Gait is a combination of individual characteristics of a person (body tilt, position of arms and legs, head rotation, etc.). To carry out the walking process, mechanisms are involved that regulate balance, maintaining posture and coordination of movements. One such mechanism is the deep sensitivity pathway. It is a reflex arc that closes at different levels of the central nervous system. The nerve endings of the conductors of deep sensitivity are located deep in the muscles, tendons and skin. They carry information to the spinal cord and brain about the position of the body in space, the feeling of pressure and vibration. When the nerve fibers responsible for deep types of sensitivity are damaged, sensory ataxia develops. The term ataxia is used to describe a lack of motor coordination.
Is this ataxia?
Ataxia (from the Greek ataxia - disorder, chaoticity) is a violation of movement coordination that is not accompanied by paralysis and paresis and is characterized by loss of balance at rest and/or while walking, and is also sometimes combined with symptoms such as blurred vision, disorder letters, changes in the patient’s speech, etc.
Movements in patients suffering from ataxia become excessively sweeping, awkward, and the normal sequence of movements is disrupted. This significantly impairs their life activity, prevents them from performing any work and causes disability.
Often, the diagnosis of some types of ataxia is difficult due to the lack of highly specific research methods. Treatment of ataxia is directly related to the cause of its occurrence and in most cases is symptomatic.
What is ataxia in adults, pregnant women and children
Ataxia is a lack of coordination of movements. The name of the disease comes from the Greek word ataxia - disorder. Patients with this pathology may indeed experience chaotic movements both when walking and when trying to move their fingers, etc. The person begins to complain about the inability to maintain balance and the appearance of awkwardness and inaccuracy while performing any actions. Ataxia can develop at any age, including in young children. In pregnant women, the severity of the disease in some cases may increase, and then additional examinations and more careful monitoring of the functioning of the heart and respiratory system are required.
Coordination is a very delicate process that depends on the coordinated work of certain parts of the central nervous system: the cerebellum, the cortex of the temporal and frontal lobes, the vestibular apparatus and conductors of deep muscle sensitivity. If at least one link of this chain is damaged, a person experiences various disturbances in the coordination of movements of body parts.
Patients with ataxia have poor coordination of movements and sometimes find it difficult to maintain their body in a standing position.
In people with ataxia, there is a discrepancy in the actions of different muscles, which leads to the impossibility of full coordination. This causes many problems in everyday life, sometimes it becomes almost impossible to go outside and generally exist independently. Sometimes even strength in the upper and lower extremities decreases.
Video about coordination, ataxia and methods of its treatment
Types of ataxia
Depending on the affected part of the nervous system, ataxia is divided into:
- cerebellar ataxia (occurs as a result of degenerative processes in the hemispheres or the cerebellar vermis);
- sensitive ataxia (appears when peripheral nerves are damaged - polyneuropathies or when the posterior columns of the spinal cord are damaged);
- vestibular ataxia (is a manifestation of dysfunction of the vestibular apparatus or vestibular nerve);
- cortical or frontal ataxia (occurs when the frontal lobes of the brain are damaged, less often - the temporo-occipital regions).
Also, depending on the manifestation, that is, whether ataxia appears during movement or at rest, ataxia is distinguished between dynamic and static, respectively.
Term designation
Sensitive ataxia is a disease that is manifested by a violation of the sensory sensation of pressure, vibration responses and body position in space, causing motor disturbances and incoordination. The characteristic symptoms of the anomaly are: a disorder of standard gait and coordination, low blood pressure in the skeletal muscle regions, uncontrolled movements of the distal areas of the arms. Deviations from the norm in medical institutions are identified in the presence of specific neurological data. The main diagnostic methods include the collection of blood cells for biochemical testing, neurophysiological testing, computed tomography or magnetic resonance imaging of the brain or spinal cord, and genetic testing. Neurometabolic techniques are prescribed as therapeutic therapy, since the etiotropic method is not effective. Patients are prescribed anticholinesterase medications, vitamin complexes, ATP, massage sessions, etc.
Causes of the disease
Cerebellar ataxias
Cerebellar ataxias are the largest group of ataxias. They arise as a result of damage to the cerebellum or disruption of its connections with the cerebral hemispheres. Depending on the rate of development and progression, they are divided into acute, subacute and chronic cerebellar ataxias.
Causes of acute cerebellar ataxia
These include:
- strokes localized in the cerebellum;
- infectious diseases;
- post-infectious complications (abscesses, encephalomyelitis, etc.);
- poisoning by certain groups of drugs (lithium salts, anticholinesterase drugs - Physostigmine, Proserin).
Causes of subacute cerebellar ataxia
Subacute cerebellar ataxia appears as a result of the following diseases:
- multiple sclerosis;
- subarachnoid hemorrhage (consequence of hemorrhagic stroke or traumatic brain injury);
- a special case of Guillain-Barré syndrome is Miller-Fisher syndrome;
- cerebellar tumors;
- alcoholism, which leads to Wernicke encephalopathy;
- substance abuse;
- poisoning with gasoline vapors, mercury, etc.
Chronic cerebellar ataxias are a consequence of degenerative processes and are divided into primary and secondary.
Primary degeneration of cerebellar structures
Occurs due to:
- heredity (group of hereditary cerebellar ataxias);
- atrophic processes in the brain (for example, Parkinson's disease, Alzheimer's disease, etc.);
- idiopathic cerebellar degeneration (that is, reliable causes of this group of ataxias have not been established).
Causes of secondary chronic cerebellar ataxias
Can be:
- multiple sclerosis;
- paraneoplastic syndrome;
- hypothyroidism;
- malabsorption syndrome (impaired absorption of nutrients in the intestines, in particular vitamin E);
- gluten intolerance;
- alcoholism;
- Wilson-Konovalov disease (disorder of copper metabolism in the body);
- cerebellar tumors.
Causes of sensitive ataxia
Sensitive ataxia occurs as a result of a lesion
- peripheral nerves that carry information about deep sensitivity to the brain (diphtheria, diabetic, alcoholic polyneuropathy);
- spinal cord, namely its posterior columns, in which deep sensitivity fibers pass, supplying information from the periphery to the brain (funicular myelosis, tumors, tabes dorsalis, etc.).
Vestibular ataxia
Vestibular ataxia occurs as a result of damage to the vestibular nerve, or vestibular organ, which is located in the inner ear. This type of ataxia appears with Meniere's disease, tumors in the bottom of the fourth ventricle, infections of the inner ear, and injuries accompanied by a fracture of the base of the skull.
Causes of cortical ataxia
Cortical ataxia, or in other words, frontal, occurs as a result of pathological processes in the frontal lobes or the occipitotemporal region of the brain. Damage to these brain structures can occur with encephalitis, abscesses, traumatic brain injuries, strokes of various etiologies, etc.
Causes and types
There are many causes of ataxia, but most can be categorized as sporadic (no specific cause), genetic (also called hereditary or familial), or secondary to medical conditions, certain medications, or brain damage.
Congenital ataxias occur in children and are most often caused by structural abnormalities in the brain that occur before or during birth. These include cerebral palsy (often associated with damage to the brain at birth due to lack of oxygen or blood supply), hydrocephalus (high blood pressure caused by a blockage or buildup of cerebrospinal fluid), brain tumors, and other brain injuries.
Ataxia may occur suddenly (acute), over a period of weeks (subacute), or progress slowly over months or years (chronic). In acute cases, the most common cause is a stroke or hemorrhage in or around the cerebellum. Patients typically present with headache, vomiting, neck stiffness, or loss of consciousness. In children, bacterial or viral infections can cause acute ataxia, which usually resolves with time. They develop a fever, trouble walking, and slurred speech within hours or days, and recover within a few weeks. Subacute onset of ataxia is usually observed in paraneoplastic cerebellar degeneration. This is a condition in which breast cancer, lung cancer, ovarian cancer, or other areas of the body produce antibodies that affect the cerebellum and cause the disorder.
How to distinguish ataxia? Symptoms
Symptoms of cerebellar ataxia
The symptoms of cerebellar ataxia are very similar, and often the differences are minimal, which makes diagnosis very difficult. The main symptoms are:
- imbalance in the Romberg position (the patient stands with his eyes closed and his arms extended in front of him, his feet should be together);
- change in gait (the patient spreads his legs wide when walking, staggers, cannot turn around quickly);
- speech is impaired (dysarthria appears - the speech of such a patient is monotonous, abrupt);
- dysmetria (that is, impaired coordination of movement in the limbs. Dysmetria is detected using the finger-to-nose test - the patient with his eyes closed tries to hit the tip of the nose with his index finger and the knee-heel test - an attempt to move the foot along the other leg from the knee down);
- decreased muscle tone in the limbs;
- intention tremor (appears when approaching a target, absent at rest);
- nystagmus (involuntary pendulum-like movements of the eyeballs);
- trembling of the torso and head (titubation);
- oculomotor disorders;
- violation of synchrony when performing fast alternating movements (test for diadochokinesis - the patient is asked to make movements with his hands, as if he were screwing in a light bulb).
Symptoms characteristic of the disease that caused cerebellar ataxia also appear.
Features of hereditary cerebellar ataxias
A separate group consists of hereditary cerebellar ataxias. Their main feature is inheritance either by an autosomal dominant type (one or both parents are necessarily sick) or by an autosomal recessive type (the parents are carriers of the pathological gene and will be healthy, in this case the children are sick).
The most common variant of autosomal recessive ataxia is Friedreich's ataxia (mainly the pathways from the spinal cord to the cerebellum are affected). It is characterized by:
- onset at the age of 8 - 15 years;
- impaired coordination of movements in the trunk and limbs is combined with the absence of tendon reflexes;
- spasticity develops in the legs;
- kyphoscoliosis;
- Friedreich's foot (high arch of the foot with hyperextension of the toes);
- change in handwriting;
- dysarthria;
- cardiomyopathy (myocardial disease).
The prognosis for this disease is disappointing; after about 10–15 years, the patient loses the ability to care for himself independently.
Features of the Louis-Bar syndrome clinic
Louis-Bar syndrome, or ataxia-telangiectasia, in addition to progressive cerebellar ataxia, has the following symptoms:
- early onset (sometimes before 4 years);
- telangiectasia on the skin and in the eye area (a kind of clearly visible vascular mesh);
- impaired immunity (decreased levels of IgA and IgE);
- mental retardation;
- premature aging of skin and hair.
Ataxia Pierre-Marie
It is one of the variants of autosomal dominant cerebellar ataxia. Its features are:
- onset of the disease at the age of 20 - 30 years;
- impaired motor skills and coordination, accompanied by increased tendon reflexes and the appearance of pathological foot signs;
- dysarthria.
Clinical manifestations of sensitive ataxia
Specific signs of sensitive ataxia are deterioration in the patient’s walking in the absence of vision control (in the dark, with eyes closed). Since this group of patients has impaired joint-muscular sensation, they do not feel their movements. Often such patients complain that they walk as if on a “cotton pillow.” Therefore, they are characterized by a slow gait with their heads down (they try to control each step with the help of vision), and they lower their legs to the floor with force, as if pressing down the foot (“stamping gait”).
Symptoms of vestibular ataxia
The main symptoms of vestibular ataxia are dizziness, accompanied by nausea and sometimes vomiting; nystagmus, hearing loss on one side. Loss of coordination increases with eye and head movements. Patients avoid sharp turns and change body position very carefully. There is no dysmetry in such patients.
Signs of cortical ataxia
Cortical ataxia is manifested by weakness in the leg of the opposite side, that is, if the left frontal lobe is affected, the right leg will suffer. Control of vision while walking does not affect the severity of ataxia. Patients are usually thrown towards the pathological focus. Other symptoms characteristic of damage to the frontal lobe are also identified: personality changes, aggressiveness, impaired sense of smell, etc.
Diagnostic measures
The disease can be easily identified through various types of examination techniques. Neurologists observe muscle hypotonia, which appears in extensor and flexion reflexes, inhibition of deep types of sensitivity. When testing the patient according to Romberg standards, the patient is diagnosed with staggering, which increases its activity when the eyes are closed. When it is necessary to stretch your arms forward, uncontrolled movements appear. Individuals diagnosed with sensory ataxia have complaints about coordination abilities: the inability to place a finger on the tip of the nose or place the heel of the left foot to the knee of the right. An abnormal gait of the “table” type is noted. The neurological picture is presented along with deficits and cerebral manifestations. The main task of doctors when examining a patient’s condition is to correctly distinguish sensitive ataxia from other types. The main factor is an increase in symptoms when the eyelids close. Instrumental tests help identify the source of the disease. For diagnosis, doctors prescribe:
- Donation of blood cells for biochemical analysis.
- Electroencephalogram.
- MRI scan of the brain.
- Computed tomography of the spine.
- Electroneurography.
- Assessment of evoked potentials.
- Electromyography.
In cases where the anomaly may be hereditary, the attending physician will prescribe a consultation with a geneticist and DNA testing and genealogical research.
Diagnosis of ataxia
| Cerebellar ataxias | Sensitive ataxia | Vestibular ataxia | Cortical ataxia |
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Diagnostics
A person suffering from sensitive ataxia describes his sensations when walking as “walking on a soft carpet” or “walking on cotton wool.”
Sensitive ataxia is detected during the initial examination of the patient by a neurologist. To identify the cause of the disease, the following diagnostic methods are used:
- a neurological examination allows you to exclude other types of ataxia (cerebellar, cortical or vestibular) or identify a combination of them;
- blood chemistry;
- Lumbar puncture. A study of cerebrospinal fluid is necessary to identify multiple sclerosis, neurosyphilis or other inflammatory processes in the central nervous system;
- CT and MRI of the brain and spinal cord;
- ENMG (electroneuromyography). Peripheral nerves and muscles are examined;
- consultation with a geneticist to identify hereditary diseases.
Modern aspects of the treatment of ataxias
The basic principle of treatment for all types of ataxia (except for hereditary cerebellar ataxia) is to eliminate the underlying disease that caused impaired motor skills and coordination. Symptomatic (Betagistin, Cinnarizine, Thiotriazoline, anticholinesterase drugs - Proserin; Neuromidin, Keltikan, Nucleo-CMF), restorative (B vitamins - Combilipen, Neurovitan; ATP, Aloe, Tsioflavin, Mexidol) and neuroprotective therapy (Cerebrolysin, Gliatilin, Ceraxon).
In the treatment of cerebellar ataxia, physical therapy has a special place. Thanks to specific sets of exercises, coordination improves and the muscles of patients are strengthened. To reduce the severity of tremors in the hands, patients wear weighted bracelets (about 600 - 800 g). Among the drug correction of tremor, drugs such as Clonazepam, Valpracom, Depakine Chrono, Topiramate should be highlighted. Isoniazid is used extremely rarely in combination with Vit B6.
In the treatment of Friedreich's ataxia, an analogue of coenzyme Q10, Idebenone, has some effect. For Pierre-Marie ataxia, Amantadine (Midantan) and Buspirone have a positive effect. Also often, for cerebellar ataxia, a course of treatment with multivitamins and vitamin E is prescribed.
The prognosis for cerebellar ataxia is unfavorable. Methods that completely eliminate the disease have not yet been developed. Over time, the disease progresses and leads to permanent disability.
Treatment
Treatment of sensitive ataxia is aimed at eliminating the factors that caused the pathology. Appointed:
- B vitamins (for funicular myelosis, vitamin B12 is administered intramuscularly at 500-1000 micrograms daily or every other day, and after 5 days the drug is administered once a week at 100 micrograms for 6 months). For the folate-deficient form of funicular myelosis, folic acid is used at a dose of 5-15 milligrams per day.
- Nootropic drugs (Cerebrolysin, amiridine, etc.) for vascular pathology of the brain.
- Immunoglobulin, which is necessary to maintain immunity.
- Riboflavin, which is used in the presence of neoplasms.
- Anticholinesterase drugs, which promote better conduction of excitation in nerve endings and are used in the presence of peripheral neuritis.
Treatment includes a set of gymnastic exercises, which are aimed at reducing incoordination and strengthening muscles. Daily walking of up to 1 km is also recommended.
Treatment and prognosis of ataxia
Ataxia, which is treated primarily with medication, can be completely eliminated with timely consultation with a doctor. A neurologist treats the disease. He may prescribe the following types of treatment:
- Taking strengthening vitamins. According to medical observations, patients’ bodies most often lack vitamin B. It can be consumed in the form of capsules or solutions;
- Normalization of blood circulation. Drugs are prescribed to stimulate blood flow to the brain;
- Taking hormonal drugs (steroids) to remove plasma from the blood (if it was detected during the analysis);
- Surgical treatment of the cerebellum. Tardive cerebellar ataxia is eliminated mainly by surgery: the hemorrhage is removed, allowing blood flow to the brain.
Spinocerebellar ataxia and other types of diseases are treated in this way.
Treatment tactics
Sensitive ataxia is difficult to treat; basic therapy is limited to symptom correction. There are several areas of treatment, in one of which the leading place is given to the methods of physiotherapy and exercise therapy. The use of medications is limited to maintenance treatment:
- B vitamins – affect the condition of muscle tissue; individually selected dosages can smooth out spasms and other unpleasant signs of the disease. Used mainly in injection form;
- the appointment of riboflavin and immunoglobulins - these substances are necessary to stimulate nerve fibers;
- nootropic drugs are selected to normalize brain activity and transmit neural impulses. Some nootropics are used as mild tranquilizers to correct the psycho-emotional state;
- to correct the immune state, multivitamin complexes are additionally prescribed;
- if neoplasms or hyperthyroidism are present, vitamin B2 must be used.
It is possible to prescribe anticholinesterase drugs if severe muscular dystrophy or neuritis is observed. The complex use of drugs noted in these groups stimulates the normal functioning of muscles and nerve receptors responsible for movement and coordination. However, they cannot eliminate the key causes of ataxia.
Nootropic drugs are used mainly when the functioning of the brain vessels is disrupted and intellectual abilities are reduced.
However, medication is not always a reliable and effective way to combat the disease. In most cases, physical exercise gives the best effect.
