Cargando…
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...
Autores principales: | , , , , , , |
---|---|
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 |
_version_ | 1783535460678631424 |
---|---|
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. |
format | Online Article Text |
id | pubmed-7232827 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT huangjuan effectivenessofcombinedimmunoglobulinandglucocorticoidtreatmentsinapatientwithstifflimbsyndromecasereportandreviewoftheliterature AT menghuanyu effectivenessofcombinedimmunoglobulinandglucocorticoidtreatmentsinapatientwithstifflimbsyndromecasereportandreviewoftheliterature AT duanxian effectivenessofcombinedimmunoglobulinandglucocorticoidtreatmentsinapatientwithstifflimbsyndromecasereportandreviewoftheliterature AT liwenwen effectivenessofcombinedimmunoglobulinandglucocorticoidtreatmentsinapatientwithstifflimbsyndromecasereportandreviewoftheliterature AT xuhongwei effectivenessofcombinedimmunoglobulinandglucocorticoidtreatmentsinapatientwithstifflimbsyndromecasereportandreviewoftheliterature AT zhouyafang effectivenessofcombinedimmunoglobulinandglucocorticoidtreatmentsinapatientwithstifflimbsyndromecasereportandreviewoftheliterature AT zhoulin effectivenessofcombinedimmunoglobulinandglucocorticoidtreatmentsinapatientwithstifflimbsyndromecasereportandreviewoftheliterature |