Cerebellar disorders: causes, symptoms, signs, treatment. Cerebellar ataxia: causes, clinical picture and treatment methods

The structure is complex and divided into several parts. One of them is called the flocculonodular lobe. This part receives all signals from the vestibular nuclei. Responsible for maintaining balance. The other lobe (anterior or paleocerebellum) is responsible for body position, gait and muscle tone. The posterior lobe coordinates voluntary movements. Damage to any of the lobes leads to dysfunction. Treatment of cerebellar ataxia must be comprehensive to restore motor activity.

Causes of cerebellar ataxia

The nature of the development of pathology depends on the cause of ataxia. In cases of alcohol or barbiturate poisoning, or intoxication with certain drugs (anticonvulsants), acute disorders occur. Reversible processes appear with a viral infection. Reasons for persistent acute disorders I can be heat, coma, inhalation of vapors of certain substances (glue, dyes).

There are subacute forms that do not go away within several weeks. The causes of such ataxias can be:

(medulloblastoma, astrocytoma, hemangioblastoma);
carcinoma of the mammary glands and ovaries;
alcohol intoxication;
food poisoning;
brain abscess;
Creutzfeldt-Jakob disease.

Chronic cerebellar ataxia can bother the patient for several months or years. The reasons are:

hereditary ataxias (Friedreich; cerebellar ataxia of Pierre-Marie);
degenerative changes in the brain;
hereditary metabolic diseases.

Characteristic symptoms

The main symptom of ataxia is a characteristic oscillation of the fingers when approaching a target. When trying to hit an object, your fingers begin to oscillate from side to side. This symptom is called “atoxic tremor.” Other characteristic symptoms include “rub tremor.”

When a patient tries to hold a certain position, high-amplitude tremor begins to appear in the limbs. When performing any complex sequential actions, a person cannot perform them accurately and smoothly.

During diagnosis and examination, the doctor can determine the patient’s lack of movement. The patient carries out all commands intermittently, with excessive speed or slowness, and cannot immediately pick up an object, write or draw a line of a given length. Other signs of pathology include:

inability to perform rapidly alternating movements;
inability to sit on a chair without using your hands;
speech impairment (slow, drawn out, chanting of words);
involuntary rhythmic movements of the eyeballs (nystagmus);
handwriting disorder.

With acute cerebellar dysfunction, other symptoms may appear:

weakness;
rapid fatigue in the arms and legs;
gait disturbance.

The following types of ataxia are distinguished:

Static.
Static-locomotor.
Dynamic.

As a rule, hereditary forms of cerebral ataxia manifest themselves in early age. Patients except characteristic symptoms have various pathologies. At hereditary ataxia Friedreich in patients is diagnosed with a combined lesion nervous system, heart, pancreas, eyes. In half of the cases, patients die from myocardial dystrophy. Hereditary pathology Pierre Marie is combined with an increase in tendon reflexes and a decrease in intelligence of varying degrees of severity.

Diagnostics

Before treating ataxia, patients undergo a comprehensive diagnosis. Patients are prescribed CT, vascular Dopplerography. In families where cases of ataxia have been reported, prenatal DNA diagnosis is indicated.

In case of static or static-locomotor ataxia in a patient, the doctor can immediately note a gait disturbance. The patient spreads his legs wide when walking, staggers from side to side, and cannot stand straight. With extensive lesions of the cerebellum, the patient falls. This type of walking is reminiscent of walking while intoxicated.

In order to find out the cause of cerebellar damage, consultations are indicated various specialists(infectious disease specialists, traumatologists, oncologists, geneticists, etc.). If an infectious process is suspected, blood and cerebrospinal fluid tests are prescribed.

Treatment

Treatment of cerebellar ataxia begins with eliminating the cause of the pathology. For infectious processes, antibacterial or antiviral drugs. For pathology vascular system brain, agents that improve the rheological properties of blood and angioprotectors are indicated. If the cause of the disorders is poisoning, patients are prescribed detoxification therapy.

In the case of hereditary genesis of the disease, drugs that improve metabolic processes are indicated (Riboflavin, succinic acid, Nicotinamide, Tocopherol, Idebenone, Mildronate, ATP). Patients are prescribed drugs that improve trophic processes in the brain (Piracetam, Cerebrolysin, Nootropil).

Surgical treatment is used when diagnosing tumors, cysts, etc. in a patient. Besides drug therapy, massage and physical therapy are indicated to improve muscle strength.

Forecast

The prognosis depends on the cause that caused cerebellar ataxia. An unfavorable prognosis for the patient in the case of genetic ataxia or inoperable brain tumors. If the consequences of cerebral ischemia or the toxic effects of drugs, alcohol, etc. are promptly eliminated, coordination can be restored completely or partially.

IN Lately Increasingly, such a phenomenon as the genetic nature of the development of the disease has begun to appear, or in other words, the patient’s inability to influence the development of the disease on initial stage. Ataxia also belongs to similar diseases, but what is ataxia and what types of this disease occur, we will consider in this article.

Ataxia is characterized by a violation of human motor skills and is a neuromuscular type of disease. The diagnosis of ataxia is made by a neurologist, since this disease relates to diseases of the nervous system. During its development, the patient’s central nervous system is affected; there may be some stiffness in movements, straining when walking and dependence of movements on visual contact, and impaired coordination of movements.

At the same time, the muscles do not lose their strength, or lose it only slightly.

The reasons for the development of this disease are associated primarily with genetic nature. In addition, there is reason to believe that the formation of such a disease is influenced by a deficiency of vitamin B12 in the body.

In addition, the reasons include:

inflammatory processes in the body ();
drug use;
conversion disorders;
alcohol abuse;
the presence of infection in the body or against the background of an infectious disease;
presence of tumor processes;
disruptions in the endocrine system.

Many experts believe that violations of this kind arise not as independent species illness, but as a consequence of a previous or existing disease. That is, ataxia is one symptom of some more severe and dangerous disease, which the patient is not yet aware of.

Classification of ataxia and its symptoms

The symptoms of ataxia vary greatly depending on the type of disease. Since when a particular organ is damaged, individual manifestations are observed. This disease has the following subtypes:

Cerebellar.
Vestibular.
Cortical.

Determination of ataxia by gait

In addition to the above subtypes of the disease, there are several more of its varieties, which one way or another can be classified as one of the main types, but have some features that make it possible to distinguish them into a separate group, including:

cerebellar ataxia of Pierre-Marie;
familial Friedreich's ataxia (Friedreich's hereditary ataxia);
telangiectasia ().

There are several more subspecies that will not be discussed in detail in this article, including:

Optical is a disease that occurs as a result of damage to the posterior parietal cortex of the brain. It is characterized by the inability of a person to touch any object within sight due to inconsistency of hand movements.

Intrapsychic ataxia is part of schizophrenia and is a disorder of the individual’s psyche.

Despite the rich classification of this disease, it has general symptoms, and the first symptoms include:

violation of movement coordination;
nausea;
vomit;
involuntary muscle twitching;
change in handwriting;
slow speech;
blurred vision;
changes in mental state;
presence of hallucinations (auditory, visual);
memory impairment;
violation of facial expressions.

Often the symptoms of this disease are similar to those that occur with neurosis, as a result of which many people confuse the first signs of the disease. In any case, if you suspect any deviations in the behavior of a potential patient, there is no need to delay, but rather consult a doctor as soon as possible.

Sensitive ataxia is the most common disease and develops as a result of damage to the posterior columns of the spinal cord, namely the pathways located there.

There are several types (severities) of the disease:

damage to the upper extremities with loss of coordination of movement;
damage to the lower extremities with loss of coordination and sensitivity in this area of the body;
damage to one of the limbs, with loss of coordination and sensitivity;
damage to all limbs;
loss of coordination of movement of the whole body (the case when the brain cannot determine how relatively earth's surface it is located, as well as how the arms and legs are positioned relative to each other).

If this type of disease develops, a person may experience imbalances, since it is often the lower limbs. Walking becomes visually dependent (the lack of visual contact with the area where the foot will be placed makes movement extremely difficult). The patient may feel that walking resembles walking on thick cardboard or cotton wool. The foot is placed on the floor with characteristic pressure on the foot.

Damaged spinal cord stem

In addition, the patient is unable to maintain balance in a position with his arms and legs spread to the sides.

When testing the patient using the Romberg method, the result is negative.

The Romberg test is a method of determining movement coordination, which is expressed in observing the patient when he is in a position specified by the doctor. The usual Romberg pose is legs out to the sides, arms forward, fingers spread. Difficult pose - Legs stand in one line, with the heel of one leg touching the toe of the other, arms extended forward, fingers spread. The average time spent in this position with eyes closed ranges from 30 to 50 seconds without loss of coordination.

The more severe the illness, the worse the ability to walk. At the most difficult stages, this opportunity may be completely lost.

Cerebellar

Cerebellar ataxia (sometimes called truncal ataxia) belongs to the second large subtype of this disease. The main difference between the disease and the sensitive type is that when the cerebellum is damaged, the symptoms spread to all limbs and the body as a whole, while with the sensitive type of the disease only one limb can be affected.

Main symptoms of this disease the following:

change in gait, inability to stand straight;
imbalance;
tremor of the limbs;
tense pronunciation of words with their noticeable separation;
eye twitching.

The reasons for the development of this disease are:

poisoning medications(abuse or excessive long-term use the same medicine)
cerebellar stroke ( this phenomenon dangerous not only by the risk of ataxia, but also by the risk of death);
infectious diseases (encephalitis, chickenpox, etc.);
children's cerebral paralysis;
traumatic brain injuries.

The patient and the people around him will definitely notice that something is wrong with the patient. For example, the patient’s gait will resemble a pendulum. He will walk on straight legs, swaying from side to side. When standing still with legs wide apart, if you sharply push one of the legs, the patient will fall and not even notice it.

During turns while walking, the patient may even fall.

Gradually, the patient ceases to understand in what order it is necessary to rearrange his legs and move his arms when walking. A kind of mask freezes on the face, which is why the patient looks like a drunk person. Speech slows down, handwriting deteriorates, strabismus may develop and visual acuity may deteriorate. Gradually developing mental disorders and depression.

In children, this disease can develop after 3 years, but the main age ranges from one to three years.

Cerebellar ataxia of Pierre-Marie

This disease is one of the subtypes of cerebellar ataxia and is a hereditary disease.
The likelihood of the disease among the patient's first-degree relatives is quite high. The disease is progressive and difficult to diagnose in the early stages of development.

The first manifestations of the disease are visualized by others at the age of 35–40 years. The symptoms are the same as with ordinary cerebellar ataxia.

The main difference is the cause of development - cerebellar hypoplasia.

Vestibular

This type of disease is a direct continuation of the underlying disease. That is, vestibular ataxia does not occur independently, but only as an accompanying symptom.

The main manifestations of this disease are as follows:

impaired coordination of movement and rest (standing or sitting);
nausea;
vomit;
nystagmus;

Nystagmus - involuntary movement eye (twitching)

sleep disturbance (dizziness may bother the patient when lying down);
dizziness;
autonomic disorders (pallor or redness, tachycardia, pulse instability).

The main reasons for the development of the vestibular type of disease are the following:

damage to hair cells as a result of inflammatory processes during inner ear(otitis, trauma, aerootitis, tumor, cholesteatoma of the ear)

Hair cells are auditory receptors

Cholesteatoma is a tumor neoplasm containing capsule-shaped growths

defeat vestibular nerve(tumor, infectious disease, toxic poisoning)
damage to the vestibular nuclei in the medulla oblongata (tumor, encephalitis, arachnoiditis, multiple sclerosis, atherosclerosis)
traumatic brain injury

The main difference between vestibular disease and others is the dependence of symptoms on turning the head or body. Usually, these actions cause maximum inconvenience to the patient, for this reason he tries to perform them smoothly and without sudden movements; in addition, with his eyes closed, he feels a greater lack of coordination of movement.

Since hair cells are usually affected on one side of the body, the patient will also be “skewed” to this side.

Cortical

Cortical or frontal ataxia is one of the varieties of the disease, which in many symptoms is similar to cerebellar ataxia, but also has its own characteristics.

Some scientific facts about the disease

The main symptoms of the progression of this disease, in addition to the general ones, are the following:

development of the grasping reflex;
mental change;
impaired sense of smell;
falling backwards when walking;
instability.

A patient with a cortical type of illness moves along one line with a constant deviation of the body backwards.

The cause of the disease is damage to the frontal lobe of the brain as a result of:

tumors;
abscesses;
circulatory disorders;
stroke.

Friedreich's ataxia

Friedreich's ataxia is hereditary disease, which has almost all the symptoms characteristic of ataxia. The main difference between this disease and the others is the route of its transmission - heredity.

Friedreich's ataxia can be of both cerebellar and sensitive types. The disease develops from 10 to 20 years, there is a possibility of later manifestation, but in rare cases.

The main symptoms are as follows:

unsteady gait;
stumbling and falling when walking;
uncertainty when moving;
hand tremors;
change in handwriting;
hearing loss;
deterioration and slowing of speech;
loss of ability to care for oneself;
development of dementia;
disorders of the nervous system (arrhythmia, formation of “Friedreich’s foot”, disorders in the endocrine system).

This is what Friedreich's foot looks like

The causes of the disease are mutations that lead to the accumulation of toxic substances and heavy metals in the body. This, in turn, leads to damage to neurons, cells of the pancreas and heart, as well as the muscles of the eye, etc. All these processes, one way or another, lead to the development of Friedreich's ataxia.

The dynamic nature of cerebellar ataxia

In modern neurology, two types of ataxia are distinguished according to the nature of its manifestation - dynamic and static

As a rule, most often you can come across such a thing as dynamic cerebellar ataxia, which manifests itself, as it became clear, with damage to the cerebellum.

Its main difference from the static one is that all violations, one way or another, are associated with the movement of the patient.

It is believed that the dynamic type of the disease is the most severe form of the disease, since the patient is not able to move independently.

Static nature of cerebellar ataxia

As for the static type of this disease, or as it is sometimes called static locomotor disease. In this case, the severity of the disease is lower than in the dynamic case. The basis of the symptoms are manifestations of imbalance when the patient’s body is positioned at rest (standing, sitting and even lying down).

Diagnosis of ataxia

In order to correctly diagnose and differentiate different types disease applies comprehensive diagnostics, which includes several stages.

So, at the very first stage, the doctor needs to determine what type of disease the disease belongs to, based on the type of course. For this purpose, a special neurological classification is used:

Spicy.
Subacute.
Progressive.
Episodic.

Depending on the underlying disease and the nature of the symptoms, one can draw conclusions about one or another type of illness. There may be several options, so the doctor never relies only on the results of any one study.

After the type of course is established, the doctor begins to deal with the type of illness itself, from the above classification. As a rule, complex neurological tests are used for this:

Romberg pose;
finger tests;
knee-heel tests;
hand lowering test;
reverse shock symptom;
pay attention to the presence of Burdzinski syndrome.

In addition to studying the patient’s medical history and conducting various tests, instrumental studies are prescribed. Including:

magnetic resonance imaging;
general and clinical analysis blood;
CT scan;
Ultrasound of the brain;
cerebrospinal fluid analysis.

Depending on the severity of the diagnosis, additional tests may be prescribed.

Treatment and prognosis of ataxia

To treat such a disease, it is important not to delay, as it progresses and can develop into a more severe form.
Since the main cause of the disease is a concomitant disease, ataxia is treated symptomatically, and the main therapy is already directed to the disease that caused this symptom.

In most cases, it is not worth saying that the disease is curable; perhaps, it can only reduce the symptoms and make the person’s future life easier. However, this does not mean that it is impossible to completely get rid of it. How to cure an unpleasant illness forever?

First of all, by completely curing the underlying disease. However, if the development of the disease is advanced, the chances of getting rid of ataxia forever are slim.

massotherapy;
medicinal Physical Culture(physical therapy);
application vitamin complex(vitamin E, coenzine Q10);
use of riboflavin and succinic acid.

The prognosis is highly dependent on the severity of the disease. In severe forms, it is possible to limit the patient’s work activity; in the case of a favorable outcome, there is a high probability that patients will survive to a ripe old age. At mild form and exceptions re-development infectious diseases, it is possible to get rid of the disease completely.

Treatment of this disease folk remedies, and even without the supervision of a specialist, this is an extremely unreasonable decision and our editors are strongly against such decisions.

Exercise therapy

Exercises for ataxia play an important role in the treatment of the disease, which can be divided into several blocks:

General strengthening (turns, swings of limbs).
Developing accuracy (the main condition is to hit the indicated plane with your hand or foot, gradually the complexity of the tasks increases, loads are added).
Developing coherence in the joints (consists in setting tasks for lifting or moving various objects, and joints should be involved in this work; it is gradually possible to use weights during these exercises).
To stimulate ballistics (throwing, pushing or simulating such activity, the throwing distance increases each time).
To improve balance (exercises to improve balance with the help of additional movements, standing, sitting and walking, you can gradually introduce the use of parallel bars for these purposes).
Training the eye muscles (Fixation with the eyes of a certain point, which is followed during head tilts or turns, as well as in movement).
To develop muscle-joint sense (guessing an object by sensations with eyes closed).

There is an opinion that it is possible to use such a device as a balancer for children, however, its use can only be prescribed by a doctor; in no case do you self-medicate, as you can injure the baby.

Prevention

Prevention is understood as a set of measures that prevent further spread of the disease and prevent its occurrence in healthy people.

exclusion of consanguineous marriages;
exclusion of pregnancy in families with ataxia;
timely treatment infectious diseases;
preventing the accumulation of toxic and heavy metals in the body;
conducting healthy image life;
compliance with sleep and nutrition;
timely contact a specialist for help.

So, ataxia is a serious concomitant disease that requires mandatory medical intervention. The longer a patient delays contacting a specialist, the less likely he is to fully recover from such an illness; moreover, the underlying disease may even kill him. Take care of yourself, do not self-medicate and get treatment from the right doctors!

Shoshina Vera Nikolaevna

Therapist, education: Northern medical University. Work experience 10 years.

Articles written

Cerebellar ataxia is characterized by impaired coordination and motor functions. In this case, the patient’s movements become awkward, and difficulties arise with maintaining balance and walking. This problem comes in many forms and can occur with a variety of medical conditions.

The cerebellum performs the following functions:

Controls the coordination of movements, their smoothness and proportionality.
Maintains body balance.
Regulates muscle tone and ensures muscles perform their functions.
Provides center of gravity.
Synchronizes movements.
Has anti-gravity properties.

All these functions are very important for normal human life. If violations occur in their implementation, and signs of these violations appear, then this condition is called cerebellar syndrome. It leads to disturbances in muscle tone and motor function, which negatively affects a person’s health and life. Ataxia is considered part of this syndrome.

The disease can develop as a result of:

poisoning with drugs containing lithium, antiepileptic drugs, benzodiazepine drugs, and toxins. The person suffers from drowsiness and confusion;
. It may occur due to a blockage in the brain;
infarction in the medulla oblongata with Horner's syndrome;
infectious processes. Ataxia is often a consequence of encephalitis and abscess;
transferred viral infection. Most often, cerebellar ataxia develops in children for this reason. Acute form this pathology may occur after chickenpox. The outcome in this case will be favorable. After several months of treatment, the patient's condition is completely restored.

In addition, the development of the pathological process is facilitated by neoplasms in the brain, cerebral palsy, multiple sclerosis, and insufficient intake of vitamin B12.

Similar problems can arise as a result of genetic pathologies. This usually occurs in Fredreich spinal ataxia and Pierre Marie cerebellar ataxia. The latter disease affects people in middle and old age. Although it develops late, it proceeds quickly and is accompanied by impaired speech functions and increased tendon reflexes.

At first, the pathological process occurs with nystagmus, after which there is a loss of coordination in the upper extremities, deep reflexes are revived, and muscle tone increases. If they atrophy optic nerves, then the prognosis will be unfavorable.

In addition, with such ataxia, not only does gait worsen, but problems with memory and intellect also arise; the person cannot control emotions and will. Complications develop quite quickly, so the prognosis is unfavorable.

This type of hereditary ataxia often cannot be distinguished from a neoplasm in the posterior cranial fossa. But since there are no stagnant processes in the fundus of the eye, and intracranial pressure does not increase, these signs can be used to make a correct diagnosis.

Manifestations of cerebellar ataxia

Ataxia has quite specific symptoms that are difficult to miss. As the disease progresses, the following is observed:

Sweeping and uncertain movements, which often leads to a person falling.
Unsteadiness of gait. The patient cannot walk in a straight line; due to instability, he spreads his legs wide and balances with his hands.
The motor act stops earlier than the person planned.
The body sways from side to side, the patient cannot stand straight.
When moving, the hands begin to tremble, although this symptom is absent when the patient is at rest.
The eyeballs twitch involuntarily.
A person cannot quickly perform opposite movements.
Handwriting changes. In this case, the letters will be large, sweeping, and uneven.
Speech function is impaired. The patient begins to speak at a slow pace with long pauses between words, placing emphasis on each syllable.
Weakness occurs in the muscles and deep reflexes decrease. This symptom is more pronounced if a person tries to move quickly, stands up suddenly or changes the direction of movements.

Depending on the nature of the symptoms, the disease can be:

static. When manifestations of ataxia occur at rest, and the patient cannot be in vertical position;
dynamic. In this case, disorders occur during movement.

How to make a diagnosis

There are no difficulties in diagnosing this pathological process. The specialist examines the patient and conducts functional tests to assess the degree of impairment and determine the type of ataxia. In addition, diagnostics includes instrumental studies. They allow us to identify pathological changes in the cerebellum, congenital anomalies, degenerative disorders and other negative processes. This information can be obtained using:

Vestibulometry.
Electronystagmography.
Magnetic resonance and tomography.
Dopplerography of cerebral vessels.
Angiographic examination.

In addition, the patient must take a blood test and undergo a polymerase test. chain reaction. A lumbar puncture may be ordered, during which a sample of cerebrospinal fluid is taken to check for infection or hemorrhage.

To determine whether ataxia is hereditary, a DNA test can be performed. This procedure is usually performed to determine the risk of developing ataxia in a child if there have been cases of such disorders in the family.

Treatment methods

Treatment of cerebellar ataxia can begin only if the cause of the disorders has been identified. If it is not eliminated, then all therapeutic measures will not produce results. Treatment will vary depending on the underlying cause. Common features It has symptomatic treatment. To alleviate the patient’s condition and eliminate the main manifestations of the disease, therapy begins with the use of:

antioxidants and in:
means to improve blood circulation in the brain;
betahistines;
vitamin complexes containing B vitamins;
medications to improve muscle tone;
anticonvulsants.

If a person is diagnosed with an infectious-inflammatory disease, then antibacterial and antivirals. In the presence of vascular disorders, one cannot do without thrombolytics, antiplatelet agents, angioprotectors, anticoagulants, etc. They are necessary to normalize the blood circulation process.

If cerebellar dysfunction occurs as a result of poisoning with toxic substances, detoxification therapy is necessary, which includes intensive infusion therapy, the use of diuretics and hemosorption.

If ataxia is genetic, do not use radical methods treatment. The patient's condition is stabilized with the help of drugs to improve metabolic processes:

B vitamins;
adenosine triphosphate;
Meldonia;
Piracetam and Ginkgo Biloba.

If the disorders were caused by a tumor in the brain, then without surgical intervention it’s impossible to get by. Malignant cells are destroyed using radiation and chemotherapy. Different procedures may be prescribed depending on the type of tumor. If the tumor is eliminated successfully, you can expect a partial or complete recovery.

Regardless of the treatment method, all patients with ataxia are advised to undergo physiotherapy and therapeutic massage. Thanks to these procedures, atrophic changes in muscle tissue and the development of contractures can be avoided. If you do these exercises regularly, you can improve your coordination and gait, as well as keep your muscles toned.

A set of gymnastic exercises is also prescribed. It helps reduce coordination problems and strengthens muscle tissue.

Cerebellar ataxia is dangerous not only because it significantly worsens a person’s quality of life. These violations can lead to the development of serious complications. Among them:

increased tendency to develop the same infectious diseases;
chronic heart failure;
respiratory dysfunction.

The prognosis for this pathological process depends on what caused the disorders. If the acute and subacute forms of the disease, which were provoked by toxin poisoning, inflammatory or infectious process, vascular disorders, then you can completely restore the functioning of the cerebellum. But very often, compliance with all therapeutic measures does not lead to recovery. The disease tends to progress rapidly, especially if it is late cerebellar ataxia.

As a result of disorders caused by ataxia, a person’s quality of life is significantly reduced, and serious disturbances in the functioning of all organs and systems develop. Therefore, it is very important to consult a specialist at the first manifestations of the disease and undergo an examination. If the diagnosis is made on time and the treatment is selected correctly, then there are higher chances that motor functions will be restored or the development of the pathological process will slow down than if a person consults a doctor for late stage development of the disease.

Especially severe course Ataxia, which arose under the influence of genetic factors, is different. In this case, the disease takes on a chronic form, symptoms gradually increase, and the patient’s condition worsens. Gradually the person becomes disabled.

Prevention

There are no preventive measures that would avoid the development of ataxia. To prevent damage to the cerebellum, it is necessary to avoid trauma, poisoning, infectious diseases, and monitor the condition of the blood vessels. At the first signs of development similar diseases– treat them in a timely manner.

Hereditary pathology can be prevented by consulting a geneticist during pregnancy planning. Before this, you need to collect all the information about what diseases your closest relatives suffered from. After this, a DNA test can be performed to estimate the percentage of the likelihood of developing ataxia in the fetus.

It is through careful planning of pregnancy that many pathological processes can be avoided.

Cerebellar ataxia is a neurological disorder that manifests itself as a lack of coordination of movements due to damage to the cerebellum and its connections. Symptoms of the pathology include specific disturbances in gait and balance, problems with fluency of speech, muscle hypotonia, incoordination of movements, and dizziness. Treatment is selected depending on the disease that provoked the condition.

The cerebellum is the part of the brain that is located right at the base of the brain. This organ consists of two hemispheres, which are responsible for the accuracy of movements. The cerebellar hemispheres are separated by the vermis, which provides balance and stability when walking. Depending on which part of the cerebellum was affected, two forms of pathology are distinguished: static-locomotor (damage to the vermis, in which there is a disorder of stability and gait) and dynamic (the hemispheres are affected, due to which the ability to perform voluntary movements of the limbs is impaired).

Causes of cerebellar ataxia

In neurology, a classification of cerebellar ataxia is used, based on the criteria for the course of the disease. It distinguishes three main types of ataxia: acute onset, subacute onset and chronic. Each of these types can be caused by different diseases.

Ataxia with acute onset (develops suddenly after exposure to provoking factors on the body):

ischemic stroke provoked by atherosclerotic occlusion or embolism of the cerebral arteries that feed the cerebellar tissue (considered one of the most common causes of pathology);
hemorrhagic stroke;
cerebellar injury resulting from intracerebral hematoma or traumatic brain injury;
multiple sclerosis;
Guillain-Barre syndrome;
encephalitis and post-infectious cerebellitis;
intoxication of the body (lithium, barbiturates, diphenine);
hyperthermia;
metabolic disorders;
obstructive hydrocephalus.

Ataxia with subacute onset (develops over one or more weeks):

tumors different kinds abscesses and other space-occupying processes in the cerebellum (astrocytoma, medulloblastoma, hemangioblastoma, ependymoma);
normal pressure hydrocephalus caused by subarachnoid hemorrhage after brain surgery or meningitis;
endocrine disorders (hyperparathyroidism, hypothyroidism);
vitamin deficiency;
overdose of anticonvulsants;
toxic and metabolic disorders associated with absorption and nutritional disorders;
malignant tumor diseases (lung cancer, ovarian cancer);
paraneoplastic cerebellar degeneration.

Chronically progressive ataxia (develops over a couple of months or years):

spinocerebellar ataxia (Friedreich's ataxia, "Nefriedreich's" ataxia);
cortical cerebellar ataxias (Cortical cerebellar atrophy of Holmes, Late cerebellar atrophy of Marie-Foy-Alaguanine);
late-onset cerebellar ataxias (OPCA, Machado-Joseph disease, cerebellar dysgenesis).

Symptoms of cerebellar ataxia

Patients with cerebellar ataxia usually make swinging and unsteady movements when walking. Their gait is unsteady; for greater stability they try to spread their legs wide apart. If the patient is asked to try to walk along one line, he may be seen to sway from one side to the other. Typically, the symptoms of the disease become more pronounced when the patient suddenly changes direction of movement or gets up from a chair and immediately tries to walk quickly.

When walking, the following conditions are observed: hypometria ( sudden stop movements even before a certain goal has been achieved) and hypermetry (excessive range of movements). Patients with cerebellar ataxia are usually unable to perform contralateral movements quickly. Due to problems with coordination, a pathological change in handwriting occurs: macrography appears, it becomes sweeping and uneven.

Cerebellar pathology is also characterized by deviation when walking or even falling to one side. During an appointment with a neurologist, the patient performs a finger-nose test. Typically, patients experience not only waving a finger past the tip of the nose, but also a noticeable tremor of the hands. In this case, it does not matter at all whether the patient’s eyes are open or closed, since this does not affect the test results. Using this test, it is possible to distinguish cerebellar ataxia from vestibular and sensitive.

The following symptoms are characteristic of congenital types of cerebellar ataxia:

impaired coordination of individual muscles;
vertical instability;
abruptness of speech;
speech delay and mental development;
late onset of walking and sitting in children.

Cerebellar ataxia is often accompanied by dysarthria and nystagmus. It is impossible not to notice that the patient has problems with speech: it slows down, loses its smoothness, becomes intermittent and scanned. As the disease progresses, patients' handwriting becomes impaired, facial expressions become poor, lower back pain appears, convulsive twitching occurs, vision deteriorates, strabismus develops, difficulties with swallowing and eating in general arise, hearing is impaired, the psyche changes and depression occurs.

A characteristic sign of the disease, thanks to which it can be diagnosed, will be a violation of combined movements, which normally should go together. For example, when bending the body back, a person usually bends his legs at the knees, and when he tries to look up, throwing his head back, he wrinkles his forehead a little. In patients with ataxia, these movements are usually not coordinated.

Diagnosis of cerebellar ataxia

When diagnosing, the doctor must take into account that it can be caused by the most various diseases. That is why such specialists as a neurologist, traumatologist, oncologist, endocrinologist, geneticist, and neurosurgeon participate in the diagnosis of pathology. The first stage of diagnosis is examination of the patient and collection of anamnesis. Patients with coordination problems usually complain of staggering and falling while walking.

To make an accurate diagnosis, the doctor asks the patient whether the symptoms of the pathology increase in the dark, and asks whether frequent dizziness bothers him. During the clinical examination, the neurologist evaluates the patient's gait and balance. For example, to test gait, a doctor may ask a patient to walk in a straight line and then make a turn.

The most reliable methods for making an accurate diagnosis for cerebellar ataxia are CT, MSCT and MRI of the brain. With their help, it is possible to detect brain tumors, congenital anomalies, and pathological changes in the cerebellum. For more accurate diagnosis pathologies, Doppler sonography and MRA of cerebral vessels can also be used. It is possible to identify the disease in case of its hereditary etiology genetic analysis and DNA diagnostics.

The symptoms of cerebellar ataxia are in many ways similar to the symptoms of other neurological pathologies. Therefore, it is important for the neurologist to carry out the most accurate differential diagnosis. Namely, with the help of studies of the vestibular analyzer (vestibulometry, stabilography, electronystagmography) it is possible to exclude vestibular disorders. CSF hypertension, symptoms of hemorrhage, tumor or inflammatory processes can be detected by performing a lumbar puncture. Exclude infectious lesion brain can be obtained by obtaining the results of a PCR study or a blood test for sterility.

Treatment of cerebellar ataxia

Treatment of cerebellar ataxia is aimed at eliminating the disease that provoked it. In any case, how to treat cerebellar ataxia will be decided by a group of doctors who are competent in this field, taking into account the patient's condition and his chances of recovery.

Drug therapy

If the pathology was caused by an infectious-inflammatory process, then the doctor prescribes an antiviral or antibacterial therapy. Vascular disorders that cause cerebellar ataxia are treated by stopping cerebral bleeding or normalizing blood circulation. For this, the patient is prescribed the following groups of drugs: thrombolytics, angioprotectors, antiplatelet agents, anticoagulants, vasodilators.

If cerebellar ataxia has a hereditary etiology, it is impossible to completely get rid of it. Basically, doctors resort to metabolic therapy, which involves prescribing drugs such as cerebrolysin, vitamins B12, B6 and B1, ATP, ginkgo biloba preparations, mildronate, piracetam. To increase the tone of skeletal muscles and improve metabolism in them, massage is recommended for patients.

Surgical therapy

The most difficult and time-consuming treatment will be for ataxia caused by a tumor disease. In this case, doctors resort to radical surgical treatment. If, during the diagnosis of the tumor, its malignant nature is revealed, doctors additionally prescribe a course of chemotherapy. To eliminate cerebellar ataxia caused by hydrocephalus, shunt surgery is prescribed.

Conservative therapy

Besides drug therapy And surgical operations, complex therapy disease involves the appointment of other conservative methods. According to indications, patients may be prescribed speech therapy sessions, occupational therapy, physiotherapy, physical therapy. The latter involves both performing sports exercises and repeatedly repeating various household skills to improve coordination of movements: turning pages, pouring liquids, buttoning clothes.

Prognosis and prevention

The prognosis for the patient depends solely on the cause that triggered the onset of the disease. Acute and subacute ataxia caused by intoxication of the body, vascular disorders, may completely regress or partially persist. inflammatory processes. In this case, the prognosis for the patient will be as favorable as possible if the provoking factor can be eliminated in time: infection, toxic effects, vascular occlusion.

For chronic form ataxia is characterized by a gradual increase in symptoms, which ultimately leads to disability of the patient. The greatest danger to the patient's life is cerebellar ataxia caused by tumor processes. The rapid development of the disease and disruption of the functioning of many organs leads to serious complications that significantly worsen the patient’s quality of life.

Prevention of cerebellar ataxia involves preventing traumatic brain injuries, infection of the body, the development of vascular disorders, and timely treatment chronic ischemia brain, compensation for metabolic and endocrine disorders, mandatory genetic counseling during pregnancy planning.

Cerebellum(in lat. cerebellum) in humans is located inside the skull, in the area of the back of the head. Typically, the cerebellum has an average volume of 162 cubic meters. cm, and its weight varies between 135-169 g. The cerebellum has two hemispheres, between which is located its oldest part - worm . In addition, with the help of three pairs of legs, it is connected to the medulla oblongata, the pons and the midbrain. It consists of white and gray matter, from the latter the cerebellar cortex and paired nuclei in its body are formed. The worm is responsible for balance and stability of the body, and the hemispheres are responsible for the accuracy of movements. To maintain body balance, the cerebellum receives information from proprioceptors. various parts body and from other organizations that are involved in monitoring the position of the human body ( lower olives , vestibular nuclei ).

When muscle groups are tensed when performing purposeful actions, maintaining body position in balance, they enter the cerebellum. nerve impulses . They run from the spinal cord and from the part of the cerebral cortex that is responsible for movement. Having passed through a complex system of contacts, a stream of nerve impulses enters the cerebellum, which, in turn, analyzes it and produces an “answer” that already enters the human consciousness, i.e. into the cerebral cortex and spinal cord. Thanks to the coordinated work of all organs, the work of the muscles of the body becomes clear and beautiful. Lesions of the cerebellum and its vermis manifest themselves in static violations, i.e. a person cannot consistently maintain a stable position of the body’s center of gravity, as well as balance.

The term " ataxia “relative to diseases of the nervous system has been used since the time of Hippocrates, and then it meant “disorder” and “confusion”, today ataxia is understood as impaired motor coordination. Ataxia manifests itself in a disorder of gait and movements of the limbs, which manifests itself in trembling when performing actions, missing the mark, as well as imbalance in a standing and sitting position.

There are ataxias cerebellar , sensitive , vestibular And frontal . When the cerebellum and its pathways are damaged, cerebellar ataxia occurs. This disease manifests itself with symptoms of ataxia when standing and walking. The gait of a patient with cerebellar ataxia resembles the gait of a drunk; he walks uncertainly, spreads his legs wide, and is thrown from side to side, usually towards the location of the pathological focus.

The limbs are intentional, a person cannot quickly change body position. It can also often be observed asynergy , that is, inconsistency of movements, for example, when tilting the body back, the legs do not bend in knee joints, so a fall is possible. Very often, with cerebellar ataxia, “chopped” speech is observed, changes in handwriting may be observed, sometimes - muscle hypotonia .

If the lesion spreads to the cerebellar hemispheres, then ataxia of the limbs may develop on the side in which the hemisphere is located; if the vermis is affected, then ataxia of the trunk develops.

If a patient with cerebellar ataxia is placed in Romberg pose (that is, stand with your legs tightly together, your arms pressed to your body and your head raised), then this position is unstable, the person’s body can sway, sometimes pulling on one side, even to the point of falling. If a worm is affected, then the patient falls backward, and if one of the hemispheres is affected, then towards the pathological focus.

In a normal state, if there is a threat of falling to the side, the leg located on the side of the fall moves in the same direction, and the other one comes off the floor, that is, a “jump reaction” occurs. Cerebellar ataxia disrupts these reactions; if he is slightly pushed to the side, he easily falls ( pushing symptom ).

IN at a young age The human nervous system has a fairly high potential neuroplasticity, i.e. the property of the nervous system to quickly restructure under the influence of external or internal changes. For the development of neuroplasticity, a series of repetitions of the same impact is necessary, due to which biochemical and electrophysiological changes occur in the central nervous system. As a result of this, new contacts are formed between the cells of the central nervous system, or old contacts become active. And with ataxia, due to damage to nerve tissue, the skills of subtle and harmonious movements cannot be formed.

It should be noted that ataxia occurs in approximately 1 to 23 people per 100 thousand (prevalence depends on the region). The actual development of ataxia is usually genetically determined, and the symptoms of congenital cerebellar ataxia appear in childhood.

According to modern classifications, cerebellar ataxias are:

(non-progressive, when the hemispheres or cerebellar vermis are underdeveloped or absent).
Autosomal recessive ataxias, arising at an early age (). Friedreich's ataxia was first described in 1861, and symptoms usually appear in children or young adults under 25 years of age. The disease also manifests itself in symptoms of ataxia, static disorders, unsure gait, decreased muscle tone. The resulting sensitivity disorder leads to a decrease in tendon reflexes. Friedreich's ataxia is characterized by abnormal development of the skeleton, the appearance of "Friedreich's foot", i.e. shortened foot, high arch. The disease itself progresses quite slowly, but leads to disability of patients and bedriddenness.
Recessive ataxias, associated with chromosome X ( X-chromosomal ataxia ). This type of ataxia is very rare, mainly in males in the form of progressive cerebellar insufficiency.
Betten's disease, inherited in an autosomal recessive manner, is congenital disease. It is characterized by congenital cerebellar ataxia, which is transmitted in the first years of life in the form of disturbances in statics, coordination of movements and gaze. Such children begin to hold their heads by the age of 2-3 years, and walk and talk even later. With age, the patient adapts to his condition.
Autosomal dominant ataxias late age (they are also called spinocerebellar ataxias). This includes Pierre Marie's disease . This type of hereditary cerebellar ataxia manifests itself at the age of 25-45 years, and affects the cells of the cerebellar cortex and nuclei, spinocerebellar tracts in spinal cord and pontine nuclei. Its signs are ataxia and pyramidal insufficiency, intention tremor, tendon hyperreflexia, “chopped” speech. Sometimes - ptosis, decreased vision. The size of the cerebellum gradually decreases, and a decrease in intelligence and depressive states are very often observed. By the way, most authors consider cerebellar ataxia of Pierre Marie a syndrome, which includes olivopontocerebellar (Dejerine-Thomas) and olivocerebellar atrophy (Holmes type), cerebellar atrophy of Marie-Foy-Alaguanin, as well as olivorubrocerebellar atrophy of Lhermitte.

Symptoms of congenital cerebellar ataxias

Common symptoms for all types of ataxia are ataxic manifestations. This is a violation coordinated work all muscles to achieve the goal of the act of movement. Symptoms of congenital cerebellar ataxias include dysmetria - disproportionality of the efforts made to perform a targeted movement. Dyssynergia is observed when the coordination of individual muscles is impaired, intentional trembling, rhythmic deviation from the correct trajectory of targeted movement, which increases as the target approaches.

Frequent signs of instability in an upright position, nystagmus (rhythmic rapid movements eyeball), as well as abrupt speech, stress on each syllable. A symptom of cerebellar ataxia in children is that the child begins to sit and walk late, and his gait is uncertain, the child seems to “sway.”

Symptoms of congenital cerebellar ataxia are manifested by a delay in the child’s motor functions, he begins to sit and walk late, there is a lag in mental development, and speech delay. Usually by the age of 10, compensation of brain functions occurs.

To confirm the diagnosis, it is necessary to carry out CT And MRI studies , and DNA research to identify genes that lead to the development of certain forms of pathologies.

Treatment of congenital cerebellar ataxias

Treatment of the disease includes measures for motor and social rehabilitation patients, so that the patient adapts to his defect. Recommended classes physical therapy, walking training, classes with a speech therapist, training on stabilometric platform .

Sometimes, depending on the type of ataxia, drug treatment congenital cerebellar ataxias with the use of muscle relaxants, nootropics , anticonvulsant drugs. To treat Pierre Marie's disease, drugs are used to reduce muscle tone (, melictin , Condolphin ).