Cerebellar ataxia in humans: how to treat it. Cerebellar ataxia: symptoms, treatment, other types of ataxia.
Cerebellar ataxia . Movement coordination disorders due to a lesion and (or) its connections are one of the most common groups of neurological symptoms. The spectrum of neurological pathology associated with cerebellar disorders is extremely diverse. It could be neurological diseases with predominant (even isolated) damage to the cerebellum and its connections, which primarily include hereditary and sporadic forms of a number of degenerative diseases, as well as cerebellar tumors.
Other simple diagnostic examination carried out by the doctor is as follows: the patient, in an upright position, must first extend his hand, then touching the tip of his nose with index finger. The ataxia sufferer is unable to perform this simple action: Typically, the patient's finger hits the cheek or "Another part of the face. In some cases" the action is successful, but the ataxic patient uses obvious effort and a certain uncertainty during the movement. Clinical diagnosis can be confirmed by "possible molecular tests: in general, transmission nerve signals normally or, in other cases, only slightly slowed down, in contrast, however, the potential d" action of the sensitive type, in which the damage is very obvious.
Disorders coordination of movements may be the first and dominant, and sometimes the only focal neurological symptom in this pathology.
Cerebellar disorders often detected in many other CNS diseases ( acute disorders cerebral circulation in the vertebral-basilar basin, discirculatory encephalopathy, multiple sclerosis, volumetric pathological processes trunk and bark cerebral hemispheres, consequences of severe head injury and meningoencephalitis, multiple systemic degenerations, etc.), although they are usually characterized by a combination of cerebellar symptoms with other neurological symptoms and syndromes (for example, some alternating syndromes and cerebellopontine angle syndrome with focal pathology of the brainstem).
However, the potential and auditory brain stems are degenerated and damaged as ataxic disease advances. Other useful remedies diagnostics At the moment there is no effective drug therapy in ataxia of a neurological type of muscle: in this regard, rather than talking about real therapy, it should be understood as rehabilitation, which has the goals to restore motor changes, to monitor pathological kinetic movements and, above all, to increase “self-sufficiency and” sense of self patient's dignity.
They are neurologists, physical therapists and orthopedic surgeons, three figures managing ataxic patients who do not want to stop fighting and driven by the desire to defeat the disease, or who strive to at least intervene in the degenerative evolution of the same. With physical exercise and willpower, the patient's quality of life may be better than ataxic, due to increased motor activity and movement coordination.
IN similar cases it is especially important to establish lateralization of coordination disorders and the features of their symptomatology, since all this significantly facilitates the formulation of a topical diagnosis.
Main symptom, indicating damage to the cerebellum or its connections, is cerebellar ataxia - incoordination of movements caused by the collapse of the synergistic (coordinated) functioning of the muscles of the trunk, limbs and parts of the muscles innervated cranial nerves(bulbar, masticatory, oculomotor).
Friedreich's ataxia is an ataxic form caused by frataxin deficiency, the cause of which lies in a gene mutation. Frataxin deficiency causes inevitable neuronal degeneration. In addition, deficiency of this important protein causes hypersensitivity to oxidative stress, in turn caused by the accumulation of mitochondrial iron, especially in the heart. This brief introduction is necessary to understand why they have proposed some innovative therapeutic strategies based on the administration of iron enterosorbents and antioxidants. it is only right to point out that such therapies may be useful in modulating symptoms, and may hypothetically improve the living conditions of patients, even dramatically, however, neither is the administration.
To obligate symptoms Damage to the cerebellum also includes diffuse muscle hypotonia, manifested by a decrease in the tonic angle when flexing the limbs and excessive hyperextension in the elbow and knee joints.
It is customary to distinguish two types of cerebellar ataxia- static and dynamic.
Main mechanism of static ataxia is a violation of the anti-gravity function of the cerebellum, which leads to a violation of the ratio of the area of support and the angular vector of gravity both when standing and when walking, as well as during a sudden change in posture (for example, normally during sudden braking of a vehicle). As a result, the support reaction and the jump reaction are disrupted, and when standing, disproportionate tension in the leg muscles and incoordination when walking are observed. Clinical symptoms Static cerebellar ataxia includes disturbances in standing and walking, as well as cerebellar asynergies.
The term ataxia describes a lack of muscle coordination during voluntary movements, such as walking or picking up objects. Atassia can affect movement, speech, eye movements, and your ability to swallow. Permanent ataxia is usually caused by damage to the cerebellum, the part of the brain that controls muscle coordination. Many conditions can cause ataxia, including alcohol abuse, stroke, tumors, cerebral palsy and multiple sclerosis. It is also possible to inherit a faulty gene that can cause one of many variants of ataxia.
1. Impaired standing and walking. Damage to the anterior part of the cerebellar vermis results in a predominantly forward fall. Damage to the middle part of the worm is often accompanied by trunk ataxia, manifested by swaying when walking and standing. Hitting the back of the worm usually results in backward falls. Along with falls, peculiarities of standing are also observed: the patient stands with his legs widely spaced and at the same time balances with his arms. Characterized by a “drunk gait” and difficulty turning (the patient “slides” to the side or falls).
Treatment for atasia depends on the underlying cause. You may receive physical therapy, occupational therapy, and speech therapy. Atassia may develop over time or suddenly, depending on the cause. Atassia is actually a symptom of a number of neurological disorders that can cause.
- Walking that becomes unsteady and then causes you to stumble.
- Difficulty eating, writing, or buttoning a shirt.
- Changing the way you speak.
- Unintentional eye movements.
To identify standing and walking disorders varying degrees severity caused by static cerebellar ataxia, the following basic tests can be used:
Romberg pose- the patient stands in the “heels and toes together” position with arms extended forward, fingers spread, eyes open (then closed). In the complicated (sensitized) Romberg position, the patient places one leg in front of the other in a straight line. Static cerebellar ataxia is characterized by falls or instability in the Romberg position with both eyes open and closed.
Degeneration or loss nerve cells in the part of the brain that controls muscle coordination, leading to loss of coordination or ataxia. The cerebellum is made up of two pieces of folded tissue located at the base of the brain near the brain stem. Right side cerebellum controls coordination right side body, the left side of the cerebellum controls coordination on the left side of the body. Diseases that damage the spinal cord and the peripheral nerves that connect the cerebellum to the muscles can cause ataxia. Brain damage or spinal cord caused by a head shot, as can occur in a motor vehicle accident, can cause sudden onset ataxia, also known as acute cerebellar ataxia. When the blood supply to part of the brain is interrupted or significantly reduced, brain cells begin to die. This is a general term for a group of diseases that cause damage to a child's brain. Multiple sclerosis is a chronic, potentially debilitating disease affecting the central nervous system, which includes the brain and spinal cord. These are rare degenerative pathologies caused by a reaction immune system for a tumor. Ataxia may appear months or years before cancer is diagnosed. Atassia is side effect certain medications such as barbiturates such as phenobarbital and sedatives such as benzodiazepines. Alcohol and drug intoxication; Toxin poisoning heavy metals and solvents can cause ataxia. This is known as sporadic degenerative ataxia, which can take various shapes, including multiple system atrophy, a progressive degenerative disease.
- Muscle coordination is lost in the arm, arm or leg.
- You have problems walking.
- Damage.
- Transient ischemic attack.
- Ashya may be a rare complication chickenpox and other viral infections.
- Cancer growth can damage the cerebellum.
Standing and walking in a straight line. They become almost impossible, since when walking the patient is forced to spread his legs wide apart or he deviates (falls) towards the predominance of static ataxia.
« Flank walking“- with static cerebellar ataxia, when walking with extended steps, the patient seems to be dancing, the body lags behind the limbs, an abrupt stop is impossible (the patient is “thrown” in the direction of the prevailing static ataxia).
Abnormal proteins interfere with the ability of nerve cells, especially in the cerebellum and spinal cord, and cause them to degenerate over time. Genetic ataxia can be inherited either by a dominant gene from a parent or by a recessive gene from each parent. IN the latter case It is possible that neither parent became ill, so there may not be a history of obvious familial attrition.
Autosomal dominant atassia, including. Autosomal recessive atassia, including. This neurological disorder involves damage to the cerebellum, spinal cord and peripheral nerves. Muscles weaken and warm over time, causing deformities, especially in the legs, feet and arms. This rare infantile disease causes progressive degeneration of the brain and other body systems. The disease leads to the destruction of the immune system, which increases susceptibility to other diseases. This type refers to ataxia, which results from damage to the cerebellum, which is present at birth. People with this condition accumulate copper in the brain, liver and other organs, which can cause neurological problems including ataxia.
- The first indication is usually difficulty walking.
- The condition usually progresses towards the arms and trunk.
- Signs and symptoms usually appear around age 10.
- Congenital cerebellum kali.
Panov's test (“stars”). It is used in clinical practice to determine the lateralization of cerebellar disorders in patients with mild static ataxia who can walk independently. The technique is that the patient sequentially performs three steps forward and three steps back, first with open and then with closed eyes. After closing the eyes, a patient with cerebellar disorders seems to turn towards the lateralization of the cerebellar disorders.
Some blood tests can confirm or rule out a suspicious condition. Microscopic examination of a urine sample may reveal some systemic abnormalities that may be associated with some forms of ataxia. A CT scan or magnetic resonance imaging scan of the brain can help identify potential causes. The needle is inserted into bottom part lumbar bones to remove the sample cerebrospinal fluid. A doctor may recommend genetic testing to determine whether a gene mutation is present that causes one of the atasia conditions. In some cases, dealing with the underlying cause resolves the ataxia.
2. Positive tests for cerebellar asynergies. As mentioned above, the occurrence of cerebellar asynergies in cerebellar ataxia is associated with a violation of synergistic muscle contractions mainly in the muscles of the trunk and limbs. Static cerebellar ataxia is accompanied by the following positive asynergy tests:
Your doctor may recommend help or therapy to help treat the disease. There may be no treatment for ashyas caused by conditions such as multiple sclerosis or cerebral palsy. In this case, your doctor can tell you.
- Edited food tools.
- Special computers or other devices to help you talk.
Occupational therapy to help with daily activities such as eating.
- Physical therapy to help improve strength and mobility.
- Speech to improve language and help swallowing.
Babinski's asynergy in the supine position. The patient is asked to sit up sharply from a lying position with his arms crossed over his chest, which is normally accompanied by a synergistic contraction of part of the trunk muscles and the posterior group of thigh muscles (Babinsky's physiological synergy in the supine position). With static cerebellar ataxia, such synergies disintegrate, which leads to the inability to sit up from a lying position due to the patient falling backwards, as well as raising the leg(s) on the side of the lateralization of cerebellar disorders.
All of these disease processes manifest with some fundamental symptoms that express the loss of the regulating, coordinating "modulating" function performed by the cerebellum in and on voluntary movements. It is a pathology that causes loss of motor coordination due to problems arising from the brain and nervous system.
The parts affected by ashtrays are included. Lower limbs; Upper limbs; Tongue and mouth; eyes. . Once these parts of the body are affected, it is obvious that the consequences will be remarkable in terms of. Walking; talk; Martin; perform basic tasks such as eating and writing. Hereditary, when symptoms occur over many years and are associated with genes; Acute, when symptoms occur suddenly due to a blow, for example. At the time of conception, each child will have two pairs of chromosomes, inherited by the father and one by the mother, but it may be that genetic transmission can lead to changes in genes that lead to disease development, as in this particular case of ataxia.
Babinski's asynergy in a standing position. The standing patient is asked to bend back, throwing back his head. Normally, this action is accompanied by flexion at the knees and extension at the knees. hip joints(physiological synergy of Babinsky in a standing position). With a positive symptom, such synergies disintegrate, which leads to the patient falling backwards.
Positive Orzechowski test. A sitting or standing patient is asked to firmly rest the palms of his outstretched arms on the palms of the doctor, who then sharply moves them down. In this case, a synergistic contraction of part of the trunk and limb muscles normally occurs, and as a result the patient leans back or remains motionless. Positive symptom due to the collapse of this synergy, the patient falls forward.
Positive Stewart-Holmes test, or phenomenon of no pushback. The patient bends forcefully elbow joint hand, and the doctor counteracts this movement. Then the opposition suddenly stops, which healthy person accompanied by a so-called reverse push due to contraction of antagonist muscles. In the presence of a positive test, synergistic contraction of the extensor muscles does not occur, as a result of which the patient punches his chest with force.
Ataxia is a violation of coordination of movements and motor skills.
With this disease, strength in the limbs is slightly reduced or completely preserved. Movements are imprecise, awkward, their sequence is disrupted, and it is difficult to maintain balance when walking or standing.
If balance is disturbed only in a standing position, they speak of static ataxia.
If coordination problems are observed during movement, then this is dynamic ataxia.
Types of ataxia
There are several types of ataxia:
- Sensitive. The occurrence of the disorder is caused by damage to the posterior columns, less often - to the posterior nerves, peripheral nodes, cortex of the parietal lobe, thalamus. The disease can occur in all limbs, or in just one arm or leg. Sensitive ataxia manifests itself most clearly in the disorder of joint-muscular sensations in the legs. The patient has an unsteady gait, bends his knees excessively or steps too hard on the floor when walking. Sometimes it feels like walking on a soft surface. Patients compensate for the violation motor function through vision - when walking, they constantly look at their feet. Severe lesions of the posterior columns render the patient virtually unable to stand or walk.
- Vestibular. Develops when one of the sections of the vestibular apparatus is damaged. The main symptom is systemic dizziness. It seems to the patient that surrounding objects are moving in one direction; when turning the head, this sensation intensifies. The patient avoids sudden head movements and may stagger erratically or fall. Vestibular ataxia may cause nausea and vomiting. The disease accompanies brainstem encephalitis, tumor of the fourth ventricle of the brain, Meniere's syndrome.
- Cortical. Appears when the frontal lobe of the brain is damaged, caused by dysfunction of the fronto-pontocerebellar system. In this condition, the leg opposite the affected hemisphere suffers the most. Walking is characterized by instability (especially when turning), bending or leaning on one side. At severe defeat the patient cannot walk or stand. This type of ataxia is also characterized by the following symptoms: impaired sense of smell, changes in the psyche, and the appearance of a grasping reflex.
- Cerebellar. With this type of ataxia, the cerebellum, its hemispheres or legs are affected. When walking, the patient falls over or falls. When the cerebellar vermis is damaged, the fall occurs to the side or backward. The patient staggers when walking and spreads his legs wide. Movements are sweeping, slow and awkward. Speech function may also be impaired - speech becomes slow and drawn out. The patient's handwriting is splayed and uneven. Sometimes there is decreased muscle tone.
Cerebellar ataxia manifests itself in malignant tumors.
Features of cerebellar ataxia syndrome
The main difference between cerebellar lesions is evidence of hypotonia in the ataxic limb(decreased muscle tone). At 
In cerebellar disorders, symptoms are never limited to a specific muscle, muscle group, or individual movement.
It has a widespread character.
Cerebellar ataxia has characteristic symptoms:
- impaired gait and standing;
- impaired coordination of limbs;
- intention tremor;
- slow speech with separate pronunciation of words;
- involuntary oscillatory eye movements;
- decreased muscle tone.
Hereditary ataxia of Pierre Marie
Cerebellar ataxia of Pierre Marie - hereditary disease, characterized by a progressive nature.
The likelihood of the disease manifesting itself is high - skipping generations is extremely rare. A characteristic pathological feature is cerebellar hypoplasia, which is often combined with degeneration of the spinal systems.
The disease most often begins to manifest itself at the age of 35 in the form of gait disturbances. Then it is joined by speech and facial expression disorders, and ataxia of the upper extremities.
Tendon reflexes are increased, and involuntary muscle twitches occur. Strength in the limbs decreases, oculomotor disorders progress. Mental disorders manifest themselves in the form of depression and decreased mental activity.
What causes the pathology?
The causes of the disease may be:
- Drug intoxication(lithium drugs, antiepileptic drugs, benzodiazepines), toxic substances. The disease is accompanied by drowsiness and confusion.
- Cerebellar stroke, early detection which can save the patient's life. The disorder occurs due to thrombosis or embolism of the cerebellar arteries.
- Manifests itself as hemiataxia and decreased muscle tone on the affected side, headache, dizziness, impaired mobility eyeballs, impaired facial sensitivity on the affected side, weakness of facial muscles.
- Cerebellar hemiataxia may be a consequence of developing medulla oblongata infarction, caused by .
- Infectious diseases. Cerebellar ataxia often accompanies viral encephalitis and cerebellar abscess. IN childhood after viral infection(for example, after chickenpox), acute cerebellar ataxia may develop, which is manifested by gait disturbance. This condition usually ends full recovery for several months.
In addition, pathology can be a consequence of:
- lack of vitamin B12.
The manifestation of the disease cannot be missed
The main symptom of cerebellar ataxia is static violations. The characteristic posture of the patient in a standing position is legs wide apart, balancing with arms, avoiding turns and bending of the body.
If you push the patient or move his leg, he will fall without even noticing that he is falling.
With dynamic disorders, the disease manifests itself in the form of a peculiar gait (sometimes called “cerebellar”). When walking, the legs are wide apart and tense, the patient tries not to bend them.
The body is straight, slightly thrown back. When turning, the patient falls over and sometimes even falls. When rearranging the legs, a wide amplitude of swing is observed.
Adiadochokinesis appears over time– the patient cannot alternate movements (for example, alternately touch the tip of the nose with his fingers).
Speech function is impaired, handwriting deteriorates, the face acquires mask-like features (due to impaired facial expressions). Such patients are often mistaken for drunks, which is why medical assistance is not provided on time.
Patients complain of pain in the legs and arms, lower back, and neck. Muscle tone is increased, convulsive twitches may appear.
Ptosis, convergent strabismus, and dizziness when trying to focus the gaze often occur. Visual acuity decreases, mental disorders and depression.
Diagnostics
When conducting a full range of examinations, making a diagnosis does not cause difficulties.
Required to be taken functional test, assessment of tendon reflexes, blood and cerebrospinal fluid examination.
Appointed computed tomography and ultrasound of the brain, anamnesis is collected.
It is important to start treatment on time!
Cerebellar ataxia is a serious disorder that requires immediate action; treatment is carried out by a neurologist.
It is most often symptomatic and covers the following areas:
- restorative therapy (prescription of cholinesterase inhibitors, Cerebrolysin, B vitamins);
- physiotherapy, the purpose of which is to prevent various complications (muscle atrophy, contracture), improve gait and coordination, and maintain physical fitness.
Training complex
Appointed gymnastics complex, consisting of exercise therapy exercises.
The goal of the training is to reduce incoordination and strengthen muscles.
At surgical treatment(elimination of cerebellar tumors) one can expect partial or full recovery or stopping the development of the disease.
In this case, drugs aimed at maintaining mitochondrial functions (vitamin E, Riboflavin) play an important role.
If the pathology is a consequence, in mandatory treatment for this infection is prescribed. The doctor prescribes drugs that improve cerebral circulation, nootropics, betahistine.
A special massage helps reduce the severity of symptoms.
The consequences can be the saddest
Complications of ataxia may include:
- tendency to recurrent infections;
- chronic heart failure;
- respiratory failure.
Forecast
It is impossible to completely cure cerebellar ataxia.
The prognosis is rather unfavorable, since the disease tends to progress quickly. The disease significantly impairs the quality of life and leads to numerous organ disorders.
Cerebellar ataxia is very serious illness. At the first symptoms, you must immediately contact a specialist for a comprehensive examination.
If the diagnosis is confirmed, timely adoption of measures will significantly alleviate the patient’s condition in the future and slow down the progression of the disease.