According to screening epidemiological trials, prevalence of celiac disease is up to 1% (1:100) in the countries of Europe and North America. In Russia this value may be near 1:100—1:250. Oligosymptomatic and latent forms of the disease are currently predominant. Incidence of neurological or mental symptoms is up to 22%. Neurological symptoms in celiac disease are characterized by gradual onset (usually 50—53 years old) and slow progressive course. Gluten ataxia as one of the most frequent neurological symptoms of celiac disease accounts 20% among all other forms of ataxia and 45% — among idiopathic forms. Vegetative disorders including postural dizziness, syncope, orthostatic hypotension, urinary incontinence, impotence in men are also frequent manifestations of celiac disease. Thus, neurological symptoms of celiac disease may be similar to those in multiple system atrophy (MSA). Neurodegenerative diseases are a large group of human diseases which do not have etiotropic therapy as a rule and characterized by progressive course followed by rapid disability and death of the patient. It is presented case report of 53-year-old patient with suspected diagnosis of «multiple system atrophy». Prevalence of MSA is 3.4—4.9 cases per 100 000 with an increase up to 7.8 per 100 000 among people over 40 years old. There are certain case reports of this disease in available literature and absent specific markers followed by difficult diagnosis are emphasized. Instrumental diagnostic methods are uninformative to confirm MSA although they are able to exclude other pathological processes. Hypointensive metabolism within putamen, brainstem and cerebellum identified by positron emission tomography (PET) may be a hallmark of MSA. Differential diagnosis with various neurodegenerative diseases and potentially curable diseases including celiac disease is discussed in the article.