This article presents a review of international data on primary progressive multiple sclerosis (PPMS) and an analysis of factors influencing timely diagnosis of PPMS in a number of regions of the Russian Federation.
Acute infl ammatory polyneuropathy is an important research problem of modern neurology. Guillain — Barré syndrome is a severe form of acute polyneuropathy, which is based on autoimmune infl ammation of the myelin sheath of roots and peripheral nerves. Guillain — Barré syndrome is an example of one of the most severe diseases of the nervous system, in which timely diagnosis, proper therapy and qualifi ed care facilitate the achievement of the full recovery of lost functions in most patients. Following an extensive review of Russian and foreign literature, this article discusses modern concepts of Guillain — Barré syndrome, in particular questions related to its epidemiology, etiopathogenesis, classifi cation, clinical features, diagnosis, treatment and prognosis.
Aim. In this paper, we aim to analyse our own clinical observation of a patient with CLIPPERS syndrome on the basis of available literature data. Materials and methods. The medical record of a patient treated at the Neurology Unit of the S.V. Ochapovsky Regional Clinical Hospital No. 1 (Kradnodarsy Krai) was investigated, along with available national and foreign literary sources. Results. The patient was diagnosed with autoimmune brainstem encephalitis (CLIPPERS syndrome) on the basis of the diagnostic CLIPPERS syndrome criteria modified and adapted by W. Tobin and co-authors in 2017. Among them are: 1) subacute development of symptoms for the pons and cerebellum lesions along with other signs of CNS damage (onset is characterised by diplopia and ataxia); 2) good response to glucocorticosteroid (GCS) therapy; 3) lack of involvement of the peripheral nervous system; 4) enhanced MRI lesions homogeneously accumulating contrast agents without a mass effect; 5) a significant decrease in the accumulation of contrast material during GCS therapy; 6) absence of myelin loss. Conclusion. Considering that the GCS therapy produces a rapid and significant effect, it is important to focus on early detection and treatment of this disease through the development of more accurate markers and evaluation criteria for its diagnosis.
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