Some diseases develop on their own, while others are formed in addition to some other diseases as a complication or an obligatory phenomenon. Most often, such secondary ailments require special treatment - elimination of symptoms and correction of the main cause of their occurrence. Ataxia is just such a disease, we will discuss it, the main symptoms and causes of treatment.
What is ataxia in humans?
Ataxia is inherently serious violation coordination of movements, which has nothing to do with muscle weakness. Such a pathological phenomenon can relate to the coordination of the limbs, and in some cases, almost the entire body. In addition, the disease can disrupt gait coordination and cause breathing and speech problems.
How is ataxia manifested, what are its symptoms?
There are several types of ataxia, which may differ in their manifestations. So the most common hereditary type of such ailment is considered to be Friedreich's ataxia. The first manifestations of such a disease become noticeable in adolescence or adolescence... They include noticeable awkwardness and some uncertainty while walking, weakness and atrophy of the leg muscles, and the onset of discoordination in the hands.
Often, such patients are faced with dysarthria, which is a violation of pronunciation due to a deficiency in the innervation of the speech apparatus. Over time, this ataxia leads to the development of diabetes mellitus, obesity, hypogonadism, cardiomyopathy, optic atrophy and cataracts, scoliosis, hollow foot, etc.
With the vestibular type of ataxia, the patient suffers from dizziness, which is often accompanied by nausea and vomiting, he is worried about nystagmus, and in some cases hearing loss. Symptoms increase if the patient turns his eyes or his torso, or the head takes a certain position. Characteristic feature vestibular ataxia - staggering while walking with a deviation in a certain direction.
A sensitive type of ataxia leads to impaired walking in the dark, the patient has difficulty maintaining balance, taking the Romberg position or simply closing his eyes. A patient with such an ailment walks with extreme caution, raising his leg high and lowering it to the surface of the floor with his entire sole.
Cortical actaxia leads to a change in gait, which becomes, as it were, "drunk". In the event that severe damage to the cerebral cortex occurs, the patient may suffer from paralysis, lose the ability to stand or walk. Also, the symptoms of this type of pathology include the appearance of olfactory and auditory hallucinations, mental changes, memory impairments and visual impairments.
Why does ataxia occur, what are the reasons for this?
There are many factors that can cause ataxia. Among such pathological conditions various ailments of the brain (tumors, injuries, defects, acute violation circulation, hydrocephalus, as well as demyelinating ailments). Also, ataxia can be triggered by diseases of the vestibular apparatus - labyrinthitis, vestibular neuronitis, neurinoma of the vestibular nerve.
In certain cases, the disease starts as a result of poisoning with hypnotics or potent medications, for example, benzodiazepines, barbiturates, etc. Experts say that it can also be caused by a serious vitamin B12 deficiency. In some cases, the disease is hereditary.
How is ataxia corrected, what is it?
Therapy of ataxia consists in correcting the causes of its development. So, if the ailment was provoked by disturbances in the activity of the brain or cerebellum, the doctor can carry out an operative - elimination of tumor formation, hemorrhage or abscess. Measures may also be taken to relieve pressure in the posterior area. cranial fossa, sometimes a decision is made to create an outflow from the cerebrospinal fluid.
In certain cases, ataxia therapy involves normalizing blood pressure, as well as taking drugs that optimize cerebral blood flow and metabolic processes. These drugs include angioprotectors, as well as nootropic drugs... Such measures help to cope with violations. cerebral circulation.
If infectious lesions of the brain or inner ear have been diagnosed, antibiotic therapy is performed. With demyelinating ailments, the patient has to take hormonal medications (steroids), and the plasmapheresis procedure is also indicated.
To eliminate poisoning, they resort to the introduction of solutions and different vitamins, especially B vitamins, ascorbic acid and provitamin A.
Deficiency of cyanocobalamin requires its timely administration.
Friedreich's ataxia can be corrected by using drugs that can support mitochondrial function, such drugs include tocopherol, coenzyme Q10, riboflavin, and succinic acid.
Symptomatic
Cerebellar ataxia- this is a whole complex of symptoms, which is characterized by impaired coordination of movements, tremors of the limbs and head, chanted speech. Treatment will entirely depend on what exactly caused the development of such a pathology. Therapy folk remedies impossible.
Cerebellar ataxia is diagnosed in children and adults alike. The diagnosis is made by physical examination and instrumental examination.
Unfortunately, it is impossible to completely eliminate this disease, and due to its rapid progression, the forecasts are extremely unfavorable. An exception is the case when early cerebellar ataxia is caused by an infectious disease.
Due to the fact that this disease has several forms based on the etiology of development, according to international classification diseases of the tenth revision, there are several ciphers for this ailment... Thus, according to ICD10, cerebellar ataxia has G00 - G99, G10 - G14, G11.
Etiology
Clinicians note that hereditary cerebellar ataxia is most often diagnosed, much less often an acquired form. The acquired form of the disease develops due to the following etiological factors:
- acute lack of vitamin B12;
- head injury;
- formation of benign or malignant;
- diseases of a viral or infectious nature;
- and diseases with a similar etiology and clinical picture;
- the body with heavy metals, toxic substances and poisons.
It is noted that most often a violation of this nature, if we talk about the acquired form, develops against the background of a stroke or head injury.
The hereditary form of such a pathology of the cerebellum is due to gene mutation. Why such a process takes place, scientists have not established, however, the fact genetic predisposition is not excluded.
Classification
The hereditary form of such a pathology of the cerebellum is considered in the form of the following forms:
- congenital non-progressive;
- Friedreich's ataxia (autosomal recessive);
- recessive with progressive cerebellar insufficiency;
- Batten's disease or congenital form with delayed development of the child, but its subsequent adaptation;
- Pierre Marie ataxia - refers to late pathologies, manifests itself after 25 years.
In addition, they consider the classification of the disease based on its development. Thus, cerebellar ataxia can be:
- acute - as a rule, it results from severe infectious or viral diseases;
- subacute - develops under the influence of an oncological process in the region of the brain or multiple sclerosis;
- chronically progressive;
- paroxysmal episodic.
To establish exactly what form of cerebellar ataxia occurs in a person, only a doctor can, by carrying out the necessary diagnostic measures.
Symptoms
Clinical picture progressive cerebellar ataxia is quite specific, therefore, as a rule, the diagnosis is not difficult.
except common symptoms, Friedreich's ataxia or any other form can be assumed by the behavior of the patient himself, namely:
- a specific posture of a person - he stands with his legs wide apart and his arms outstretched to the sides, as if trying to balance. At the same time, he tries not to turn his head and body;
- when trying to move his legs and with a simultaneous light push, the person falls arbitrarily, that is, without realizing it;
- the gait of a drunk person, legs are tense;
- when trying to make a turn, the patient falls, since the work of the vestibular apparatus is disrupted;
- the body is erect and tilted back a little.
As the progression progresses, the clinical picture will be complemented by the following symptoms:
- the patient loses the ability to change his movements;
- cannot touch the tip of the nose;
- speech deteriorates and handwriting changes, the patient's letter becomes sweeping and illegible;
- masked face;
- muscle tissue tone increases;
- pain in the back, neck and limbs;
- convulsions;
- observed,. In some cases, vision deteriorates;
- hearing acuity decreases;
- trouble swallowing food;
- violations psychological nature, depressive state.
With a congenital form of pathology, the following clinical picture may appear:
- the wrong balance of efforts to the performed action;
- vertical instability;
- nystagmus;
- the child begins to crawl and walk much later than the due date;
- speech "chopped" - the pronunciation of words is carried out strictly by syllables with a clear distinction;
- delay in speech and psychological development.
Due to the fact that a similar clinical picture can be present in other diseases associated with the vestibular apparatus and the brain, in the presence of such symptoms, an urgent need to consult a doctor. Similar symptoms in a child or an adult may also be present when a benign or malignant character is formed in the brain.
Diagnostics
Since the etiology of this ailment is quite wide range diseases and its congenital nature is not excluded, the diagnosis of an ailment implies the consultation of such specialists:
- neurosurgeon;
- neuropathologist;
- traumatologist;
- oncologist;
- endocrinologist.
The diagnostic program may include the following activities:
- lumbar puncture and cerebrospinal fluid analysis;
- functional tests;
- CT and MRI of the brain;
- dopplerography of the brain;
- electronystagmography;
- PCR studies;
- MSCT;
- DNA diagnostics.
In addition, you may need to consult a geneticist.
Treatment
Friedreich's ataxia and other congenital forms of this disease do not respond to therapy. Treatment will be aimed only at inhibiting development. pathological process and improving the patient's quality of life.
In this case, both conservative and radical methods therapy. Conservative ones include taking medication, special patient care, and physiotherapy.
Pharmaceutical treatment involves the use of such drugs:
- nootropics;
- muscle relaxants;
- substances to improve cerebral circulation;
- betagestin;
- anticonvulsants;
- to reduce muscle tone.
In addition, the course of treatment involves the following activities:
- massotherapy;
- physiotherapy;
- occupational therapy;
- physiotherapy procedures;
- a set of speech therapy exercises;
- classes with a psychotherapist.
Also, to facilitate the patient's life, special adaptive items can be selected - walking sticks, strollers, special beds, household items.
Unfortunately, no matter what type of treatment is chosen, it is impossible to completely eliminate the disease. With the right approach and strict implementation of all the recommendations of doctors, you can only slow down the progression of the pathological process and improve the quality of its life.
The prognosis will completely depend on what caused the development of such a pathology, in what form it manifests itself, and the patient's age and general clinical indicators are also taken into account.
Prophylaxis
Unfortunately, specific preventive measures regarding this pathology, no. However, you can minimize the risk of its development if you take into account the following recommendations of neurologists:
- it is necessary to carry out prevention of viral and infectious diseases in children, including vaccination when possible;
- if there is such a disease in the family, before conceiving a child, you need to consult a geneticist;
- closely related marriages with subsequent childbirth should be excluded.
At the very first clinical signs such an ailment, you need to contact a neurologist for advice and a subsequent full examination.
Is everything in the article correct from a medical point of view?
Only answer if you have proven medical knowledge
V recent times more and more often, such a phenomenon began to manifest itself as the genetic nature of the development of the disease, or in other words, the inability of the patient to influence the development of the disease on initial stage... Ataxia also belongs to such diseases, but what is ataxia and what types of this ailment occur, we will consider in this article.
Ataxia is characterized by impaired motor movement of a person and is a neuromuscular type of disease. The diagnosis of ataxia is made by a neurologist, since this ailment refers to diseases of the nervous system. In the course of 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, impaired coordination of movement.
In this case, the muscles do not lose their strength, or lose it slightly.
The reasons for the development of this ailment are associated primarily with the 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 are:
- inflammatory processes in the body ();
- drug use;
- conversion disorders;
- alcohol abuse;
- the presence of an infection in the body or against the background of an infectious disease;
- the presence of tumor processes;
- disruptions in the endocrine system.
Many experts believe that a violation of this kind does not arise as an independent type of disease, but as a consequence of a past or existing disease. That is, ataxia is one of the symptoms of some more severe and dangerous disease, about which the patient is not yet aware.
Classification of ataxia and its symptoms
Symptoms of ataxia differ greatly depending on the type of disease. Since with the defeat of one or another organ, individual manifestations are observed. This ailment has the following subspecies:
- Cerebellar.
- Vestibular.
- Cork.
Determination of ataxia by gait
In addition to the above subspecies of the disease, there are several more of its varieties, which in one way or another can be attributed to one of the main types, but have some features that allow them to be distinguished into a separate group, including:
- Pierre-Marie's cerebellar ataxia;
- familial ataxia of Friedreich (hereditary ataxia of Friedreich);
- telangiectasia ().
There are several other subspecies that will not be discussed in detail in this article, including:
Optical - a disease resulting from damage to the posterior parietal cortex of the brain. It is characterized by the inability of a person to touch any object at a distance of visibility due to the inconsistency of hand movements.
Intrapsychic ataxia - part of schizophrenia, is a violation of the psyche of the individual.
Despite the rich classification of this ailment, he has general symptoms, and the first symptoms include:
- violation of coordination of movement;
- nausea;
- vomit;
- involuntary muscle twitching;
- change of handwriting;
- slow speech;
- deterioration of vision;
- changes in mental state;
- the presence of hallucinations (auditory, visual);
- memory impairment;
- violation of facial expressions.
Often, the symptoms of this ailment are similar to those that occur with neurosis, as a result of which many confuse the first signs of the disease. In any case, if you suspect any deviations in the behavior of a potential patient, you do not need to pull, but you should consult a doctor as soon as possible.
Sensitive ataxia is the most common ailment and develops as a result of damage to the posterior columns spinal cord, namely, the pathways located there.
There are several types (severities) of the disease:
- defeat of the upper limbs with loss of coordination of movement;
- damage to the lower extremities with a loss of coordination and sensitivity in this area of the body;
- defeat of one of the limbs, with a loss of coordination and sensitivity;
- defeat of all limbs;
- loss of coordination of movement of the whole body (the case when the brain cannot determine how it is located relative to the earth's surface, as well as how arms and legs are located relative to each other).
In the case of the development of this type of disease, a person may feel imbalance, since it is often the lower extremities that are affected. Walking becomes visually dependent (lack of visual contact with the area where the foot will be placed makes movement extremely difficult). The patient may feel that walking is like moving on thick cardboard or cotton wool. The foot is placed on the floor with a characteristic pressure on the foot.
Affected spinal cord stem
In addition, the patient is unable to maintain balance in a position with arms and legs spread apart.
When testing a patient using the Romberg method, the result is negative.
Romberg's test is a method of determining the coordination of movement, which is expressed in observing the patient when he is in a pose that the doctor will indicate. Common Romberg Pose - Legs to the sides, arms forward, fingers outstretched. Difficult Pose - Legs are on the same line, with the heel of one foot touching the toe of the other, arms extended forward, toes spread out. The average time spent in this position with eyes closed is from 30 to 50 seconds without disturbing coordination.
The more severe the illness, the worse the ability to walk. On the most difficult stages such an opportunity may be completely lost.
Cerebellar
Cerebellar ataxia (sometimes called trunk ataxia) refers to the second major subspecies of this disease. The main difference between the disease and the sensitive type is that when the cerebellum is affected, the symptomatology extends to all limbs and the body as a whole, while in the sensitive type of the disease, only one limb can be affected.
The main symptoms this disease the following:
- change in gait, inability to stand upright;
- imbalance;
- tremor of the limbs;
- tight pronunciation of words with their noticeable separation;
- twitching eyes.
As the reasons for the development of this ailment, there are:
- poisoning medications(abuse or excessive long use the same medicine);
- cerebellar stroke ( this phenomenon dangerous not only with the risk of ataxia, but also with the danger of a lethal outcome);
- infectious diseases (encephalitis, chickenpox, etc.);
- cerebral palsy;
- traumatic brain injury.
The patient and the people around him will certainly notice that something is wrong with the patient. For example, the patient's gait will resemble a pendulum. He will move on straight legs swinging from side to side. When standing still with legs wide apart, if you sharply push one of the legs, the patient will fall and will not even notice it.
During the turns while walking, the patient may even fall.
Gradually, the patient ceases to understand in what sequence it is necessary to rearrange the legs and move the arms when walking. A kind of mask freezes on the face, which makes the patient looks like a drinking person. Speech slows down, handwriting deteriorates, squint and visual acuity may deteriorate. Develop gradually mental disorders and depression.
In children, this ailment can develop after 3 years, but the main age falls precisely on the interval from a year to three.
Pierre-Marie's cerebellar ataxia
This disease is one of the subspecies of cerebellar ataxia and belongs to hereditary diseases.
The likelihood of illness in relatives of the patient on the first line 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. Symptoms are the same as for normal 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 on its own, but only as a concomitant symptom.
The main manifestations of this disease are as follows:
- violation of coordination of movement and rest (standing or sitting);
- nausea;
- vomit;
- nystagmus;
Nystagmus - involuntary eye movement (twitching)
- sleep disturbance (dizziness can disturb the patient while lying down);
- dizziness;
- vegetative disorders (pallor or redness, tachycardia, pulse inconsistency).
The main reasons for the development of the vestibular type of ailment are as follows:
- damage to hair cells, as a result inflammatory processes in inner ear(otitis media, trauma, aerotitis, tumor, ear cholesteatoma)
Hair cells - auditory receptors
Cholesteatoma - a tumor neoplasm containing capsule-like growths
- damage to the 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 ailment and the rest is the dependence of symptoms on turning the head or trunk. Usually, these actions cause the patient maximum inconvenience, for this reason he tries to perform them smoothly and without sudden movements, in addition, with his eyes closed, he feels a violation of coordination of movement more strongly.
Since hair cells are usually affected on one side of the body, then the patient will be "mowed" to this side.
Cork
Cortical or frontal ataxia is one of the varieties of the disease, which is similar in many symptoms to cerebellar ataxia, but has its own characteristics.
Some scientific facts about ailment
The main symptoms of the progression of this disease, in addition to the general ones, are as follows:
- development of the grasping reflex;
- change in the psyche;
- violation of the sense of smell;
- falling back when walking;
- instability.
A patient with a cortical type of ailment moves along one line with a constant deviation of the body back.
The reason for the development 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 a hereditary disease that has almost all the symptoms characteristic of ataxia. The main difference of this disease from the rest is the way of its transmission - heredity.
Friedreich's ataxia can be of both cerebellar and sensitive types. The disease develops from 10 to 20 years old, there is a possibility of a later manifestation, but in rare cases.
The main symptoms are as follows:
- wobbly gait;
- stumbling and falling when walking;
- uncertainty when moving;
- hand tremor;
- change in handwriting;
- hearing impairment;
- deterioration and slowdown of speech;
- loss of the ability to serve oneself;
- the development of dementia;
- disorders of the nervous system (arrhythmia, the 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 in the body and heavy metals... 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 by the nature of its manifestation - dynamic and static
As a rule, most often you can find such a concept 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 patient's movement.
It is believed that the dynamic type of the disease is the most severe form of the disease, since the patient is unable 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. Then in this case the severity of the disease is lower than with the dynamic one. Symptoms are based on manifestations of imbalance when the patient's body is at rest (standing, sitting and even lying).
Diagnosis of ataxia
In order to correctly diagnose and differentiate different types of disease, it is used complex diagnostics, which includes several stages.
So, at the very first stage, the doctor needs to determine what type of disease, according to the type of course, the ailment belongs to. For this, a special neurological classification is applied:
- Sharp.
- Subacute.
- Progressive.
- Episodic.
Depending on the underlying disease and the nature of the manifestation of symptoms, conclusions can be drawn about this or that type of ailment. There may be several options, so the doctor never relies only on the results of any one study.
After the type of flow is established, the doctor begins to deal with the type of ailment directly, from the above classification. As a rule, complex neurological tests are used for this:
- Romberg's pose;
- finger tests;
- knee-calcaneal tests;
- test of lowering the hands;
- kickback symptom;
- pay attention to the presence of Burdzinsky's syndrome.
In addition to studying the patient's history and conducting various tests, prescribe instrumental research... Including:
- magnetic resonance imaging;
- general and clinical analysis blood;
- CT scan;
- Ultrasound of the brain;
- analysis of cerebrospinal fluid.
Additional tests may be ordered depending on the severity of the diagnosis.
Treatment and prognosis of ataxia
For the treatment of such a disease, the absence of delay is important, since it progresses and can flow into a more severe form.
Since the main cause of the onset of the disease is concomitant illness, 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 only to reduce the symptoms and alleviate further life person. However, this does not mean that it is impossible to completely get rid of it. How to cure an unpleasant ailment forever?
First of all, by completely curing the underlying disease. However, if the development of the disease is neglected, the chances of permanently getting rid of ataxia are slim.
- massotherapy;
- curative physical education(Exercise 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. At severe forms it is possible to limit the patient's employment, in case of a favorable outcome, the probability of the patients surviving up to deep old age... At mild form and exceptions re-development infectious diseases, it is possible to get rid of the disease completely.
Treatment of this ailment with folk remedies, and even without the supervision of a specialist, is an extremely unreasonable decision and our editors are sharply opposed to 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, swinging limbs).
- Developing accuracy (the main condition is to hit the specified plane with your hand or foot, gradually the complexity of the tasks increases, weights are added).
- Developing consistency in the joints (consists in setting tasks for lifting or moving various objects, and in this work joints should be involved, gradually it is possible to use weights during these exercises).
- To stimulate ballistics (throwing, pushing or imitation of such an activity, each time the throw distance increases).
- To improve balance (exercises to improve balance with additional movements, standing, sitting and walking, you can gradually introduce the use of bars for these purposes).
- Training eye muscles (Fixation with the eyes of a certain point, which is followed during tilting the head or turning, as well as in motion).
- To develop a muscular-articular feeling (guessing an object by sensations with closed eyes).
It is believed that it is possible to use such a device as a balancer for children, however, only a doctor can prescribe it, in no case do not self-medicate, as you can injure the baby.
Prophylaxis
Prevention is understood as a set of measures that exclude the further spread of the disease and the exclusion of its occurrence in healthy people.
- exclusion of related marriages;
- exclusion of pregnancy in families with ataxia;
- timely treatment of infectious diseases;
- preventing the accumulation of toxic and heavy metals in the body;
- conducting healthy way life;
- adherence to sleep, nutrition;
- timely appeal to a specialist for help.
So ataxia is serious concomitant disease, which requires compulsory medical intervention. The longer the patient drags on to see a specialist, the less chances he has to fully recover from such a disease, moreover, the underlying disease can even kill him. Take care of yourself, do not self-medicate and get treatment from the right doctors!
Ataxia(from the Greek. ataxia - disorder) - movement coordination disorder; a very common violation of motor skills. Strength in the limbs is slightly reduced or completely retained. The movements become inaccurate, awkward, their continuity and consistency is upset, balance in the standing position and when walking is disturbed. Static ataxia - imbalance in a standing position, dynamic ataxia - impaired coordination during movement. Diagnosis of ataxia includes neurological examination, EEG, EMG, MRI of the brain, and if a 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
Ataxia(from the Greek. ataxia - disorder) - movement coordination disorder; a very common violation of motor skills. Strength in the limbs is slightly reduced or completely retained. The movements become inaccurate, awkward, their continuity and consistency is upset, balance in the standing position and when walking is disturbed. Static ataxia - imbalance in the standing position, dynamic ataxia - impaired coordination during movement.
Normal coordination of movements is possible only with highly automated and friendly activity of a number of parts of the central nervous system - conductors of deep muscle sensitivity, the vestibular apparatus, the cortex of the temporal and frontal regions and the cerebellum - the central organ for coordinating movements.
Classification of ataxia
Symptoms of ataxia
Emergence sensitive ataxia due to damage to the posterior columns (Gaulle and Burdach's bundles), less often the posterior nerves, peripheral nodes, the cortex of the parietal lobe of the brain, the optic tubercle (funicular myelosis, tabes dorsal, vascular disorders). Its manifestation is possible, both in all limbs, and in one leg or arm. The most indicative are the phenomena of sensitive ataxia resulting from disorders of the joint-muscular feeling in lower limbs... The patient is unstable, when walking excessively bends the legs in the hip and knee joints, steps too hard on the floor (stamping gait). Often there is a sensation of walking on cotton wool or carpet. Patients try to compensate for the disorder motor functions with the help of sight - when walking, they constantly look at their feet. This allows you to noticeably reduce the manifestations of ataxia, and closing the eyes, on the contrary, aggravates them. Severe lesions of the posterior columns make it virtually impossible to stand and walk.
Cerebellar ataxia- a consequence of the defeat of the cerebellar worm, its hemispheres and legs. In the Romberg position and when walking, the patient falls (up to a fall) towards the affected cerebellar hemisphere. In case of damage to the cerebellar vermis, it is possible to fall in any direction or backwards. The patient staggers when walking, puts his legs 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 is almost invariable with control of vision (open and closed eyes). There is a violation of speech - it slows down, becomes stretched, jerky, often chanted. The handwriting becomes sweeping, uneven, macrography is observed. A decrease in muscle tone is possible (to a greater extent on the side of the lesion), as well as a violation of tendon reflexes. Cerebellar ataxia can be a symptom of encephalitis different etiology, 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 frontocerebellar system. With frontal ataxia, the leg, contralateral to the affected cerebellar hemisphere, suffers to the maximum extent. When walking, there is instability (mostly when cornering), tilting or falling to the side, ipsilateral to the affected hemisphere. With severe lesions of the frontal lobe, patients cannot walk and stand at all. Vision control does not in any way affect the severity of disturbances when walking. Cortical ataxia is also characterized by other symptoms characteristic of lesions of the frontal lobe - grasping reflex, mental changes, impaired sense of smell. The symptom complex of frontal ataxia is very similar to cerebellar ataxia. The main difference between cerebellar lesions is evidence-based hypotension in the atactic limb. The causes of frontal ataxia are abscesses, tumors, and cerebrovascular accidents.
Hereditary cerebellar Pierre-Marie ataxia- 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; generation skipping is very rare. A characteristic pathological 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 of the spinal systems (the clinical picture resembles Friedreich's spinocerebellar ataxia).
The average age of the onset of the disease is 35 years, when gait disturbance appears. Subsequently, he 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 twitching is possible. Strength in the muscles of the limbs is reduced. Progressive oculomotor disorders are observed - paresis of the abducens nerve, ptosis, failure of convention, less often - Argyll Robertson's symptom, atrophy of the optic nerves, decreased visual acuity, narrowing of the visual fields. Mental disorders are manifested in the form of depression, decreased intelligence.
Friedreich's family ataxia- 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. Consanguineous marriages are very common among the parents of patients. A characteristic pathological sign of Friedreich's ataxia is increasing degeneration of the lateral and posterior columns of the spinal cord (up to medulla oblongata). Gaulle's beams 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 boldly, deviating from the center to the sides and placing his legs wide. Charcot designated such a gait as tabetic-cerebellar. With the development of the disease, discoordination spreads to the arms, 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 impaired.
With the development of Friedreich's ataxia, extraneural disorders appear - heart damage and skeletal changes. On the ECG - deformation of the atrial tooth, rhythm disturbance. Observed paroxysmal pain in the heart, tachycardia, shortness of breath (as a result of physical exertion). Skeleton changes are expressed in characteristic change the shape of the foot - a tendency to frequent dislocation of the joints, an increase in the arch and extension of the fingers, as well as kyphoscoliosis. Among the endocrine disorders accompanying Friedreich's ataxia, diabetes, hypogonadism, and infantilism are noted.
Ataxia-telangiectasia(Louis-Bar syndrome) is a hereditary disease (group of phakomatoses) transmitted in an autosomal recessive manner. Very often accompanied by dysgammaglobulinemia and hypoplasia thymus... The development of the disease begins early childhood when the first ataxic disorders appear. In the future, ataxia progresses and walking is almost impossible by the age of 10. Often Louis-Bar syndrome is accompanied by extrapyramidal symptoms(hyperkinesis of the myoclonic and athetoid type, hypokinesia), mental retardation, defeat 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, there is a high risk of malignant neoplasms.
Complications of ataxia
Diagnosis of ataxia
The diagnosis of ataxia is based on the identification of the disease 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 disorders: diffuse delta and theta activity, reduction of the alpha rhythm. V laboratory research there is a violation of the metabolism of amino acids (the concentration of leucine and alanine is reduced, and 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. With the help of electromyography, axonal-demyelinating damage to the sensory fibers of peripheral nerves is revealed.
When differentiating ataxia, it is necessary to take into account the variability of the clinical picture of ataxia. V clinical practice there are rudimentary varieties of ataxia and its transitional forms, when clinical manifestations are similar to the symptoms of familial paraplegia (spastic), neural amyotrophy and multiple sclerosis.
To diagnose hereditary ataxias, it is necessary to carry out direct or indirect DNA diagnostics. With the help of molecular genetic methods, ataxia is diagnosed in a patient, after which an indirect DNA diagnosis is performed. With its help, the possibility of inheritance of the ataxia pathogen by other children in the family is established. It is possible to carry out complex DNA diagnostics, for it you will need the biomaterial of all family members (biological parents of the child and all other children of this parental couple). In rare cases, prenatal DNA diagnosis 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 drugs); special complex gymnastic exercises Exercise therapy aimed at strengthening muscles and reducing discoordination. In Friedreich's ataxia, given the pathogenesis of the disease, drugs that support the functions of mitochondria can play an important role in 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 it is contraindicated, in addition, excessive X-rays and prolonged exposure to the sun should be avoided.
Genomic prediction hereditary diseases unpleasant. There is a slow progression neuropsychiatric disorders... The ability to work in most cases is reduced. However, thanks to symptomatic treatment and the prevention of repeated infectious diseases, injuries and intoxications, patients have the opportunity to live to old age. WITH preventive purpose children should be avoided in families where there are sick hereditary ataxia... In addition, it is recommended to exclude the possibility of any kindred marriages.
Ataxia (or incoordination) is a violation of the coordination of movements caused by damage to neuromuscular tissues. This disease is considered a common pathology, which includes impaired motor skills (imprecision, clumsiness of movements) and balance.
Occurs in both sexes and at any age.
Load and set of exercises for this treatment is established by the doctor in accordance with the type of disease, its severity, damaged limb and organ and the individual characteristics of the patient's body.
Despite the great advances in medicine in this area, the prognosis for patients with ataxia often remains uncomfortable. But avoiding intoxication, injury and other factors that negatively affect nervous system, treatment can bring tremendous success up to full recovery.
To reduce the risk of ataxia, excessive alcohol abuse should be ruled out and drugs should be completely abandoned.
You should also minimize the risk of injury to the nervous system.
The neurologist will tell you more about ataxia in this video:
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