Multiple system atrophy (MSA) is a progressive neurodegenerative pathological process that leads to pyramidal, cerebellar and autonomic disruption. It connects three of the pathological process, once considered separate nosologic units:
In postprocess in 2 times more often involved males as compared to females. The disease first manifests mostly at the age of fifty years. As for lifespan, it does not exceed 10-12 years from the initial manifestations.
Etiological factor hitherto not fully understood. Neural degenerative processes observed in several cerebral areas. The initial manifestations of postprocess depend on the localization of the damaging process and its severity.
Pathognomonic Morphosyntax ISA is the identification of cytoplasmic components that contain α-synuclein in oligodendria-glial cells.
Clinical manifestations
The initial manifestations are characterized by variability, but usually consist of a combination of Parkinson’s disease, tolerant to the drug Levodopa, cerebellar disorders and vegetative manifestations.
Symptomatic Parkinsona pattern consists of muscle rigidity, bradykinesia, postural disorders and a pronounced postural tremor of the extremities. Often you can find the high-pitched trembling disorder of pronunciation (dysarthria).
Differs from Parkinson’s because the tremor of peace and dyskinesia are rare and levodopa therapy only in the initial stages partially relieves the manifestations of the pathological process.
Cerebellar dysfunctionraven presented atactic, dimitrieski, dialogoptions disorder, loss of coordination and the presence of abnormal nystagmus. The characteristic features of the vegetative deficiency are orthostatic decompression, delayed or, on the contrary, enuresis, constipation, and upset with the sexual sphere (erectile dysfunction).
To the described manifestations may in the future connect the disorder of the functioning of the sweat, salivary and lacrimal glands, respiratory disorders (dyspnea), dysphagia, fecal incontinence, etc. Also characteristic of the disease are attacks of apnea and snoring during sleep.
Diagnosis
Appropriate diagnosis is exposed on the basis of clinical manifestations at simultaneous for autonomic disorders and parkinsonism, or cerebellar symptoms.
Surveys, which can help to differentiate this disease from similar, but there is a pathological transformation in the striatum, the bridge and the cerebellum on the results of magnetic resonance imaging allow to set the appropriate diagnosis. On the basis of the described manifestations, combined with signs of widespread autonomic failure and incomplete response to levodopa the appropriate diagnosis is sometimes exposed during the life of the patient.
Therapeutic measures
Specific therapeutic measures has so far not developed, is based on symptomatic treatment.
Mild orthostatic hypotension provides infusion treatment using aqueous salt solutions. Also assigned fludrocortisone oral 0.1-0.4 mg disposable a day.
A definite effect of wearing elastic materials and midodrine stimulation of α-adrenergic receptors of the oral method at 10 mg three times a day by mouth. The negative aspect of this treatment is the increase in peripheral azocoralline and HELL in the supine position. The method of lifting the head of the bed by 100 mm, it is possible to reduce nocturnal polyuria and increased blood pressure in the position on the back as well as the intensity of morning orthostatic decrease in blood pressure.
Allowed oral levodopa (carbidopa) 25-100 mg at bedtime or pergolide 0.1 mg once a day increase the dosage to 0.25 and 1 mg three times a day to reduce muscle stiffness and other manifestations parkinsonia. However, in most cases, the treatment proposed for medicaments is marked as inconclusive.
Urinary incontinence in the background hyperreflexive state of the detrusor allowed to treat with oxybutynin chloride in the dosage 5 mg orally three times a day, or to tolterodine 2 mg twice daily, oral. Patients must be trained to smokedmarijuana bladder.
With the goal of therapy for constipation is assigned a diet with increased fiber and means for softening the stool. In the case of aggressive patients attributed to constipation enema.
Erectile dysfunction requires the use of sildenafil in a dosage of 50 mg orally method allowed the combination of different physical methods.
