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Multisystem atrophy


Multisystem atrophy, or Shai's Syndrome — Dreydzhera — a degenerative neurologic disease. It is connected with a degeneration of nervous cells of certain sites of a brain. This cellular degeneration causes problems with the movement, balance and other vegetative functions of a body, such as control of an urination or regulation of blood pressure. The reasons of this syndrome are unknown and no specific risk factors were revealed. About 55% of cases are the share of men from 50 to 60 years.
The general prevalence of a multisystem atrophy is estimated at 4,6 cases on 100 000 people. This disease meets more often at men, than at women, and researches show a ratio ranging from 1,4:1 with increase to 1,9:1 here.

Symptoms of a multisystem atrophy:

The multisystem atrophy is characterized by a combination of the following signs which can be present at any combination:
- vegetative dysfunction
- parkinsonism (a hypertension with a tremor or a tremor and the slowed-down movements)
- ataxy (bad coordination or uncertain walking)

At dominance of vegetative insufficiency the term Shai's syndrome — Dreydzhera is sometimes used though it officially does not exist because of changes in terminology any more.

Also there can be an option with features of a multisystem atrophy and dementia of a body of Levi.

Primary signs.

The most widespread first sign of a multisystem atrophy is emergence "akinetically - a rigid syndrome" (that is slowness of initial movements, reminding Parkinson's disease), comes to light in 62% of primary survey. Other general symptoms of a disease include problems with balance (a cerebellar ataxy), comes to light in 22% on primary survey, and then urinogenital problems (9%). For men erectile dysfunction (inability to reach and support an erection) can be the first sign. Both men, and women with this disease often have problems with an uric puzyryom or inability to an urination (an urine incontience). About 1 of 5 patients with a multisystem atrophy suffer from falling in the first year of display of their disease.

Symptoms of the progressing disease.

At the progressing disease three groups of signs prevail. It is the following:
parkinsonism (the slow, awkward movements, the letter becomes small and pauchyeobrazny)
cerebellum dysfunction (zatrudnyonnost of coordination of movements and balance)
vegetative dysfunction (disturbance of automatic functions of a body), includes:
postural or orthostatic hypotension that leads to dizziness or a faint at a standing position
Incontience of urine or delay of an urination
paralysis of phonatory bands
dryness in a mouth and skin
problems of thermal control of a body because of sweating disturbance
loud snore, asthma or respiratory stridor during sleep
other frustration of a dream, including apnoea and frustration with the bystry movement of eyes.

There can be also other symptoms, such as doubling in eyes.

Some patients (in one research — 20%) as a result of a multisystem atrophy experience considerable cognitive disturbances.

Мультисистемная атрофия (гистология)

Multisystem atrophy (histology)

Reasons of a multisystem atrophy:

Possible communication was found with the gene (Src the homology containing 2 domains) turning 2 proteins located in distal 350-kb subletomerny area of the 19th chromosome (19p13.3).

Treatment of a multisystem atrophy:

Basis of treatment of MSA are dofaminergichesky drugs. Nevertheless, even when using in high doses drugs of a levodopa do not lead to considerable improvement. Lack of effect of treatment can probably be objyasyonit loss of postsiyaaltichesky neurons in a striate body and the widespread pathological process involving other neuromediator systems.

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