Ataxia is a motor disorder not accompanied by paralysis, the characteristic features of which are disorders of rhythm and coordination of movements. Translated from Greek, the word ataxia means "chaotic" and "disorderly".
With this diagnosis, movements become disproportionate, awkward, inaccurate, walking often suffers, and in some cases even speech. Consider what types of ataxia stand out, and what are their characteristic features.
Friedreich's Ataxia
Friedreich's hereditary ataxia is a genetically determined neurological disease that is progressive. The initial manifestations of the disease become noticeable in the first few decades of life.
First, handwriting and gait disturbances appear. For children, however, it is difficult to identify a violation of handwriting due to the fact that it has not yet been fully formed. As for the gait, the patient needs support, he constantly sways. All leg movements are more jerky than progressive.
Inability to stand develops over time(astasia) and even walking (abasia). However, the latter is a characteristic manifestation of the rapidly progressive course of the disease and its last stages.
Changes in the spine are observed, which is especially important for adolescents in whom the process of its formation has not yet been completed. A few years later, the patient develops diabetes due to malfunctions in the pancreas. A little later, due to dystrophic changes in the gonads, hypogonadism occurs. In the last stages of ataxia, visual impairment is added, which occurs as a result of dystrophic changes in the optic and oculomotor nerves. On top of that, dementia develops due to damage to brain neurons.
Cerebellar lesion
Cerebellar ataxia is a disorder of motor coordination that develops when the main organ responsible for it is damaged - the cerebellum of the brain. In some cases, minor changes develop, while in others, more serious and severe changes.
The development of such ataxia occurs due to the involvement of various areas of the cerebellum by the pathological process. Often, cerebellar ataxia is diagnosed with encephalitis, vascular diseases of the cerebellum, multiple sclerosis, malignant brain tumors, intoxications, as well as certain ailments of genetic origin. There are 2 types of cerebellar ataxia - static and dynamic.
The static nature of cerebellar ataxia
Cerebellar lesion is expressed in static ataxia due to reduced tonemuscles. In the process of this, it becomes difficult for the patient to stay in one position for a long time, and he also has an insignificant violation of motor coordination. The person moves with very wide and staggering steps, as if in a state of intoxication. In the case of a severe course of the disease, the patient is not able to sit and stand on his own, because, not even having the strength to hold his head, he constantly falls. Static ataxia in severe form deprives the patient of the ability to independently maintain balance. It should be noted that motor coordination is not affected by whether the patient is with eyes open or closed.
Dynamic nature of cerebellar ataxia
Dynamic ataxia develops when the cerebellar hemispheres are involved in the pathological process. With this type of disease, coordination disorders are observed exclusively during physical movement. The smoothness and accuracy of movements are lost, they become sweeping and awkward. Discoordination and slowing down of movements are observed on the side of the lesion. Dynamic ataxia is characterized by hypermetry (excessiveness, opposite movements), adiadochokinesis, overshooting, as well as intentional tremor and speech disorders (patients speak slowly, dividing words into syllables).
In the standing position and when walking, the patient deviates to the side corresponding to the damaged hemisphere of the cerebellum. The patient's handwriting changes: it becomes uneven, sweeping, with largeletters. Decreased tendon reflexes are possible.
Sensitive ataxia
This ataxia is a movement disorder in which a change in gait occurs due to loss of sensation in the legs resulting from damage to the peripheral nerves, the medial loop, the posterior columns, or the posterior roots of the spinal cord. The patient does not feel the position of the legs, and therefore has difficulty both walking and standing. As a rule, he stands with his legs wide apart and at the same time he can only maintain balance with his eyes open, but if they are closed, the person will begin to stagger and, most likely, fall (positive symptom of Romberg). When walking, patients also spread their legs wide and raise them much higher than necessary, and also sway impetuously forward and backward. Their steps have different lengths, and the feet, touching the floor, make popping sounds. When walking, the patient usually uses a stick for support and slightly bends the torso at the hip joints. Gait disturbances exacerbate visual defects. Patients often become unsteady, sway and fall when washing, because when they close their eyes, they lose visual control for a while.
Spinocerebellar ataxia
This term refers to a variety of movement disorders, most of which occur as a result of ischemic damage to the central nervous system in the perinatal period or hypoxia. The severity of gait changes can be different and depends on the severity and nature of the lesion. So, light limited lesions can causeBabinsky's symptom, increased tendon reflexes and not accompanied by a pronounced change in gait. Larger and more severe lesions usually result in bilateral hemiparesis. There are changes in gait and postures characteristic of paraparesis.
Cerebral palsy causes movement disorders that lead to a change in gait. In patients with this, involuntary movements in the limbs occur, which are accompanied by grimaces on the face or rotational movements of the neck. As a rule, the legs are extended and the arms are bent, however, this asymmetry of the limbs can become noticeable only with careful observation of the patient. So, for example, one arm can be pronated and extended, while the other is supinated and flexed. The asymmetric position of the limbs often occurs when the head is turned in different directions.
Diagnosis of ataxia
To establish a diagnosis, diagnostic methods such as:
- MRI of the brain;
- electroencephalography of the brain;
- DNA diagnostics;
- electromyography.
In addition to any of the indicated methods, it is necessary to take a blood test, undergo an examination by specialists such as a neurologist, psychiatrist and ophthalmologist.
Axia treatment
Ataxia is a serious disease that requires timely action. The treatment provided by a specialist neurologist is predominantly symptomatic and includes the following areas.
- Fortifying therapy(anticholinesterase agents, Cerebrolysin, ATP, B vitamins).
- Physiotherapy aimed at preventing various kinds of complications (muscle atrophy and contracture, for example), improving walking and coordination, maintaining physical fitness.
Special gymnastic complex of exercise therapy exercises, the purpose of which is to reduce discoordination and strengthen muscles. With a radical method of treatment (surgery of cerebellar tumors, for example), one can expect a partial or complete recovery, or at least a cessation of further progression.
In Friedreich's ataxia, taking into account the pathogenesis of the disease, drugs aimed at maintaining mitochondrial functions ("Riboflavin", vitamin E, coenzyme Q10, succinic acid) can play a huge role.
Prognosis for disease
The prognosis of hereditary diseases is rather unfavorable. Over time, especially with inactivity, neuropsychiatric disorders only progress. People diagnosed with ataxia, whose symptoms become more pronounced with age, tend to have significantly reduced ability to work.
However, thanks to symptomatic treatment, as well as the prevention of intoxication, injury and infectious diseases, patients live to an advanced age.
Prevention
It is impossible to carry out preventive measures specifically for ataxia. First of all, it is necessary to preventthe possible emergence and development of acute infectious diseases (sinusitis, otitis media, pneumonia, for example) that can provoke ataxia.
Blood marriages should be avoided. In addition, it should be remembered that there is a high probability of transmission of hereditary ataxia from parent to child, and therefore patients are often advised to refuse the birth of their own babies and adopt someone else's child.
Ataxia is a serious neurological disorder that must be treated immediately. That is why the earlier this disease was detected, the more favorable the prognosis for the patient will be.
