicated for cleansing the ear canals and restoring bone tissue.

Physiotherapy and exercise therapy

Stand up, you can leave your arms at your sides or raise them up. Raise your left and right leg alternately and stay in these poses for as long as possible. Repeat the steps, only standing on your toes. To make it more difficult, you can perform the exercise with your eyes closed.
Take a light ball and mark a target on the wall at which you will throw it. It is necessary to practice accuracy, gradually lengthen the distance and use heavier objects.
To develop muscle-joint sense, you need to take objects with your eyes closed and describe their shape and approximate weight.

Various methods of physiotherapy can also be used: ozone therapy, electrophoresis (exposure to the body with direct electric current in combination with the introduction of various medicinal substances through the skin or mucous membranes), myostimulation (exposure to the body of current through special electrodes that are applied to the body).

Video about therapeutic exercises for ataxia

Folk remedies

Ataxia is a very serious condition and cannot be treated on its own. In most cases, it is not possible to achieve recovery using only folk remedies. But they can be prescribed after consulting a doctor as an auxiliary method. It is possible to use various herbs that help strengthen the nervous system:

infusion of 3 teaspoons of peony root;
infusion from tsp. chamomile, lemon balm and oregano flowers;
infusion of half a glass of birch leaves, 3 tsp. chamomile flowers, spoons of honey.

All components must be poured with a glass of boiling water and left to steep for about two hours. Peony is taken 1 tablespoon 4 times a day, other infusions - 150 ml 3 times a day before meals.

Treatment prognosis

Doctors talk about a favorable prognosis for the treatment of ataxia if drug therapy or surgical intervention stops the development of degenerative processes and eliminates disturbances in the functioning of the nervous system. If the cause of the disease is genetic or a malignant tumor has been discovered, the prognosis is usually unfavorable. In this case, specialists try to use treatment to stop the progression of the pathology and maintain the patient’s motor activity. Louis-Bar syndrome has a poor prognosis; children with this disease very rarely survive to adulthood. With Friedreich's ataxia, the prognosis is relatively favorable, many patients live more than twenty years from the onset of the first symptoms, especially if there is no damage to the heart muscle and diabetes. It is impossible to completely recover from genetic varieties of ataxia.

It is not always possible to become pregnant and give birth to a child. There may be contraindications that can lead to danger and even death during childbirth. It is very important to consult with doctors in advance before planning the birth of your baby.

Very often, patients with different types of ataxia experience the following complications:

paralysis and paresis (weakening of motor activity) of the limbs;
deterioration of vision and hearing;
respiratory and heart failure;
frequent relapses of infectious diseases;
loss of the ability to move independently and take care of oneself;
lethal outcome.

Prevention

If future parents are suspected of having hereditary forms of ataxia, they should be examined by a geneticist to find out the risk of having a sick child. During 8–12 weeks of pregnancy, chorionic villi (the outer membrane of the embryo) can be tested to identify the presence of defective genes in the fetus. Consanguineous marriages should be avoided, as children may develop multiple genetic diseases.

It is also very important to maintain your health, you should eliminate bad habits, promptly treat infectious diseases and try to prevent all kinds of head and spine injuries.

Ataxia is a very serious diagnosis, which often develops dangerous complications. At the first signs of a violation of the coordination of movements, you should consult a doctor. Remember that many types of coordination disorders can be prevented if treatment is started in a timely manner. Unfortunately, inherited ataxias almost always progress and often lead to disability and even death. When planning a pregnancy, you need to find out about cases of such diseases from your closest relatives or contact a geneticist for advice.

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 of the 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 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 treatments.

Fast Facts

Ataxia is caused by a wide range of 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 .

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:

lethargy;
lack of coordination between organs, muscles, limbs, joints;
weakened ability to control distance, power, speed of movement of arms, legs, eyes;
it is difficult to estimate exactly how much time has passed;
inability to perform fast, alternating movements;

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 neighboring 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 with your eyes closed and your feet together, the instability will get worse. 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 neuromuscular motor disorder characterized by impaired coordination of movements, as well as loss of balance both at rest and when walking. Inconsistency in the actions of various muscles can arise as a result of damage to certain parts of the brain or vestibular apparatus, which is sometimes due to genetic predisposition. Treatment of ataxia and the prognosis of its development depend on the cause of the disease.

Types of ataxia

In clinical practice, the following types of ataxia are distinguished:

Sensitive;
Vestibular;
Cortical or frontal;
Cerebellar.

With sensitive ataxia, deep sensitivity fibers are damaged, which carry information about the features of the surrounding space and the position of the body in it. The cause may be damage to the posterior columns of the spinal cord, thalamus or spinal nerves, as well as polyneuropathy and vitamin B12 deficiency.

Upon examination, the following symptoms of sensitive ataxia are revealed:

Dependence of coordination on visual control;
Violation of vibration and joint-muscular sensitivity;
Losing balance with eyes closed in Romberg pose;
Loss or decrease in tendon reflexes;
Unsteady gait.

A characteristic sign of sensory ataxia is the sensation of walking on carpet or cotton wool. In order to compensate for movement disorders, patients constantly look at their feet, and also lift their legs high and strongly bend their legs at the knee and hip joints, and then forcefully lower them to the floor with the entire sole.

In vestibular ataxia, dysfunction of the vestibular apparatus leads to specific gait disturbances, systemic dizziness, nausea and vomiting. All symptoms intensify with sudden turns of the head and changes in body position. Possible hearing impairment and horizontal nystagmus - involuntary movements of the eyeballs. This type of disease can be caused by brainstem encephalitis, ear diseases, tumors of the brain ventricles and Meniere's syndrome.

Cortical ataxia is caused by dysfunction of the frontal lobe of the brain as a result of dysfunction of the fronto-pontine-cerebellar system. The cause may be improper cerebral circulation, tumors or abscesses.

Frontal ataxia occurs on the side of the body opposite the affected hemisphere. Instability, bending or falling over begin at turns, and with severe injuries, patients are not able to stand or walk at all. This coordination disorder is also characterized by disturbances in the sense of smell, mental changes and a pronounced grasping reflex.

Features of cerebellar ataxia are loss of fluency of speech, tremors of various types, muscle hypotonia and oculomotor dysfunction. The gait also has characteristic features: patients spread their legs widely and sway from side to side. In the Romberg position there is extreme instability, often falling backward. A severe lack of coordination of movements occurs during tandem walking, when the heel of one leg is placed against the toe of the other. Cerebellar ataxia can be caused by a wide range of diseases - from vitamin deficiency and drug intoxication to a malignant tumor.

Hereditary degenerative changes in the cerebellum cause spinocerebellar ataxia - chronic diseases of a progressive nature, which can be of a dominant or recessive type.

The autosomal dominant cerebellar form of the disease is often accompanied by the following symptoms:

Tremor;
Hyperreflexia;
Amyotrophy;
Ophthalmoplegia;
Pelvic disorders.

The pathological sign of Pierre Marie's ataxia is cerebellar hypoplasia, less often - atrophy of the inferior olives and pons. The first gait disturbances begin, on average, at 35 years of age. Subsequently, disturbances in facial expressions and speech are added. Mental disorders manifest themselves in the form of depression and decreased intelligence.

Autosomal recessive spinocerebellar ataxia is associated with the following symptoms:

Areflexia;
Dysarthria;
Hypertonicity of muscles;
Scoliosis;
Cardiomyopathy;
Diabetes mellitus.

Friedreich's familial ataxia occurs due to damage to the spinal systems, most often as a result of consanguineous marriage. The main pathological symptom is increasing degeneration of the posterior and lateral columns of the spinal cord. At about 15 years of age, unsteadiness when walking and frequent falls appear. Over time, skeletal changes cause a tendency to frequent joint dislocations and kyphoscoliosis. The heart suffers - the atrial waves are deformed, the heart rhythm is disturbed. After any physical exertion, shortness of breath and paroxysmal pain in the heart begin.

Diagnosis and treatment of ataxia

For cerebellar ataxia, the following studies are performed:

EEG. Reveals reduction of alpha rhythm and diffuse delta and theta activity;
MRI. Carry out to detect atrophy of the brain stems and spinal cord;
Electromyography. Shows axonal demyelinating damage to peripheral nerve fibers;
Laboratory tests. Allows observation of amino acid metabolism disorders;
DNA test. Establishes a genetic predisposition to ataxia.

Treatment of ataxia is aimed at eliminating symptoms. It is carried out by a neurologist and includes:

General restorative therapy - anticholinesterase drugs, cerebrolysin, ATP, B vitamins;
Exercise therapy complex – strengthens muscles and reduces incoordination.

When treating spinocerebellar ataxia, a course of immunoglobulin may be required to correct immunodeficiency, and any radiation is contraindicated. Sometimes succinic acid, riboflavin, vitamin E and other drugs are prescribed to maintain mitochondrial function.

The prognosis for hereditary ataxia is unfavorable. Working capacity, as a rule, decreases, and mental disorders progress.

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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 disease 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 there?

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 appear against the background of encephalitis or paralysis, cancer or an abscess. If a patient suffers from epilepsy in childhood, 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 brain stem 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, when sensitive, the legs suffer, primarily the muscles and joints. 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 in order to compensate for the lack of activity of the motor 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 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 abnormalities. 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 the strength of the muscles of the lower extremities 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 by a high rate of 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 number of additional studies are done. The patient is examined by an ophthalmologist, neurologist, and psychiatrist. Laboratory tests can identify metabolic problems.

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, lowering pressure levels in the posterior fossa of the skull are indicated.

Treatment of ataxia involves the practice of gymnastic exercises, a complex of which is developed based on the patient’s condition. The main goal of gymnastics is to strengthen muscle tissue and ease coordination problems. The patient is prescribed general tonics, vitamins, and 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 a lack of functioning of the respiratory system and heart in a chronic form, and the 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 and 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. With the rapid development of the disease, the prognosis 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 can be explained by a gene mutation. At present there is no detailed explanation of the causes of the processes.

Types and forms: cerebellar

The hereditary disease 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. Late form 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

The clinical picture of the progressive form of the disease is specific, so it is usually easy to establish an accurate diagnosis. The disease can be assumed based on the general symptoms, the patient’s behavior, and 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, and psychotherapy. The doctor will recommend what items will help you adapt to life - canes, beds, and other household items.

Strict adherence to medical recommendations helps to slow down the progress of the condition, but it is impossible to completely get rid of congenital cerebellar ataxia. The prognosis is determined by the causes of the pathological condition, the form of manifestation, the patient’s age, and the body’s vital signs.