Manifestations and treatment of multisystem atrophy

Multisystem atrophy is a severe sporadic degenerative pathology of the central nervous system, in which cerebellar structures, basal ganglia, trunk and spinal cord are affected. It can manifest itself in varying degrees in the form of ataxias, vegetative and pyramidal disorders, and parkinsonism. This type of disease is an independent nosology and is included in the concept of "multisystem degeneration", which includes many diseases, differing in the degree of expression of changes and polysymptomicity.

Such a pathology develops more often in men and the greatest number of cases are between 50 and 60 years of age.

How multi-system atrophy of

manifests Three variants of manifestation of this disease are distinguished:

1. Striatongral degeneration. With it, there is a predominance of parkinsonian features.
2. Olivopontocerebellar atrophy with a predominance of cerebellar ataxia symptoms.
3. Shay-Draeger Syndrome. On the first place in the symptomatology there are pronounced disorders of the autonomic nervous system.

In some countries, only the first two types of disease are isolated, without using the isolation of the latter syndrome into a separate clinical pathology variant.

It should be noted that Parkinson's syndrome with slowing down of movement and tremor of extremities comes to the fore with multisystem atrophy. Its prevalence at the initial stage requires careful examination and differentiation with Parkinson's disease. Symptoms of multisystem atrophy are very diverse:

• Oculomotor disorders, manifested in the early stages of the disease.
• Lack of effectiveness in prescribing levodopa.
• Rapid progression of all signs.
• Acrocyanosis.
• Sleep apnea, pronounced snoring or respiratory stridor.
• Dysphagia.
• Dysarthria.
• Diplomacy.
• Violation of thermoregulatory functions against a background of increased sweating.
• The emergence of contractures.
• Violent crying or fun.
• Violation of emotion control.
• Decreased sex drive.

Almost half of the patients have pyramidal changes in the form of amplification of peripheral reflexes and positive Babinsky syndrome. Violation of speech functions is a very characteristic feature with multisystem atrophy, which is noted at the apogee of the clinical picture. Threats to life may be episodes of sleep apnea that occur due to obstructive changes in the lungs, but they can have a central origin.

In the fifth part of the patients with multisystem atrophy, memory and mental performance may be disturbed.

For this disease is not a characteristic symptom of the emergence of severe forms of dementia. Progression of the pathological process leads to the risk of sudden death. The prognosis after the approval of the diagnosis is unfavorable - the average lifetime of such patients is about 7 years. Particularly difficult is the disease, if signs of damage to the autonomic functions of the body predominate.

Disturbance of vegetative influences on processes in an organism are caused by destructive changes in central neurons. The most characteristic pathology in this disease is the occurrence of orthostatic hypotension - a sharp decrease in systemic pressure in the standing position and after abundant food, physical activity. Orthostatic hypotension is subjectively manifested in sensation of weakness, dizziness and appearance of "lightness" in the head, lack of clarity of visible images, pain in the back of the neck, with spreading to the shoulders. Many experts believe that the appearance of these symptoms is associated with retinal and muscular ischemia.

These signs are combined with a rise in pressure in the lying position. One of the reasons for the death of such a patient is the lack of physiological pressure reduction during a night's sleep. There are cases of impaired consciousness when the patient passes from a horizontal position to a vertical position. There may also occur attacks on the type of angina pectoris, with absolutely intact coronary vessels. All signs are amplified with the addition of cardiac pathology, increased environmental temperature, dehydration of the body.

Many patients have symptoms of cerebellar ataxia, with impaired coordination processes, changes in handwriting and difficulty in moving. In 9% of cases there are dysuric phenomena, including urinary incontinence. Men suffer from impaired erectile function and decreased sexual desire. In patients, a characteristic inclination of the head in the forward direction, as well as cooling of the extremities-the "cold hands syndrome" can be observed.

Paralysis of the vocal cords, which occurs with multisystem atrophy, leads to speech impairment. In addition, dryness of the skin and mucous membranes, marked sweating, impaired vision( diplopia) can be noted. Changing the swallowing function can lead to aspiration.

Vegetative dysfunction, orthostatic hypotension, dysuria and impotence occur with such a disease quite quickly - they join the initial symptoms of motor function impairment for two years.

Symptoms of this disease vary throughout the day and intensify most often in the morning. The diagnosis is confirmed by MRI.Orthostatic tests are used for vegetative abnormalities.

How to help with the disease?

Treatment of multisystem atrophy is to improve the quality of life of the patient by eliminating the underlying conditions that can lead to disability or death.

Unfortunately, the severity of destructive changes in neurotransmitter systems does not allow the effective use of dopaminergic drugs in a number of cases. Even at maximum therapeutic doses, a drug such as levodopa helps only in 30% of cases.

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