Sensitive ataxia
In most cases, this pathology occurs due to damage to the posterior columns, posterior nerves, peripheral nodes, thalamus opticus, and parietal cortex. Signs of sensitive ataxia can be observed in all limbs at once, or in only one of them. Doctors often diagnose signs of ataxia, which occurs due to a disorder of the joint-muscular sensation in the legs.
Characteristic signs of ataxia will be the patient's instability, excessive bending of his legs at the knee and hip joints. Many patients often feel as if they are walking on cotton wool or a carpet. Patients try to compensate for walking impairments 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.
Types of ataxia
- Sensitive. The cause of the disease is usually a tumor, vascular pathologies, lesions of the posterior nerves, etc. The patient may bend his legs excessively when walking, step on his feet too hard (as if knocking them on the floor), or fully not feel the surface on which he walks, for example, he has it may feel like he is stepping on a soft carpet rather than a hard floor. Therefore, such patients pay special attention to visual control of their movements and constantly look at their feet to calculate the strength of their step.
- Cerebellar, including hereditary cerebellar. The cause is damage to the cerebellum caused by multiple sclerosis, genetic predisposition, encephalitis, tumor or other diseases. If the lesion is caused by a hereditary factor, the disease usually makes itself felt after 35 years.
- Vestibular. The reason is damage to the vestibular apparatus, which can occur if the patient has a brain tumor, Meniere's syndrome, encephalitis and other diseases. This type of ataxia is characterized by dizziness and nausea, which intensify if the patient moves his head. That is why the patient moves slowly and carefully, and tries to keep his head still.
- Cortical. The disease is caused by the presence of abscesses, malignant neoplasms, and cerebrovascular accidents in the patient, which ultimately lead to various lesions of the frontal region. Patients most often encounter dysfunctions of the lower extremities, for example, when turning, they can lean to the side, fall, and sometimes completely lose the ability to walk or stand.
Cerebellar ataxia
It occurs due to serious damage to 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, or a malignant neoplasm in the cerebellum or brain stem. Patients with this pathology usually fall toward the damaged cerebellar hemisphere when walking. During such episodes, even falls are possible.
The patient usually staggers greatly when walking, and also places his legs too wide, his movements are slow, sweeping and awkward. Impaired coordination remains virtually unchanged even with vision control. Patients may experience serious speech disturbances, which gradually slow down, become scanned and drawn out. There are also problems with handwriting, which becomes uneven and splayed.
Classification of coordination disorders
Scientists have found that uncoordinated movements of various parts of the body are caused by lesions in various parts of the brain. When the cerebellum is damaged, ataxia is observed. Moreover, its manifestations depend on which area of this organ is functioning incorrectly. If the lesion affects the cerebellar hemispheres, then we are talking about dynamic ataxia. And the opposite case. In cases where the functioning of the cerebellar vermis is disrupted, they speak of static ataxia. Everything is extremely simple.
Static ataxia, the characteristics of which are based on disturbances in stability and stabilization of the center of gravity, is one of the common causes of uncoordinated movements. Moreover, the work of the worm can be understood by everyone, if you remember the sensations of the body during sudden braking while driving a car. It is in the ability to return the body position to a stable state that the work of the worm lies.
Vestibular ataxia
This pathology occurs due to damage to the vestibular nerve, labyrinth, cortical center and nuclei in the brain stem. This type of ataxia is observed in various ear diseases, brainstem encephalitis, Meniere's syndrome, and tumors of the fourth ventricle of the brain. The main sign of the pathology is considered to be regular severe dizziness, as a result of which the patient begins to feel as if 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, and abscesses. In the case of this type of ataxia, symptoms such as unsteadiness when walking, falling over, or bending to the side appear.
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: mental changes, grasping reflex, impaired sense 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 hypoplasia of the cerebellum, atrophy of the pons and inferior olives. Typically, the first signs of pathology appear in a patient at the age of 35 years.
The most characteristic symptoms of the disease are considered to be disturbances in gait, speech and facial expressions. In addition, patients usually experience dysmetria, static ataxia, adiadochokinesis, increased tendon reflexes, and decreased strength in the muscles of the limbs. Patients are often diagnosed with oculomotor disorders:
- lack of convergence;
- ptosis;
- paresis of the abducens nerve;
- Argyll-Robertson sign;
- decreased visual acuity;
- narrowing of visual fields;
- atrophy of the optic nerves.
Familial Friedreich's ataxia
This disease is hereditary and occurs due to damage to the spinal systems. As a result of research, it was found that many patients with this type of ataxia often have consanguineous marriages in their 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, slowed speech, and hearing loss. Further development of ataxia leads to changes in the skeleton, heart rhythm disturbances, endocrine disorders, frequent dislocations, and kyphoscoliosis.
Diagnosis of ataxia
- Diagnosis of the disease begins with the collection of complaints and anamnesis. Namely, the doctor must ask the patient how long ago he began to have complaints about unsteadiness of gait and poor coordination of movements, and how often and regularly these symptoms appear. It is definitely worth checking with the patient whether his relatives have had this disease. It is also necessary to find out whether the patient has taken any medications such as benzodiazepines and barbiturates.
- Patients suspected of having ataxia require a neurological examination. This examination involves assessing coordination of movements and gait, assessing strength in the limbs and muscle tone, the presence of nystagmus, and strength in the limbs.
- A correct assessment of hearing will also require an examination by an otolaryngologist.
3. Symptoms, diagnosis
The probable symptoms of spinocerebellar neuronal degeneration are so polymorphic that it is impossible to describe at least the main of its twenty types in one article. There are disorders of visual-motor coordination and muscle tone (tremor, extrapyramidal “stiffness”); partial paralysis of the extraocular muscles and degenerative retinopathy in combination with optic nerve atrophy; general atrophy of muscle fibers; various neurological symptom complexes. If the neurodegenerative process affects the cerebral cortex, dementia gradually develops - a debilitating decline in cognitive functions (memory, attention, various types of recognition, etc.), logical thinking, and speech organization. In case of damage to the medulla oblongata, “bulbar” symptoms develop: degradation of swallowing, palatal, chewing, and respiratory reflexes.
Diseases in this group progress relatively slowly: the course can take up to 20 years or more, although much faster developments have also been described. Patients gradually lose the ability for self-care and, in general, for productive contact with the world; they increasingly depend on the care and support of others, falling into complete helplessness towards the terminal stage and dying, as a rule, from pneumonia, exhaustion, respiratory failure, etc.
Neurodegenerative diseases, including ataxia, are diagnosed clinically, during a neurological examination and a thorough analysis of complaints and anamnestic information. Additionally, tomographic imaging methods, neuropsychological examination, etc. may be prescribed, but the final diagnosis is established and differentiated, as a rule, only pathomorphologically.
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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 in various diseases.
- No less effective techniques are also CT and MRI of the brain. They allow you to study the structure of the brain layer by layer, detect violations of the structure of its tissues, identify ulcers, tumors, and hemorrhages.
- Magnetic resonance angiography will help detect tumors in the brain and assess the integrity of the arteries in the skull.
Complications of ataxia
Treatment of ataxia must be timely, as otherwise serious complications are possible. One of the most dangerous complications of ataxia is heart failure. This is an acute or chronic condition, which is accompanied by shortness of breath, swelling, and frequent fatigue. The danger of the pathology is that it can cause pulmonary edema. Today, heart failure is one of the common causes of death. No less dangerous complications of ataxia are repeated infectious diseases and respiratory failure.
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Treatment of ataxia
Treatment of ataxia is aimed at eliminating the etiological factor that triggered 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 procedure to relieve pressure in the posterior fossa may be necessary. Namely, this procedure is carried out if a patient has an Arnold-Chiari malformation, which is characterized by a slight descent of part of the cerebellum into the opening of the skull, which ultimately leads to severe compression of the brain stem. Ataxia can also be caused by 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, which normalizes metabolism in the brain.
If the cause of this serious disease is high blood pressure, it is imperative to normalize it with the help of drug therapy. In case of circulatory problems in the brain, drugs that improve metabolism and blood flow are also indicated. These primarily include nootropics and angioprotectors.
Ear diseases can also lead to the development of ataxia. In case of infectious brain damage caused by ear pathology, a course of antibiotic therapy is prescribed. If there is a lack of vitamin B12, it must be compensated. To eliminate poisoning, vitamins A, B, and C are prescribed. For demyelinating diseases, hormonal medications are indicated, as well as plasmapheresis. This procedure involves removing blood plasma, but preserving blood cells.
Ataxia is a dangerous disease because, due to lack of coordination, the patient can experience serious injuries. This can be prevented by following a few rules:
- It is better for all patients, regardless of the stage of their disease, to avoid driving a car and working with power tools. In these situations, poor coordination can cause serious injury.
- Patients with ataxia should always keep the room well-lit to increase depth of perception and avoid injury. Twilight is especially dangerous for patients.
- Many patients experience serious difficulty climbing stairs. To avoid injury, be sure to hold on to the railing or use outside help when climbing stairs.
- If it is very difficult for a patient to move independently due to lack of coordination, he should use a walker or cane while moving.
- If you experience a feeling of nausea or severe dizziness, you should immediately sit down or lie down.
- Doctors also advise listening to your body. Therefore, if the disease worsens, you should make an appointment with a neurologist.
Treatment of early cerebellar ataxia
Treatment is prescribed only after confirmation of the diagnosis by a medical specialist. Symptomatic treatment, dosed exercise therapy, vitamins, neuroprotectors, and metabolites are indicated.
Essential drugs
There are contraindications. Specialist consultation is required.
- Idebenone (nootropic, antioxidant agent). Dosage regimen: orally, at a dose of 30 mg 2-3 times a day. Course duration: 1.5–2 months. Conduct 2–3 courses per year.
- Coenzyme Q10 (antioxidant, stimulating cellular metabolism). Dosage regimen: orally, at a dose of 75-400 mg/day. for 2 doses. Duration of treatment - 1 month. If necessary, the course is repeated.
- Retabolil (anabolic agent). Dosage regimen: IM, 25–50 mg once every 3–4 weeks.