Multiple system atrophy (MSA) is relatively rare degenerative disease of the central nervous system. The prevalence of MSA is about 2-4/100,000. The disease is included to synucleinopathies. The most characteristic neuropathological findings in MSA is argyrophilic glial cytoplasmatic inclusions in oligodendroglia which is composed of α-synuclein and ubiquitin. At present two main forms of the MSA are known: MSA-P and MSA-C. Symptoms of parkinsonism with rigidity and bradykinesia are more typical in MSA-P. Cerebellar signs are more characteristic for MSA-C. Severe autonomic failure, initially or during further clinical course in MSA-P and MSA-C is present in most patients. The main symptoms of autonomic failure are orthostatic hypotension, urinary and less commonly fecal incontinence, impotence. In 1998 consensus on the diagnosis of MSA was established. The authors described a case of 61-year-old women with 3 years symptoms of cerebellar dysfunction followed by Parkinson’s syndrome and autonomic failure. MRI of the brain revealed severe atrophy of the cerebellum and brain stem, and “hot cross bun sign” in pons. Tilt-test confirmed orthostatic hypotension. Treatment with L-dopa and midodrin was moderately effective. The authors described problems of diagnostics, especially role of MRI, and actual problems of epidemiology, pathogenesis and treatment of MSA. 

multiple system atrophy, cerebellar atrophy, magnetic resonance, orthostatic hypotension, autonomic failure