Static and dynamic ataxia. Static nature of cerebellar ataxia. How is ataxia diagnosed?

Ataxia(from the Greek ataxia - disorder) - a disorder of coordination of movements; a very common dysmotility. Strength in the limbs is slightly reduced or completely preserved. Movements become inaccurate, awkward, their continuity and sequence are upset, balance is disturbed in a standing position and when walking. Static ataxia is a violation of balance in a standing position, dynamic ataxia is a violation of coordination during movement. Diagnosis of ataxia includes neurological examination, EEG, EMG, MRI of the brain, if the hereditary nature of the disease is suspected - DNA analysis. Therapy and prognosis for the development of ataxia depend on the cause of its occurrence.

General information

Normal coordination of movements is possible only with highly automated and friendly activity of a number of departments of the central nervous system - conductors of deep muscle sensitivity, vestibular apparatus, the cortex of the temporal and frontal regions and the cerebellum - the central organ for coordinating movements.

Classification of ataxias

Symptoms of ataxia

emergence sensitive ataxia due to damage to the posterior columns (gaugles of Gaulle and Burdach), less often the posterior nerves, peripheral nodes, cortex of the parietal lobe of the brain, visual tubercle (funicular myelosis, dorsal tabes, vascular disorders). Perhaps its manifestation, both in all limbs, and in one leg or arm. The most revealing phenomena are sensitive ataxia, resulting from a disorder of the articular-muscular feeling in lower limbs. The patient is unsteady, when walking excessively bends the legs in the hip and knee joints, too much steps on the floor (punching gait). Often there is a feeling of walking on cotton wool or carpet. Patients try to compensate for the disorder of motor functions with the help of vision - when walking, they constantly look at their feet. This can significantly reduce the manifestations of ataxia, and closing the eyes, on the contrary, exacerbates them. Severe lesions of the posterior columns practically make it impossible to stand and walk.

Cerebellar ataxia- a consequence of the defeat of the cerebellar vermis, its hemispheres and legs. In the Romberg position and when walking, the patient collapses (up to a fall) towards the affected cerebellar hemisphere. In the event of damage to the cerebellar vermis, a fall to either side or backwards is possible. The patient staggers when walking, puts his feet wide. The flank gait is sharply disturbed. The movements are sweeping, slow and awkward (mostly from the side of the affected cerebellar hemisphere). Coordination disorder almost invariably under visual control (open and closed eyes). There is a violation of speech - it slows down, becomes stretched, jerky, often chanted. Handwriting becomes sweeping, uneven, macrography is observed. Perhaps a decrease in muscle tone (to a greater extent on the side of the lesion), as well as a violation of tendon reflexes. Cerebellar ataxia may be a symptom of encephalitis various etiologies, multiple sclerosis, malignant neoplasm, vascular focus in the brain stem or cerebellum.

Development cortical ataxia (frontal) is caused by damage to the frontal lobe of the brain caused by dysfunction of the fronto-pontocerebellar system. With frontal ataxia, the leg contralateral to the affected cerebellar hemisphere suffers to the maximum extent. When walking, instability is observed (to a greater extent on turns), tilt or collapse to the side ipsilateral to the affected hemisphere. In severe lesions of the frontal lobe, patients cannot walk or stand at all. Visual control does not affect the severity of violations when walking. Cortical ataxia is also characterized by other symptoms characteristic of damage to the frontal lobe - a grasping reflex, changes in the psyche, impaired sense of smell. The symptom complex of frontal ataxia is very similar to cerebellar ataxia. The main difference of the cerebellar lesion is demonstrative hypotension in the atactic limb. The causes of frontal ataxia are abscesses, tumors, cerebrovascular accidents.

hereditary cerebellar Pierre-Marie ataxia- a hereditary disease of a chronic progressive nature. It is transmitted in an autosomal dominant manner. Its main manifestation is cerebellar ataxia. The pathogen has a high penetrance, skipping generations is very rare. A characteristic pathoanatomical sign of Pierre-Marie's ataxia is cerebellar hypoplasia, less often - atrophy of the inferior olives, the pons of the brain (pons varoli). Often these signs are combined with combined degeneration. spinal systems(the clinical picture resembles Friedreich's spinocerebellar ataxia).

The median age of onset is 35 years, when gait disturbance appears. Subsequently, it is joined by a violation of facial expressions, speech and ataxia in the hands. There is static ataxia, adiadochokinesis, dysmetria. Tendon reflexes are increased (to pathological reflexes). Involuntary muscle tremors are possible. The strength in the muscles of the limbs is reduced. Progressive oculomotor disorders are observed - paresis of the abducens nerve, ptosis, insufficiency of convergence, less often - Argyle Robertson's symptom, atrophy of the optic nerves, decreased visual acuity, narrowing of the visual fields. Mental disorders manifested in the form of depression, decreased intelligence.

Familial Friedreich ataxia- a hereditary disease of a chronic progressive nature. It is transmitted in an autosomal dominant manner. Its main manifestation is mixed sensory-cerebellar ataxia resulting from a combined lesion of the spinal systems. Blood marriages are very common among the parents of patients. A characteristic pathoanatomical sign of Friedreich's ataxia is the progressive degeneration of the lateral and posterior columns spinal cord(before medulla oblongata). Goll's bundles are more affected. In addition, the cells of Clark's pillars are affected, and with them the posterior spinocerebellar pathway.

The main symptom of Friedreich's ataxia is ataxia, which is expressed in an uncertain, awkward gait. The patient walks with a flourish, deviating from the center to the sides and placing his feet wide. Charcot designated such a gait as tabetic-cerebellar. With the development of the disease, discoordination extends to the hands, muscles chest and face. Facial expressions change, speech becomes slow, jerky. Tendon and periosteal reflexes are significantly reduced or absent (primarily on the legs, later on upper limbs). In most cases, hearing is reduced.

With the development of Friedreich's ataxia, extraneural disorders appear - heart damage and skeletal changes. On the ECG - deformation of the atrial wave, rhythm disturbance. There is paroxysmal pain in the heart, tachycardia, shortness of breath (as a result physical tension). Changes in the skeleton are expressed in a characteristic change in the shape of the foot - a tendency to frequent dislocations of the joints, an increase in the arch and extension of the fingers, as well as kyphoscoliosis. Among the endocrine disorders that accompany Friedreich's ataxia, diabetes, hypogonadism, and infantilism are noted.

Ataxia-telangiectasia(Louis-Bar syndrome) is a hereditary disease (a group of phakomatoses) transmitted in an autosomal recessive manner. Very often accompanied by dysgammaglobulinemia and hypoplasia thymus. The development of the disease begins at an early age. childhood when the first ataxic disorders appear. In the future, ataxia progresses and by the age of 10 walking is almost impossible. Often, Louis-Bar syndrome is accompanied by extrapyramidal symptoms (hyperkinesis of the myoclonic and athetoid type, hypokinesia), mental retardation, damage cranial nerves. A tendency to repeated infections (rhinitis, sinusitis, bronchitis, pneumonia) is characteristic, which is primarily due to the lack of immunological reactions of the body. Due to the deficiency of T-dependent lymphocytes and class A immunoglobulins, the risk of malignant neoplasms is high.

Complications of ataxia

Diagnosis of ataxia

Diagnosis of ataxia is based on the identification of diseases in the patient's family and the presence of ataxia. EEG of the brain in Pierre Marie's ataxia and Friedreich's ataxia reveals the following violations: diffuse delta and theta activity, alpha rhythm reduction. AT laboratory research there is a violation of amino acid metabolism (the concentration of leucine and alanine is reduced, their excretion in the urine is also reduced). MRI of the brain reveals atrophy of the spinal cord and brain stems, as well as upper divisions worm. Using electromyography, an axonal-demyelinating lesion of sensory fibers is detected. peripheral nerves.

When differentiating ataxia, it is necessary to take into account the variability of the clinical picture of ataxia. AT clinical practice rudimentary varieties of ataxia and its transitional forms are observed when clinical manifestations similar to the symptoms of familial paraplegia (spastic), neural amyotrophy and multiple sclerosis.

Diagnosis of hereditary ataxia requires direct or indirect DNA diagnostics. With the help of molecular genetic methods, ataxia is diagnosed in a patient, after which indirect DNA diagnostics are performed. With its help, the possibility of inheritance of the pathogen of ataxia by other children in the family is established. It is possible to conduct a comprehensive DNA diagnosis, it will require the biomaterial of all family members (the biological parents of the child and all other children of this parental couple). AT rare cases prenatal DNA diagnostics is indicated.

Treatment and prognosis of ataxia

Ataxia is treated by a neurologist. It is predominantly symptomatic and should include: general strengthening therapy (vitamins of group B, ATP, anticholinesterase agents); special complex gymnastic exercises Exercise therapy aimed at strengthening muscles and reducing discoordination. In Friedreich's ataxia, taking into account the pathogenesis of the disease, drugs that support mitochondrial function can play an important role in the treatment ( succinic acid, riboflavin, coenzyme Q10, vitamin E).

For the treatment of ataxia-telangiectasia, in addition to the above algorithms, correction of immunodeficiency is necessary. For this, a course of treatment with immunoglobulin is prescribed. Radiation therapy in such cases is contraindicated, in addition, excessive x-ray radiation and prolonged exposure to the sun.

Prediction of genomic hereditary diseases unfavorable. There is a slow progression of neuropsychiatric disorders. The ability to work in most cases is reduced. However, due to symptomatic treatment and prevention of recurrent infectious diseases, injuries and intoxications, patients have the opportunity to live to an advanced age. FROM preventive purpose the birth of children in families where there are patients with hereditary ataxia should be avoided. In addition, it is recommended to exclude the possibility of any kindred marriages.

Pathology, which is manifested by impaired motor skills, is called ataxia. It occurs as a result of the development of neoplasms or serious illnesses brain and spinal cord. There are several types of ataxia, depending on the location of the lesion. Installing the latter is prerequisite effective treatment.

general information

The term "ataxia" has Greek roots and literally translates as "disorder". The disease he describes is quite common and is manifested by impaired coordination of movements and balance. When it occurs in a person, there is an inconsistency in the interaction various groups muscles. Moreover, the strength of the limbs is either completely preserved, or decreases slightly. At the same time, lesions entail fuzziness and clumsiness of movements, not only during walking, but also in the usual standing position.

Due to the fact that the body has several parts of the nervous system that provide balance and coordination, in the event of ataxia, the doctor, first of all, conducts an examination to exclude malfunctions in the work of one of them. That is, in the work of the cerebellum, the cortex of the frontal lobe, the vestibular apparatus, conductors of deep joint-muscular sensitivity, the occipital and temporal lobes of the brain.

Note!Ataxias are pathologies that entail uncoordinated movements. In other words, in a person with such a diagnosis, speech, walking, fine motor skills, swallowing, eye movements are violated. Her motor activity can become choppy, intermittent, or even more complicated.

Kinds

First of all, hereditary and acquired ataxia is diagnosed. There is another classification - depending on the nature of the lesions. According to her, pathology can be:

static, when balance is disturbed (it is difficult for a person to stand still);
dynamic, when there is a violation of movements, for example, when walking;
static-dynamic, when signs of both types are manifested.

Depending on the location of the lesion, ataxia is distinguished:

Sensitive. It appears when there is a violation of the musculo-articular sensitivity. The latter is possible with damage to the posterior columns of the spinal cord, peripheral nerves, thalamus, parietal cortex, the development of neurosyphilis, or various vascular pathologies. The disease manifests itself with instability and an unusual gait, when a person excessively bends his legs at the knees or hip joints, stomps his foot expressively. hallmark ailment is an increase in the symptoms of the disease at the moment when the patient closes his eyes.
Cerebellar. Damage to the cerebellum can provoke both the development of dynamic ataxia, diagnosed when problems arise in the hemispheres, and statodynamic. The latter is observed against the background of pathologies of the cerebellar vermis and is manifested by gait disturbances, instability. The causes of diseases can be both brain tumors and vascular diseases structures of the cerebellum. In addition to everything, cerebellar ataxia handwriting and speech disorders may develop.
vestibular. Such ataxia is diagnosed when there is a malfunction of the vestibular apparatus. Along with unsteadiness in this case, a person feels constant dizziness, nausea up to vomiting, horizontal nystagmus. The cause of the development of the disease is most often diseases of the ear, especially Meniere's disease. Distinctive feature such ataxia is a pronounced dependence on head movements. At sharp turns symptoms get worse.
Cortical. Pathology occurs when a tumor appears frontal lobes, with abscesses, circulatory disorders in the brain, encephalitis. Its main signs are discoordination of movements, impaired gait, sometimes the loss of the ability to walk and stand, a pronounced deviation of the body back, braiding of the limbs, the appearance of a "fox gait" when the patient puts his feet on the same line. Despite the fact that vision does not affect the strength of the manifestation of symptoms, the latter are often accompanied by impaired hearing, smell, memory, and the appearance of hallucinations.

Note! Doctors also distinguish psychogenic and intrapsychic ataxia. In the case of their development, a splitting of the functions of the psyche is noted in a person. You can recognize this type of disease by artsy movements when walking: the patient walks without unbending or crossing his legs.

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

Ataxia Pierre Marie- transmitted from parents and is characterized by the appearance of symptoms of cerebellar ataxia, cerebellar hypoplasia, atrophy of the brain bridge. At risk are people aged 30-35 years. Pathology is suspected in case of violations of facial expressions, speech, the appearance of difficulties in walking, the loss of the ability to quickly move hands, involuntary contractions of the muscles of the fingers and limbs, ptosis - omission upper eyelid, the appearance of depression, fear.
Friedreich's ataxia. Pathology that occurs in consanguineous marriage. It is manifested mainly by gait disorders, when a person spreads his legs too wide when walking. With the progression of the disease, there is a violation of the coordination of hands, facial expressions, when there is an involuntary contraction of the muscles of the face, a slowdown in speech, hearing loss, and a decrease in reflexes. If the patient is not provided with timely assistance, he develops cardiovascular diseases, the risk of fractures and dislocations of the joints increases. Often the pathology is accompanied by hormonal disorders, diabetes, sexual dysfunctions.
Louis Bar syndrome. Hereditary pathology, the signs of which appear even in early childhood, when, having reached the age of 9, the child loses the ability to walk. In addition to this, he notes mental retardation, propensity to acute infectious diseases - hypoplasia of the thymus. Often against this background malignant tumor, which aggravates the course of the disease, making its prognosis unfavorable.

Causes of ataxia

The causes of the pathology are:

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

Symptoms of ataxia

It is worth noting that each of the forms of the disease manifests itself in different ways. Meanwhile, for most pathologies, the following symptoms are characteristic:

Diagnostics

Diagnosis of ataxia is, first of all, in identifying its form. To do this, the specialist collects an anamnesis, conducts a general examination, sends the patient to the laboratory instrumental research.

When taking anamnesis, the doctor asks about previous diseases and drugs used for their treatment, heredity. During a general examination, he assesses muscle tone, the quality of vision, hearing, reflexes, and also conducts coordination tests - finger-nose and knee-heel.

In addition, he may refer the patient to:

and and - they allow to exclude or confirm the presence of failures in metabolism, as well as to identify signs of poisoning or inflammation;
– the procedure helps to diagnose atrophic processes in the upper part of the skull;
electroencephalography - a method designed to assess the electrical activity of individual parts of the brain;
and spinal cord - the results of this study help to see in layers the structure of brain tissues, while making out possible pustules, neoplasms, hemorrhages;
spinal puncture with mandatory analysis liquor;
genetic tests to detect mutations;
DNA diagnostics, if hereditary pathologies occur.

Important!For an accurate diagnosis, it is essential to refer to experienced specialist and go through everything necessary examinations. In rare cases, the pathology may resemble in its symptoms multiple sclerosis, thereby misleading physicians.

Treatment of ataxia

Treatment of ataxia is predominantly symptomatic. The neurologist writes out vitamin complexes, including those that contain, and drugs aimed at general strengthening organism, increase immunity and maintain motor activity . Additionally, means for normalization can be assigned blood pressure or improvement of microcirculation of the brain.

When an infection is detected, it is applied. May be used in multiple sclerosis hormonal drugs and plasmapheresis.

Note! The key to success in the treatment of ataxia is the timely identification of the cause of the pathology and its elimination. That is why tumors are removed surgically.

When diagnosing poisoning, maintenance solutions are introduced. Additionally, to strengthen the body, the doctor may recommend physical exercises aimed at increasing muscle tone. Canes, walkers or other devices also make the patient's condition easier.

Forecast

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

Ataxia is an incoordination of movements that is not caused by muscle weakness. In this case, the patient is able to make movements, but their discoordination is observed. The result is a violation of most body functions - movement, speech, walking, swallowing, fine motor skills. Ataxia does not count independent disease, but rather secondary, since it develops against the background of diseases of the nervous system.

Classification of ataxias

Doctors classify ataxia depending on which area of the brain is affected. In total, four forms of the disease are distinguished.

If the vestibular apparatus is affected, vestibular ataxia is diagnosed.
Patients with damage to the frontal or high-occipital region are diagnosed with cortical ataxia.
Cerebellar ataxia is found in patients with cerebellar involvement.
Sensitive - in violation of the conductors of deep muscle sensitivity.

Symptoms of ataxia

Sensitive ataxia

In most cases this pathology arises due to damage to the posterior columns, posterior nerves, peripheral nodes, visual tubercle, cortex of the parietal lobe of the brain. Signs of sensitive ataxia can be observed both in all limbs at once, and only in one of them. Often, doctors diagnose signs of ataxia, which occurs due to a disorder of the joint-muscular feeling in the legs.

Characteristic signs of ataxia will be the patient's instability, excessive bending of the legs at the knee and hip joints. At the same time, many patients often have the feeling that they are walking on cotton wool or a carpet. Patients try to compensate for walking disorders with the help of vision, as they begin to constantly look at their feet. Due to severe damage to the posterior columns, patients almost completely stop walking.

Cerebellar ataxia

It occurs due to a serious lesion of the cerebellar vermis, as well as its legs and hemispheres. However, it is worth noting that cerebellar ataxia can be a symptom of diseases such as multiple sclerosis, encephalitis, a malignant neoplasm in the cerebellum or brain stem. Patients with this pathology, when walking, usually fall in the direction of the damaged cerebellar hemispheres. During such episodes, even falls are possible.

The patient usually staggers a lot when walking, and also puts his legs too wide, his movements are slow, sweeping and awkward. Disturbances of coordination practically do not change even with visual control. Patients may experience severe speech impairment, which gradually slows down, becomes chanted and drawn out. There are also problems with handwriting, which becomes uneven and sprawling.

vestibular ataxia

This pathology occurs as a result of damage to the vestibular nerve, labyrinth, cortical center and nuclei in the brain stem. Observed this species ataxia with various diseases ear, stem encephalitis, Meniere's syndrome, tumors of the IV ventricle of the brain. The main sign of pathology is considered to be regular severe dizziness, as a result of which the patient begins to think that all objects are moving in the same direction. Turning his head, the patient feels increased dizziness.

Severe dizziness leads to unsteady gait and falls. It is also noticeable that the patient tries to make very careful head movements. Vestibular ataxia is also characterized by symptoms such as vomiting, nausea, and horizontal nystagmus.

Cortical ataxia

Cortical ataxia in most cases occurs due to damage to the frontal lobe of the brain. Its most common causes include tumors, circulatory disorders in the brain, abscesses. In the case of this type of ataxia, symptoms such as unsteadiness when walking, falling over or tilting to the side are manifested.

Due to severe damage to the frontal lobe, patients may lose the ability to walk and stand. Cortical ataxia is also characterized by other symptoms: a change in the psyche, a grasping reflex, a violation of smell. In many ways, the clinical picture of cortical ataxia is similar to the symptoms of cerebellar pathology.

Cerebellar Pierre-Marie ataxia

The main manifestation of the disease is cerebellar ataxia. The disease occurs due to cerebellar hypoplasia, atrophy of the brain bridge and lower olives. Usually the first signs of pathology appear in a patient at the age of 35 years.

Most characteristic symptoms diseases are considered to be a violation of gait, speech and facial expressions. In addition, patients usually have dysmetria, static ataxia, adiadochokinesis, increased tendon reflexes, and reduced strength in the muscles of the limbs. Often, patients are diagnosed with oculomotor disorders:

lack of convention;
ptosis;
abducens nerve paresis;
Argyle-Robertson symptom;
decreased visual acuity;
narrowing of the visual fields;
atrophy optic nerves.

Ataxia-telangiectasia

This type of ataxia is also hereditary and is transmitted in an autosomal recessive manner. Often the first symptoms of pathology appear already in a very early age. The disease progresses very quickly, so at the age of 10 years the child almost completely loses the ability to walk independently. Pathology may be accompanied by damage to the cranial nerves and mental fatigue.

Friedreich's familial ataxia

This disease is hereditary and occurs due to damage to the spinal systems. As a result of the research, it was found that in many patients with this type of ataxia, consanguineous marriages are often found in the pedigree. The main symptom of ataxia is an unsteady and clumsy gait.

The gradual development of the disease leads to impaired hand movements, problems with facial expressions, slowing down of speech, and hearing loss. Further development ataxia leads to a change in the skeleton, a violation heart rate, endocrine disorders, frequent dislocations, kyphoscoliosis.

Diagnosis of ataxia

Diagnosis of the disease begins with the collection of complaints and anamnesis. Namely, the doctor must clarify with the patient how long ago he had complaints of unsteady gait and impaired coordination of movements, how often and regularly these symptoms appear. Be sure to check with the patient whether his relatives had this disease. It is also necessary to find out if the patient has taken any medical preparations like benzodiazepines and barbiturates.
Patients who have a suspicion of ataxia need a neurological examination. This examination includes an assessment of coordination of movements and gait, an assessment of strength in the limbs and muscle tone, the presence of nystagmus, strength in the limbs.
For a correct assessment of hearing, an examination by an otolaryngologist will also be required.

Laboratory research

Laboratory tests are of great importance in the diagnosis of ataxia.

Namely, to study the signs of poisoning, which could cause a violation of coordination, a toxicological analysis is prescribed.
A blood test is mandatory, since with ataxia, signs of inflammation in the blood and an increase in the level of leukocytes may appear.
The concentration of vitamin B12 in the blood is also determined.

Additional tests

For a more detailed study of the disease, a consultation with a neurosurgeon will be required, as well as a number of instrumental studies.

In particular, a lot of information about the disease can be obtained using electroencephalography. This technique evaluates the electrical activity of various parts of the brain, which tends to change with various diseases.
Not less than effective methods are also CT and MRI of the brain. They allow you to study the structure of the brain in layers, detect violations of the structure of its tissues, identify abscesses, tumors, and hemorrhages.
Magnetic resonance angiography will allow detecting tumors in the brain and assessing the integrity of the arteries in the skull.

Complications of ataxia

Treatment of ataxia should be timely, otherwise serious complications. One of the most dangerous complications of ataxia is heart failure. Is it sharp or chronic condition, which is accompanied by shortness of breath, swelling, frequent fatigue. The danger of pathology is that it can cause pulmonary edema. Today, heart failure is one of the most common causes of death. Not less than dangerous complications ataxias are also repetitive infectious diseases and respiratory failure.

Treatment of ataxia

Treatment of ataxia is aimed at eliminating etiological factor that spurred the development. Depending on the specific disease, the following operations may be prescribed:

tumor removal;
elimination of hemorrhage;
removal of the abscess and subsequent antibiotic therapy.

In rare cases, a back pressure relief procedure may be necessary. cranial fossa. Namely, this procedure is carried out in case of detection of an Arnold-Chiari anomaly in a patient, which is characterized by a slight descent of a part of the cerebellum into the opening of the skull, which ultimately leads to a strong compression of the brain stem. The cause of ataxia can also be hydrocephalus, which is characterized by the accumulation of cerebrospinal fluid in the ventricular system of the brain. In this case, doctors create an outflow of cerebrospinal fluid, due to which the metabolism in the brain is normalized.

If the cause of this serious illness was high blood pressure, it is necessary to normalize it using drug therapy. In case of circulatory disorders in the brain, drugs that improve metabolism and blood flow are also shown. These primarily include nootropics and angioprotectors.

Ear diseases can also lead to the development of ataxia. When infection brain caused by ear pathology, a course of antibiotic therapy is prescribed. With a lack of vitamin B12, it must be compensated. To eliminate poisoning, vitamins of groups A, B, C are prescribed. In demyelinating diseases, the appointment of hormonal drugs, as well as plasmapheresis, is indicated. This procedure involves the removal of blood plasma, but the preservation of blood cells.

Ataxia - dangerous disease, because due to impaired coordination, the patient may experience serious injury. You can prevent this by following a few rules:

It is better for all patients, regardless of the stage of their disease, to refuse to drive a car and work with power tools. In these situations, lack of coordination can cause serious injury.
Patients with ataxia should always keep the room well-lit in order to increase the depth of perception and avoid injury. The twilight is especially dangerous for patients.
Many patients experience severe difficulty climbing stairs. To avoid injury, it is necessary to hold on to the railing or use outside help when climbing the stairs.
If it is very difficult for the patient to move independently due to impaired coordination, he should use a walker or cane while moving.
If you experience nausea or severe dizziness, you must immediately sit down or lie down.
Doctors also advise listening to your body. Therefore, in case of aggravation of the disease, it is worth making an appointment with a neurologist.

Prognosis for ataxia

As for hereditary diseases, their prognosis is unfavorable, since in many patients, in addition to serious violations movements gradually progress neuropsychiatric disorders. In addition, their ability to work is significantly reduced. It is possible to improve the prognosis for patients with the help of symptomatic treatment diseases, as well as the prevention of injuries and infectious diseases. To avoid hereditary ataxia, it is worth excluding any possibility of consanguineous marriages.

Prevention of ataxia

To prevent the disease, doctors advise to conduct active image life. Namely, every day it is worth walking on fresh air, observe correct mode sleep and wakefulness, adhere to the principles healthy eating, do sport. It is advisable to treat all infectious diseases in a timely manner, as well as constantly monitor blood pressure.

Ataxia is a motor disorder in which a person cannot coordinate movements normally. In patients, there is a slight decrease in the strength of the limbs, it is difficult for them to maintain balance while standing or while moving. From the side, the movements seem awkward, a person cannot do them accurately, continuity, sequence are violated.

Ataxia: how is it?

Ataxia is a disease associated with the inability to accurately control movement. 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 the central organ, its connections, systems are most important for correct coordination. Receptors are needed to assess how stretched muscles, joint bags, tendons are. Through receptors at each moment of time, information about the state of tissues enters the coordinating center. Finally, impulses are needed to assess the position of the organism in space.

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

What is?

The main types of disease:

sensitive;
vestibular;
cerebellar;
cortical.

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

Where did the trouble come from?

The causes of ataxia are various bruises and blows, injuries of the skull, brain. There may be a violation of the ability to coordinate movements 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, oncological disease or an abscess. If in childhood the patient suffers from epilepsy, the risk of developing ataxia is assessed as above average.

More about types

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

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

The vestibular type of ataxia is observed if the work is disturbed, the integrity of the vestibular apparatus (any of its parts). The brain stem nuclei, crusts in the temporal medulla, the labyrinth of the brain, or the nerve responsible for the operation of the system may be affected.

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

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

How to notice?

Symptoms of ataxia depend on the form of the disease. In particular, with sensitive, the legs suffer, in the first place - muscles, joints. The patient loses stability, when walking, bends the legs excessively, feels as if walking on cotton wool. Reflexively, such a person constantly looks under his feet while moving to compensate for the lack of activity. locomotive apparatus. If you close your eyes, the symptoms 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 while walking, unnecessarily wide leg stances, and sweeping movements. The patient is awkward, and when walking falls to one side - this shows which hemisphere of the cerebellum has suffered. Coordination problems do not depend on visual control of the situation, the symptoms persist equally if the eyes are open and closed. The patient speaks slowly and writes fluently. Muscle tone decreases, tendon reflexes are disturbed.

Manifestations: what else are possible?

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

Cortical ataxia expresses itself as unsteadiness when moving, most pronounced when turning, disturbances in the perception of smells, as well as mental disorders. The patient loses the grasp reflex.

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 is deteriorating, intelligence is deteriorating. Many suffer from depression.

Friedreich's disease is accompanied by a malfunction of the Gaull, Clark, and spinal systems. The gait becomes clumsy, the person moves unsteadily, sweepingly, while the legs are unreasonably wide apart. The patient deviates from the center in different directions. Mimicry gradually suffers, speech worsens, reflexes of certain groups of tendons decrease, hearing weakens. If the disease is severe, the skeleton changes, the heart suffers.

Louis Bar syndrome usually appears 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 work of the nerves of the skull is disrupted, the intellect lags behind, immunity decreases. Patients with this syndrome are characterized by frequent bronchitis, pneumonia, runny nose.

How to check the status?

Assuming cerebellar ataxia, vestibular or any of the other ones described above, you should consult a doctor for a full diagnosis. Only after the formulation of the final diagnosis, you can choose a treatment program.

If ataxia is suspected, the patient is referred for MRI to assess the state of the brain, electroencephalography and electromyography. If a hereditary form is suspected, DNA diagnostics are necessary. Based on this type of indirect analysis, doctors determine how likely the pathogen is to be inherited within the family. Also, the patient is prescribed MRI angiography. If there are brain tumors, it is this method that helps to determine them as accurately as possible.

To identify hereditary, static, cerebellar ataxia and any other form, a series of additional research. The patient is examined by an ophthalmologist, neuropathologist, psychiatrist. AT laboratory tests it is possible to identify metabolic problems.

How to fight?

Treatment of ataxia is possible only in the clinic. It is impossible to cope with this disease on your own - it does not matter in what form and by what type the violation develops. The control of treatment is assigned to the neurologist. The main idea of the therapeutic course is the elimination of the disease that led to ataxia. If this is a neoplasm, it is removed, hemorrhage is eliminated damaged tissues. In some cases, removal of an abscess and stabilization of pressure in circulatory system, decrease in pressure indicators in the fossa of the skull from behind.

Treatment of ataxia involves the practice of gymnastic exercises, the complex of which is developed based on the patient's condition. The main task of gymnastics is to strengthen muscle tissues and ease coordination problems. The patient is discharged restorative means, vitamins, ATP.

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

And if not treated?

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 lethal outcome. With ataxia, patients suffer from hand tremors, dizziness is severe and often, it is impossible to move independently, swallow, and the ability to defecate is impaired. Over time, there is a lack of work respiratory system, hearts in chronic form, decreases immune status. The patient has frequent infections.

Obvious complications are not observed in 100% of cases. If you strictly follow the recommendations of the doctor and use the medicines prescribed by the specialist, take measures to correct the signs of the disease, the quality of life will remain at the same level. Patients passing adequate therapy, live to a ripe old age.

Dangers and likelihood of getting sick

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

In order to minimize the birth of those 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, in principle, to refrain from having children. This is especially true if there are already children with ataxia due to genetics.

Prevention of ataxia involves the rejection of marriages between close relatives, the treatment of any infectious foci and control of pressure, as well as the observance of a normal daily routine, proper nutrition. Sports that are associated with 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 the spasm of the arms, legs, and head worries. The disease can affect both children and adults. To clarify the diagnosis requires a physical examination, instrumental studies. Eliminate ataxia completely impossible. At rapid development disease prognosis is negative. An exception is cerebellar ataxia due to 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, TBI, neoplasm, virus or infection, sclerosis, cerebral palsy and similar pathological conditions, stroke, poisoning with poisons, metals. From the statistics it is known that the acquired form is more often observed after a stroke or injury. Hereditary can be explained by a gene mutation. Currently no detailed explanation causes of processes.

Types and forms: cerebellar

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

Cerebellar ataxia is 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 it is necessary to fight in a particular case, the doctor prescribes tests and studies.

The specificity of the manifestation of the cerebellar form

The clinical picture of the form of the disease prone to progress is specific, therefore, to establish accurate diagnosis usually easy. The disease can be presumed on the basis of general symptoms, the behavior of the patient, the postures he takes. When observing a person, it seems that he is trying to balance, for which he spreads his arms to the sides. The patient tends to avoid turning his torso, head, arbitrarily falls from a slight push at the moment of trying to move his legs, and is not even aware of this. The limbs are tense, the gait is similar to that of a drunkard, the body is straightened and thrown back.

As it progresses, cerebellar ataxia results in an inability to correct movements. Unable to touch the tip of the nose. The kidneys, speech are changing, the face looks like a mask, the muscles are in good shape all the time, the back, neck, legs and arms hurt. Possible convulsions, nystagmus, strabismus. In some patients, vision, hearing weakens, it becomes difficult to swallow.

Cerebellar form: congenital

It is possible to suspect a disease in a child if the efforts applied by the baby to make 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 limited from each other. The development of speech, the psyche is slowed down.

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

What to do?

With cerebellar ataxia, the main task of treatment is to slow down negative processes. Friedreich's form, like other congenital ones, is not treated. The use of radical, conservative approaches helps to preserve the patient's quality of life for as long as possible. Medicines are prescribed, relatives are taught the rules of caring for the needy.

Treatment of ataxia involves the use of nootropics, brain blood flow stimulants, anti-seizure drugs, substances that lower muscle tone, muscle relaxants, and betagestin drugs.

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

Strict adherence to medical recommendations helps 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 reasons pathological condition, form of manifestation, age of the patient, vital signs of the organism.

Ataxia(Greek ataxia - disorder) - impaired coordination of movements; one of the most commonly observed motor disorders. Strength in the limbs can be fully preserved. However, the movements become awkward, inaccurate, their continuity and sequence, balance when standing and walking are violated.

Allocate:

static ataxia - impaired balance when standing,
dynamic ataxia - Discoordination during movements.

Normal coordination of movements is due to the friendly and highly automated activity of several parts of the central nervous system - the cerebellum, the vestibular apparatus, conductors of deep muscle sensitivity, the cortex of the frontal and temporal regions. The central organ for coordinating movements is the cerebellum..

Clinic.

In clinical practice, it is customary to distinguish between:

ataxia in violation of conductors of deep muscle sensitivity (sensitive, or posterior columnar, ataxia);
cerebellar ataxia (cerebellar ataxia);
ataxia in lesions of the vestibular apparatus (vestibular ataxia);
ataxia with damage to the cortex of the frontal or temporo-occipital region (cortical ataxia).

Sensitive, or posterior columnar, ataxia occurs when the posterior pillars (the bundles of Gaulle and Burdakh) are affected, much less often - peripheral nerves, posterior roots, thalamus, cortex of the parietal lobe of the brain ( dorsal tabes, funicular myelosis, some forms of polyneuropathy, vascular disorders, tumors).

Depending on the location of the lesion, it can be expressed in all limbs or only in the legs, in one leg or arm.
The most characteristic phenomena are sensitive ataxia, which occurs when there is a disorder of the articular-muscular feeling in the lower extremities. There is general instability. When walking, the patient excessively bends the legs at the knee and hip joints and lowers them to the floor with excessive force (punching gait). Often there is a feeling of walking on a thick carpet or wadding. The patient, with the help of vision control, tries to compensate for the violation motor function and therefore, when walking, he constantly looks at his feet. Control of vision noticeably reduces, and closing the eyes sharply increases the phenomena of ataxia. In case of severe damage to the posterior pillars, standing and walking are completely impossible.

Cerebellar ataxia observed with damage to the worm, hemispheres and cerebellar peduncles.
In the Romberg position or when walking, the patient deviates or falls towards the affected cerebellar hemisphere. With the defeat of the worm, a fall is observed in different directions, often backwards. When walking, the patient staggers, spreads his legs wide (drunk gait). The flank gait is sharply disturbed. The movements are awkward, sweeping. Slowing down and discoordination of movements are more pronounced on the side of the lesion. Vision control (closing and opening of the eyes) has relatively little effect on the severity of coordinating disorders. Speech is disturbed - it becomes slow, stretched, jerky, sometimes chanted. Changes in handwriting are manifested by its unevenness, sweeping, macrography often occurs. Usually, muscle tone decreases, more on the side of the lesion, sometimes tendon reflexes also decrease.

Cerebellar ataxia is seen in tumors vascular foci in the cerebellum and brain stem.

vestibular ataxia develops with damage to any part of the vestibular apparatus, which includes the labyrinth, the vestibular nerve, nuclei in the brain stem and the cortical center in temporal lobe brain.
Characteristic signs of vestibular ataxia: systemic dizziness (it seems to the patient that all objects are moving in a certain direction), horizontal nystagmus, nausea, vomiting. The patient staggers randomly to the sides or falls. Turning the head makes the dizziness worse. The caution with which patients make head movements is noticeable.
Vestibular ataxia is observed with (impaired circulation in the vertebral system), stem encephalitis, tumors of the IV ventricle of the brain,

Cortical ataxia develops mainly with damage to the frontal lobe of the brain due to dysfunction of the fronto-bridge-cerebellar system.
The leg contralateral to the focus suffers the most. Unsteadiness appears when walking, especially on turns, deviation in the direction opposite to the affected hemisphere. Visual control has relatively little effect on the degree of ataxia. In the case of severe lesions of the frontal lobe, the patient cannot stand and walk at all (astasia-abasia).

Frontal ataxia it is also accompanied by other symptoms of damage to the frontal lobe (change in the psyche, grasping reflex, impaired sense of smell).
Frontal ataxia is sometimes difficult to distinguish from cerebellar ataxia. In favor of the defeat of the cerebellum is the identification of evidence-based hypotension in the atactic limb. The most common cause of frontal ataxia is tumors,