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Kozyreva A.A.

Pirogov Russian National Research Medical University;
Russian Children’s Clinical Hospital

Bembeeva R.Ts.

Pirogov Russian National Research Medical University;
Russian Children’s Clinical Hospital

Druzhinina E.S.

Pirogov Russian National Research Medical University;
Russian Children’s Clinical Hospital

Zavadenko N.N.

Pirogov Russian National Research Medical University

Kolpakchi L.M.

Russian Children’s Clinical Hospital

Pilia S.V.

Russian Children’s Clinical Hospital

Modern aspects of diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy in children

Authors:

Kozyreva A.A., Bembeeva R.Ts., Druzhinina E.S., Zavadenko N.N., Kolpakchi L.M., Pilia S.V.

More about the authors

Journal: S.S. Korsakov Journal of Neurology and Psychiatry. 2024;124(2): 58‑68

DOI: 10.17116/jnevro202412402158

Views: 1223

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Table of contents

To cite this article:

Kozyreva AA, Bembeeva RTs, Druzhinina ES, Zavadenko NN, Kolpakchi LM, Pilia SV. Modern aspects of diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy in children. S.S. Korsakov Journal of Neurology and Psychiatry. 2024;124(2):58‑68. (In Russ.)
https://doi.org/10.17116/jnevro202412402158

 
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OBJECTIVE

Analysis of demographic, clinical, laboratory, electrophysiological and neuroimaging data and pathogenetic therapy of pediatric patients with chronic inflammatory demyelinating polyneuropathy (CIDP).

MATERIAL AND METHODS

Patients (n=30) were observed in a separate structural unit of the Russian Children’s Clinical Hospital of the Russian National Research Medical University named after. N.I. Pirogova Ministry of Health of the Russian Federation in the period from 2006 to 2023. The examination was carried out in accordance with the recommendations of the Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society on the Management of CIDP (2021). All patients received immunotherapy, including intravenous immunoglobulin (IVIG) (n=1), IVIG and glucocorticosteroids (GCS) (n=17, 56.7%), IVIG+GCS+plasmapheresis (n=12, 40.0%). Alternative therapy included cyclophosphamide (n=1), cyclophosphamide followed by mycophenolate mofetil (n=1), rituximab (n=2, 6.6%), azathioprine (n=3), mycophenolate mofetil (n=2, 6.6%).

RESULTS

In all patients, there was a significant difference between scores on the MRCss and INCAT functional scales before and after treatment. At the moment, 11/30 (36.6%) patients are in clinical remission and are not receiving pathogenetic therapy. The median duration of remission is 48 months (30—84). The longest remission (84 months) was observed in a patient with the onset of CIDP at the age of 1 year 7 months.

CONCLUSION

Early diagnosis of CIDP is important, since the disease is potentially curable; early administration of pathogenetic therapy provides a long-term favorable prognosis.

Keywords:

chronic inflammatory demyelinating polyneuropathy in children
flaccid paralysis
treatment of chronic inflammatory demyelinating polyneuropathy

Authors:

Kozyreva A.A.

Pirogov Russian National Research Medical University;
Russian Children’s Clinical Hospital

Bembeeva R.Ts.

Pirogov Russian National Research Medical University;
Russian Children’s Clinical Hospital

Druzhinina E.S.

Pirogov Russian National Research Medical University;
Russian Children’s Clinical Hospital

Zavadenko N.N.

Pirogov Russian National Research Medical University

Kolpakchi L.M.

Russian Children’s Clinical Hospital

Pilia S.V.

Russian Children’s Clinical Hospital

Received:

30.01.2024

Accepted:

01.02.2024

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