Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous treatable dysimmune neuropathy. The variety of clinical forms and course of the disease can be challenging for proper diagnosis and early treatment. In a quarter of cases CIDP starts acutely, mimicking GuillainBarr syndrome. The early diagnosis is especially important regarding differences in treatment and prognosis of these conditions. In this article, we present a clinical case of acute onset CIDP with a detailed analysis of the differential diagnosis between acute and chronic immune-mediated neuropathies.

Background. The use of rating scales and questionnaires is essential in an evaluation of disease course, treatment response, the disability level and quality of life in patients with chronic inflammatory demyelinating polyneuropathy. The Medical Research Council (MRC) scale and its modification Medical Research Council sum score (MRCss) are widely used for measurement of motor deficit in patients with neuromuscular disorders. However, its usage is limited by the absence of the validated version for Russian-speaking patients.Aim. To validate MRCss scale in patients with chronic inflammatory demyelinating polyneuropathy with development of a Russian version.Materials and methods. We enrolled 50 patients with chronic inflammatory demyelinating polyneuropathy (25 with typical chronic inflammatory demyelinating polyneuropathy and 25 with Lewis–Sumner syndrome). At the first step we conducted linguocultural ratification according to the standard protocol. At the second step the psychometric parameters were evaluated, such as reliability, validity and sensitivity.Results. The developed Russian version of MRCss scale demonstrated the high level of reliability, validity and sensitivity.Conclusion. As a result, we developed a validated Russian version of MRCss scale, recommended for clinical practice and research.

Background. Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common immune‑mediated polyneuropathy. CIDP is characterized by a long‑term progressive or recurrent course of the disease and the development of neurological deficits and disability, it is important to assess the quality of life of patients. The paper presents the results of the first and second steps of validation of the Chronic acquired polyneuropathy – Patient Reported Index (CAP‑PRI) questionnaire designed to assess quality of life in patients with immune‑mediated polyneuropathy.Aim. To develop the Russian version of the CAP‑PRI questionnaire.Materials and methods. 85 patients with CIDP (according to EAN / PNS 2021 criteria) were enrolled. At the first step we conducted linguocultural validation according to the standard protocol. Two Russian‑speaking professional translators in the field of medicine performed direct translation of the original English‑language questionnaire, and the back translation was performed by native speakers with a medical background. At the second step the psychometric parameters were evaluated.Results. The developed Russian version of the CAP‑PRI questionnaire demonstrated a high level of reliability, validity and sensitivity.Conclusion. The Russian version of the CAP‑PRI questionnaire is recommended for clinical practice and research. In addition, the relationship between improved quality of life and the reduction of neurological deficits, disability and fatigue (as assessed by the IRODS, NIS and FSS scales) has been shown.

Myotonic dystrophy type 1 (DM1) is one of the most common neuromuscular diseases with an autosomal dominant type of inheritance associated with expansion in the DMPK gene. A distinctive feature of the disease is the presence of muscle symptoms and multisystemic. Depending on the age of onset and the number of CTG repeats, there are congenital, infantile, juvenile, classic (adult) form and a form with a late onset. Each form is characterized by its own features of the onset, course of the disease, heterogeneity of clinical manifestations, which makes it difficult to make a timely diagnosis. Increasing the awareness of physicians of all specialties about the nature of the course of various forms will make it possible to diagnose MD1 at an earlier stage, improve the prognosis and quality of life of patients. The article provides a literature review that demonstrates the spectrum of clinical manifestations in various forms of MD1.