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Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature

Stiff limb syndrome (SLS) is a rare autoimmune-related central nervous system disorder, resulting in stiffness and spasms of limbs since onset with rare involvement of the truncal muscles. However, SLS patients will gain notable effects by appropriate therapy focusing on symptomatic treatment and im...

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Autores principales: Huang, Juan, Meng, Huan-yu, Duan, Xian, Li, Wen-wen, Xu, Hong-Wei, Zhou, Ya-fang, Zhou, Lin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7232827/
https://www.ncbi.nlm.nih.gov/pubmed/32477232
http://dx.doi.org/10.3389/fneur.2020.00284
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author Huang, Juan
Meng, Huan-yu
Duan, Xian
Li, Wen-wen
Xu, Hong-Wei
Zhou, Ya-fang
Zhou, Lin
author_facet Huang, Juan
Meng, Huan-yu
Duan, Xian
Li, Wen-wen
Xu, Hong-Wei
Zhou, Ya-fang
Zhou, Lin
author_sort Huang, Juan
collection PubMed
description Stiff limb syndrome (SLS) is a rare autoimmune-related central nervous system disorder, resulting in stiffness and spasms of limbs since onset with rare involvement of the truncal muscles. However, SLS patients will gain notable effects by appropriate therapy focusing on symptomatic treatment and immunotherapy. We reported on a 55-year-old female who showed typical painful spasms in both lower limbs and abduction of the right eyeball that partially responded to low-dose diazepam and had high-titer anti–glutamic acid decarboxylase (anti-GAD) antibody. Electromyography (EMG) only showed continuous motor unit activity (CMUA) in the anterior tibialis and right triceps. Eventually, our patient was diagnosed with SLS and treated with intravenous immunoglobulin (IVIG) and glucocorticoid combined simultaneously. She obtained notable effects. We also review and summarize the current literature on clinical characteristics, coexisting disease, treatment, and outcome of 40 patients with SLS. We hope that this report will provide a basis for further understanding of SLS and promote the formation of more advanced diagnosis and treatment processes.
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spelling pubmed-72328272020-05-29 Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature Huang, Juan Meng, Huan-yu Duan, Xian Li, Wen-wen Xu, Hong-Wei Zhou, Ya-fang Zhou, Lin Front Neurol Neurology Stiff limb syndrome (SLS) is a rare autoimmune-related central nervous system disorder, resulting in stiffness and spasms of limbs since onset with rare involvement of the truncal muscles. However, SLS patients will gain notable effects by appropriate therapy focusing on symptomatic treatment and immunotherapy. We reported on a 55-year-old female who showed typical painful spasms in both lower limbs and abduction of the right eyeball that partially responded to low-dose diazepam and had high-titer anti–glutamic acid decarboxylase (anti-GAD) antibody. Electromyography (EMG) only showed continuous motor unit activity (CMUA) in the anterior tibialis and right triceps. Eventually, our patient was diagnosed with SLS and treated with intravenous immunoglobulin (IVIG) and glucocorticoid combined simultaneously. She obtained notable effects. We also review and summarize the current literature on clinical characteristics, coexisting disease, treatment, and outcome of 40 patients with SLS. We hope that this report will provide a basis for further understanding of SLS and promote the formation of more advanced diagnosis and treatment processes. Frontiers Media S.A. 2020-05-08 /pmc/articles/PMC7232827/ /pubmed/32477232 http://dx.doi.org/10.3389/fneur.2020.00284 Text en Copyright © 2020 Huang, Meng, Duan, Li, Xu, Zhou and Zhou. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neurology
Huang, Juan
Meng, Huan-yu
Duan, Xian
Li, Wen-wen
Xu, Hong-Wei
Zhou, Ya-fang
Zhou, Lin
Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature
title Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature
title_full Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature
title_fullStr Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature
title_full_unstemmed Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature
title_short Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature
title_sort effectiveness of combined immunoglobulin and glucocorticoid treatments in a patient with stiff limb syndrome: case report and review of the literature
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7232827/
https://www.ncbi.nlm.nih.gov/pubmed/32477232
http://dx.doi.org/10.3389/fneur.2020.00284
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