Ataxy

The ataxy is a neuromuscular disorder of motility which is characterized by a lack of coordination of movements, and also loss of balance both at rest, and when walking. Inconsistency of actions of various muscles can result from defeats of certain sites of a brain or vestibular mechanism that is sometimes caused by genetic predisposition. Treatment of an ataxy and the forecast of its development depend on a disease origin.

Types of an ataxy

In clinical practice distinguish the following types of an ataxy:

• Sensitive;
• Vestibular;
• Cortical or frontal;
• Cerebellar.

At a sensitive ataxy fibers of deep sensitivity which bear information on features of surrounding space and position of a body in it are broken. Defeat of back columns of a spinal cord, thalamus or spinal nerves, and also polyneuropathy and deficit of B12 vitamin can be the cause.

At survey such symptoms of a sensitive ataxy come to light:

• Dependence of coordination on visual control;
• Disturbance vibration and joint myesthesia;
• Balance loss blindly in Romberg's pose;
• Loss or decrease in tendon jerks;
• Unstable gait.

Characteristic sign of a sensitive ataxy – feeling of walking on a carpet or cotton wool. To compensate motive frustration, patients constantly look to themselves under legs, and also highly lift and strongly bend legs in knee and hip joints, and then with a force lower on a floor all sole.

At a vestibular ataxy dysfunction of a vestibular mechanism leads to specific disturbances of gait, rotatory vertigoes, nausea and vomiting. All symptoms amplify at sharp turns of the head and changes of position of a body. Hearing disorder and a horizontal nystagmus – the involuntary movements of eyeglobes are possible. Can lead trunk encephalitis, diseases of an ear, a tumor of cerebral cavities and Menyer's syndrome to this type of a disease.

The cortical ataxy is caused by disturbances of work of a frontal lobe of a brain as a result of dysfunction frontal мостомозжечковой systems. The reason can consist in the wrong cerebral circulation, tumors or abscesses.

The frontal ataxy is shown on the side of a body opposite to the struck hemisphere. Instability, inclinations or a zavalivaniye begin on turns, and at heavy damages patients are not capable to stand and go at all. Also disturbances of sense of smell, change of mentality and the expressed grasp reflex are inherent to this coordination frustration.

Features of a cerebellar ataxy are loss of smoothness of the speech, tremors of different types, hypotonia of muscles and oculomotor dysfunction. Gait also possesses characteristic signs: patients widely place legs and are shaken here and there. In Romberg's pose extreme instability is observed, often there is a falling back. The severe incoordination of movements occurs at tandem walking when the heel of one leg is put to a sock another. The cerebellar ataxy can be caused by a wide range of diseases – from avitaminosis and intoxication medicines to a malignant tumor.

Hereditary degenerative changes of a cerebellum cause a spinotserebellyarny ataxy – chronic diseases of the progressing character which happen dominant or recessive type.

The autosomal and dominant cerebellar form of a disease quite often is followed by the following symptoms:

• Tremor;
• Hyperreflexia;
• Amyotrophy;
• Ophthalmoplegia;
• Pelvic frustration.

Pathoanatomical sign of an ataxy of Pierre Mari is the cerebellum hypoplasia, is more rare – an atrophy of the lower olives and a varoliyeva of the bridge. The first disturbances of gait begin, on average, in 35 years. Afterwards disturbances of a mimicry and the speech are added. Mental disturbances are shown in the form of depressions, decrease in intelligence.

The autosomal and recessive spinotserebellyarny ataxy is combined with such symptoms:

• Areflexia;
• Dysarthtia;
• Hyper tone of muscles;
• Scoliosis;
• Cardiomyopathy;
• Diabetes mellitus.

The family ataxy of Fridreykh arises because of defeat of spinal systems, most often – as a result of related marriage. The main pathoanatomical symptom – the accruing degeneration of back and side columns of a spinal cord. Approximately in 15 years appear unsteadiness when walking and frequent falling. Over time changes of a skeleton cause tendency to frequent dislocations of joints and a kyphoscoliosis. Heart suffers – atrial teeth are deformed, the cordial rhythm is broken. After any physical tension short wind and a pristupoobrazny heartache begins.

Diagnosing and treatment of an ataxy

At a cerebellar ataxy conduct the next researches:

• EEG. Reveals a reduction of an alpha rhythm and diffusion delta and a theta activity;
• MRT. Carry out for detection of an atrophy of trunks of a head and spinal cord;
• Electromyography. Shows axonal and demyelinating damage of fibers of peripheral nerves;
• Laboratory analyses. Allow to observe disturbance of exchange of amino acids;
• DNA test. Establishes genetic predisposition to an ataxy.

Treatment of an ataxy is directed to elimination of symptoms. It is carried out by the neurologist and includes:

• Fortifying therapy – antikholinesterazny means, Cerebrolysinum, ATP, group B vitamins;
• The LFK complex – strengthening of muscles and reduction of a diskoordination.

At treatment of a spinotserebellyarny ataxy the rate of immunoglobulin for correction of an immunodeficiency can be required, at the same time any radiation is contraindicated. Sometimes appoint succinic acid, Riboflavinum, vitamin E and other drugs for maintenance of function of mitochondrions.

The forecast of a hereditary ataxy is adverse. Working capacity, as a rule, decreases, and mental disturbances progress.