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Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome

Severe muscle rigidity and spasms are uncommon causes of Intensive Care Unit (ICU) admissions. Stiff-man syndrome (SMS) is a rare disorder characterized by continuous muscle spasms, axial muscle rigidity, “tin soldier gait,” and continuous motor unit activity on electromyography. There are three cli...

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Autor principal: Maramattom, Boby Varkey
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4548423/
https://www.ncbi.nlm.nih.gov/pubmed/26321813
http://dx.doi.org/10.4103/0972-5229.162474
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author Maramattom, Boby Varkey
author_facet Maramattom, Boby Varkey
author_sort Maramattom, Boby Varkey
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description Severe muscle rigidity and spasms are uncommon causes of Intensive Care Unit (ICU) admissions. Stiff-man syndrome (SMS) is a rare disorder characterized by continuous muscle spasms, axial muscle rigidity, “tin soldier gait,” and continuous motor unit activity on electromyography. There are three clinical variants of SMS; stiff-limb syndrome, classical SMS, and paraneoplastic encephalomyelitis with rigidity and myoclonus. Three types of antibodies have been associated with SMS; however, anti-glutamic acid decarboxylase (GAD) antibodies are the most frequent and are seen in the idiopathic type of SMS. The spasms of SMS can be very disabling and severe enough to cause muscle ruptures and skeletal fractures. We present a case of anti-GAD positive SMS with “status spasticus” causing bilateral psoas myoedema and rhabdomyolysis due to repeated axial muscle jerking in a 64-year-old man and discuss the differential diagnosis of a “jerking patient in the ICU.”
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spelling pubmed-45484232015-08-28 Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome Maramattom, Boby Varkey Indian J Crit Care Med Case Report Severe muscle rigidity and spasms are uncommon causes of Intensive Care Unit (ICU) admissions. Stiff-man syndrome (SMS) is a rare disorder characterized by continuous muscle spasms, axial muscle rigidity, “tin soldier gait,” and continuous motor unit activity on electromyography. There are three clinical variants of SMS; stiff-limb syndrome, classical SMS, and paraneoplastic encephalomyelitis with rigidity and myoclonus. Three types of antibodies have been associated with SMS; however, anti-glutamic acid decarboxylase (GAD) antibodies are the most frequent and are seen in the idiopathic type of SMS. The spasms of SMS can be very disabling and severe enough to cause muscle ruptures and skeletal fractures. We present a case of anti-GAD positive SMS with “status spasticus” causing bilateral psoas myoedema and rhabdomyolysis due to repeated axial muscle jerking in a 64-year-old man and discuss the differential diagnosis of a “jerking patient in the ICU.” Medknow Publications & Media Pvt Ltd 2015-08 /pmc/articles/PMC4548423/ /pubmed/26321813 http://dx.doi.org/10.4103/0972-5229.162474 Text en Copyright: © Indian Journal of Critical Care Medicine http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Maramattom, Boby Varkey
Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome
title Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome
title_full Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome
title_fullStr Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome
title_full_unstemmed Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome
title_short Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome
title_sort status spasticus and psoas muscle edema due to anti-gad antibody associated stiff-man syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4548423/
https://www.ncbi.nlm.nih.gov/pubmed/26321813
http://dx.doi.org/10.4103/0972-5229.162474
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