Validation of the Medical Research Council sum score (MRCss) for use in Russian-speaking patients with chronic inflammatory demyelinating polyneuropathy

Супонева, Н. А.; Ризванова, А. С.; Melnik, E. A.; Зимин, А. А.; Zaytsev, Alexandr; Yakubu, Amin; Sherbakova, E. S.; Yusupova, D. G.; Гришина, Д. А.; Gnedovskaya, E. V.; Piradov, М. А.

doi:10.17650/2222-8721-2023-13-1-68-74

Neuromuscular Diseases

2023

DOI: 10.17650/2222-8721-2023-13-1-68-74

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Validation of the Medical Research Council sum score (MRCss) for use in Russian-speaking patients with chronic inflammatory demyelinating polyneuropathy

Н. А. Супонева

А. С. Ризванова

E. A. Melnik

et al.

Abstract: 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-spe… Show more

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2024

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Cited by 4 publications

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Multifocal motor neuropathy: long-term clinical and electrophysiological features of the disease

Grishina,

Suponeva,

Tumilovich

et al. 2024

RJTAO

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Little attention has been paid abroad to the problem of the long-term course of multifocal motor neuropathy (MMN). In our country, catamnestic studies of MMN have not been conducted at all. However, the results of such an analysis are extremely important for understanding the course and prognosis of the disease.Objective: to analyse the clinical and neurophysiological data of patients with MMN with a disease duration of more than 5 years.Material and methods. The study included 28 patients with MMN: 9 women (32%) and 19 men (68%); the median age at admission was 50 [44; 56] years; the median disease duration was 10 [8; 13] years. Medical documentation, medical history, complaints, neurological examination results (scored on the MRC and INCAT scales) and results of electroneuromyography (ENMG) of the long nerves of the hands were analysed.Results. The median time between onset of the disease and diagnosis was 5.5 [2; 10] years. Paresis <3 points on the MRC scale was found in the extensor muscles of the hand and fingers (12/28; 43%), in the median (15/28; 53%) and ulnar (20/28; 71%) muscle groups of the hands, in the extensors (11/28; 39%) and flexors (9/28; 32%) of the feet. The median total score for the degree of disability on the INCAT scale was 3 [2; 3] for the hands and 1 [0; 2] for the legs. The comparative analysis of the severity of the neurological deficits on the MRC and INCAT scales at the onset of the disease and in the long-term catamnesis revealed no significant differences (p>0.05). An objective assessment of sensory disorders revealed no changes when testing tactile, pain and temperature sensitivity, while half of the cases (14/28; 50%) showed a disturbance of vibration sensitivity in the lower extremities. The ENMG examination was consistent with the electrophysiological criteria of the disease, one third of the patients showed significant secondary damage to the axons of the motor fibers of the hand nerves, and in half of the cases a slight impairment of the axons of the sensory fibers was registered.Conclusion. MMN is a curable disease. Unfortunately, our retrospective analysis showed that in the Russian Federation there are problems with its diagnosis and quality care of this category of patients. Late diagnosis, delayed start of treatment and non-compliance with the schedule of pathogenetic therapy lead to persistent disability of patients.

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Multifocal motor neuropathy: long-term clinical and electrophysiological features of the disease

Grishina,

Suponeva,

Tumilovich

et al. 2024

RJTAO

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Diﬀerential diagnosis of chronic acquired demyelinating polyneuropathies

Grishina,

Suponeva,

Piradov

2024

РНЖ

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Introduction. The rarity of chronic acquired polyneuropathies (PNP) with the demyelinating nature of peripheral nerve damage causes the diﬃculties of their diﬀerential diagnosis that persist in our country and abroad. Objective: to identify signiﬁcant clinical, neurophysiological and sonographic diﬀerential diagnostic markers in chronic inﬂammatory demyelinating polyradiculoneuropathy (CIDP) and non-IgM paraproteinemic demyelinating polyneuropathies (PDP).Material and methods: 80 patients were included in the study: 30 with CIDP, 30 with non-IgM-PDP associated with monoclonal gammapathy of unclear signiﬁcance (PDP-MGUS), and 20 with non-IgM-PDP associated with lymphoproliferative disease (PDP-LPD). The patients included in the study underwent clinical evaluation of neurological disorders according to the MRC, NIS, VAS, INCAT, IRODS, SARA scales; ENMG and ultrasound studies of peripheral nerves.Results. The predominance of men in all groups was noted (p > 0.05). Compared with patients with CIDP, patients with PDP were signiﬁcantly older, they were more likely to have neuropathic pain syndrome and trophic disorders (p < 0.05). In patients with PDP-LPD, in contrast to CIDP and PDP-MGUS, there was a predominance of the distal pattern of muscle weakness distribution and a greater severity of sensitive ataxia (p < 0.05). During NCV studies in patients with CIDP, compared with patients with PDP, blocks of conduction and dispersion of M-waves were signiﬁ -cantly more often recorded in the study of motor ﬁbers of the nerves of the hands (p < 0.05); and in the study of motor nerves of the legs, non-excitability of motor ﬁbers was signiﬁcantly less often noted (p < 0.05). Ultrasound examination of peripheral nerves showed no signiﬁcant diﬀerences between patients (p > 0.05).Conclusion. Clinical phenotype, neurophysiological and sonographic changes in patients with CIDP and PDP do not have highly speciﬁc diﬀerences. Electrophoresis of serum proteins with immunoﬁxation makes it possible to differentiate CIDP and PDP, and further examination by an oncohematologist with paraproteinemia makes it possible to distinguish MGUS from LPD.

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The Long-Term Course of Chronic Inflammatory Demyelinating Polyneuropathy: a Retrospective Study

Melnik,

Arestova,

Berdalina

et al. 2024

Annals of Clinical and Experimental Neurology

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Introduction. Chronic inflammatory demyelinating polyneuropathy (CIDP) is characterized by long-term progressive or relapsing course, neurological deficit, and disability of varied severity. The course of CIDP after specific therapy and, if necessary, long-term maintenance treatment are to be studied. Objective: To evaluate CIDP clinical and history characteristics over the long-term follow-up ( 5 years), to compare long-term CIDP course in a number of clinical variants and onset types, and to determine clinical predictors of unfavorable CIDP course. Materials and methods. The study included 45 patients diagnosed with CIDP based on EAN/PNS 2021 criteria lasting for 5 or more years. Retrospective collection and analysis of medical records and clinical history were performed. Internationally accepted scales were used to assess neurological deficit (NIS, MRCss), disability (INCAT), and disease activity status (CDAS). The criteria of unfavorable course were developed to evaluate factors affecting CIDP course. Results. Among the patients with CIDP history of 5 years, each third (34%) had no neurological deficit and remained in long-term clinical remission (CDAS 1). The vast majority (90%) responded to first-line therapy in early disease, while only 53% of patients required maintenance treatment in 5 or more years of the onset. With the developed criteria (poor response to glucocorticosteroids (GCS), need for maintenance therapy, and CDAS 3–5), unfavourable CIDP course was detected in 24 (53.3%) participants. Its probability increased in later onset (47 [30; 50] years), the chronic type of onset, and delayed specific therapy. The most significant predictors included low total NIS score at onset (60 points) and multifocal CIDP. Conclusions. The course of typical CIDP is relatively favorable if timely diagnosed, and pathogenetic treatment initiated. Patients with acute and subacute onset demonstrate the best long-term status. The predictors of unfavourable disease course include mild neurological deficit at onset (NIS total score 60 points) and multifocal CIDP.

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Validation of the Medical Research Council sum score (MRCss) for use in Russian-speaking patients with chronic inflammatory demyelinating polyneuropathy

Cited by 4 publications

References 6 publications

Multifocal motor neuropathy: long-term clinical and electrophysiological features of the disease

Multifocal motor neuropathy: long-term clinical and electrophysiological features of the disease

Diﬀerential diagnosis of chronic acquired demyelinating polyneuropathies

The Long-Term Course of Chronic Inflammatory Demyelinating Polyneuropathy: a Retrospective Study

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