Ataxia - a neuromuscular disorder of motility, which is characterized by loss of coordination, loss of balance and at rest and while walking. Lack of coordination among the various muscles can result from certain parts of the brain lesions or vestibular apparatus, which sometimes caused by a genetic predisposition. Treatment for ataxia and forecast of its development depend on the cause of the disease.
Types of ataxia
In clinical practice, the following types of ataxia:
- Cortical or frontal;
When sensory ataxia broken fiber deep sensitivity that carry information about the features of the surrounding area and the position of the body in it. The reason may be the defeat of the posterior columns of the spinal cord or spinal nerves of the thalamus, and polyneuropathy deficiency and vitamin B12.
On examination revealed sensory ataxia symptoms:
- The dependence of the coordination of the visual field;
- Violation of vibration and joint-muscle sensitivity;
- Loss of balance with eyes closed Romberg;
- Loss or reduction of tendon reflexes;
- Unsteady gait.
A characteristic feature of sensory ataxia - the feeling of walking on a carpet or wool. In order to compensate for movement disorders, patients are constantly looking at his feet, and rises high and strong legs bent at the knee and hip joints, and then lowered to the ground by force the whole sole.
In vestibular ataxia, vestibular dysfunction leads to specific gait disturbance, vertigo, nausea and vomiting. All symptoms are worse during sharp turns of the head, and changes in body position. Possible hearing loss and horizontal nystagmus - involuntary movements of the eyeballs. This type of disease can cause stem encephalitis, ear infections, tumors of the brain ventricles and Meniere's syndrome.
Cortical ataxia is caused by disorders of the frontal lobe of the brain as a result of dysfunction of fronto-cerebellopontine system. The reason may be in the wrong stroke, tumors or abscesses.
The frontal ataxia appears on the side of the body opposite to the affected hemisphere. An unstable, slanted or zavalivanija start cornering, and for severe injuries, patients are not able to stand and walk. This coordination disorder characterized as a violation of smell, changes in mentality and expressed grasping reflex.
The features of cerebellar ataxia is the loss of fluency, different types of tremors, muscle hypotonia and oculomotor dysfunction. Gait also has the characteristic signs: patients straddle and swing from side to side. The Romberg observed extreme instability often occurs fall back. Strong incoordination occurs when the tandem walking, when the heel of one foot put to another toe. Cerebellar ataxia may be caused by a wide range of diseases - from vitamin deficiency and toxicity of drugs to cancer.
Hereditary degenerative changes in the cerebellum cause spinocerebellar ataxia - chronic progressive nature that are dominant or recessive type.
Autosomal dominant cerebellar form of the disease is often accompanied by the following symptoms:
- Pelvic disorders.
Autopsy sign ataxia Pierre Marie is hypoplasia of the cerebellum, at least - atrophy inferior olive and the pons. First gait disturbance starts, on average, 35 years. Subsequently added facial expressions and speech disorders. Mental disorders are manifested in the form of depression, lower intelligence.
Autosomal recessive spinocerebellar ataxia associated with such symptoms:
- Hypertonicity of muscles;
Family Friedreich's ataxia occurs because of a spinal injury systems often - as a result of inbreeding. The main anatomopathological symptom - increasing degeneration of posterior and lateral columns of the spinal cord. Around 15 years appear unsteadiness when walking and frequent falls. Over time, changes in the skeleton of a tendency to cause frequent dislocation of joints, and kyphoscoliosis. He suffers from heart - atrial deformed teeth, broken heart rhythm. After any physical stress starts paroxysmal dyspnea and pain in my heart.
Diagnosis and treatment of ataxia
When cerebellar ataxia conducted the following studies:
- EEG. It identifies the reduction of the alpha rhythm and diffuse delta and theta activity;
- MR. Navigate to detect atrophy of the brain stem and spinal cord;
- Electromyography. It shows axonal-demyelinating lesions of the peripheral nerves fibers;
- Laboratory tests. Allows you to monitor the metabolism of amino acids;
- DNA-test. Sets genetic predisposition to ataxia.
Treatment is aimed at eliminating ataxia symptoms. It is carried out by a neurologist and includes:
- Restorative therapy - anticholinesterase agents cerebrolysin, ATP, vitamin B;
- The complex exercise therapy - strengthening muscles and reducing incoordination.
When treating spinocerebellar ataxia may be required to correct the course of immunoglobulin immunodeficiency thus contraindicated any irradiation. Sometimes administered succinic acid, riboflavin, vitamin E and other preparations for maintenance of mitochondrial function.
Forecast hereditary ataxias unfavorable. Employability is usually reduced, and mental disorders are progressing.