Efficacy and safety of therapeutic plasma exchange in stiff person syndrome

The stiff person syndrome (SPS) is an extremely rare neurological disorder with primarily immune-mediated etiology. The cardinal symptoms are progressive, fluctuating axial/proximal limb muscle stiffness and spasms. The diagnosis is based on the clinical picture, electromyography examination and det...

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Autores principales:	Czempik Piotr F., Gawryluk Justyna, Wiórek Agnieszka, Krzystanek Ewa, Krzych Łukasz J.
Formato:	article
Lenguaje:	EN
Publicado:	De Gruyter 2021
Materias:	glutamic acid decarboxylase procedure efficacy procedure safety stiff person syndrome therapeutic plasma exchange Medicine R
Acceso en línea:	https://doaj.org/article/69702ba55cbe4550b520c24ae3ddbd3d
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spelling	oai:doaj.org-article:69702ba55cbe4550b520c24ae3ddbd3d2021-12-05T14:10:54ZEfficacy and safety of therapeutic plasma exchange in stiff person syndrome2391-546310.1515/med-2021-0220https://doaj.org/article/69702ba55cbe4550b520c24ae3ddbd3d2021-03-01T00:00:00Zhttps://doi.org/10.1515/med-2021-0220https://doaj.org/toc/2391-5463The stiff person syndrome (SPS) is an extremely rare neurological disorder with primarily immune-mediated etiology. The cardinal symptoms are progressive, fluctuating axial/proximal limb muscle stiffness and spasms. The diagnosis is based on the clinical picture, electromyography examination and detection of antibodies to glutamic acid decarboxylase (anti-GAD). Adverse effects of medications might preclude its use or increase in dosing, therefore symptomatic and/or immunomodulatory medical therapy might be ineffective in acute exacerbation of the disease. We present a case of a 49-year-old female with exacerbation of SPS, in whom some standard pharmacotherapy could not be introduced (clonazepam, baclofen used in the past) and doses of existing standard medications could not be increased (diazepam, gabapentin, and levetiracetam) due to adverse effects. Moreover, a newly introduced medication (methylprednisolone) also led to a serious adverse effect (severe hyperglycemia). The patient underwent therapeutic plasma exchange (TPE) with good effect and no complications. We review the literature regarding the efficacy and safety profile of TPE in exacerbation of SPS unresponsive to medical therapy. The procedure seems to have a good safety profile as an adjunct therapy for exacerbation of SPS not responding to standard medical therapy in this patient population.Czempik Piotr F.Gawryluk JustynaWiórek AgnieszkaKrzystanek EwaKrzych Łukasz J.De Gruyterarticleglutamic acid decarboxylaseprocedure efficacyprocedure safetystiff person syndrometherapeutic plasma exchangeMedicineRENOpen Medicine, Vol 16, Iss 1, Pp 526-531 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	glutamic acid decarboxylase procedure efficacy procedure safety stiff person syndrome therapeutic plasma exchange Medicine R
spellingShingle	glutamic acid decarboxylase procedure efficacy procedure safety stiff person syndrome therapeutic plasma exchange Medicine R Czempik Piotr F. Gawryluk Justyna Wiórek Agnieszka Krzystanek Ewa Krzych Łukasz J. Efficacy and safety of therapeutic plasma exchange in stiff person syndrome
description	The stiff person syndrome (SPS) is an extremely rare neurological disorder with primarily immune-mediated etiology. The cardinal symptoms are progressive, fluctuating axial/proximal limb muscle stiffness and spasms. The diagnosis is based on the clinical picture, electromyography examination and detection of antibodies to glutamic acid decarboxylase (anti-GAD). Adverse effects of medications might preclude its use or increase in dosing, therefore symptomatic and/or immunomodulatory medical therapy might be ineffective in acute exacerbation of the disease. We present a case of a 49-year-old female with exacerbation of SPS, in whom some standard pharmacotherapy could not be introduced (clonazepam, baclofen used in the past) and doses of existing standard medications could not be increased (diazepam, gabapentin, and levetiracetam) due to adverse effects. Moreover, a newly introduced medication (methylprednisolone) also led to a serious adverse effect (severe hyperglycemia). The patient underwent therapeutic plasma exchange (TPE) with good effect and no complications. We review the literature regarding the efficacy and safety profile of TPE in exacerbation of SPS unresponsive to medical therapy. The procedure seems to have a good safety profile as an adjunct therapy for exacerbation of SPS not responding to standard medical therapy in this patient population.
format	article
author	Czempik Piotr F. Gawryluk Justyna Wiórek Agnieszka Krzystanek Ewa Krzych Łukasz J.
author_facet	Czempik Piotr F. Gawryluk Justyna Wiórek Agnieszka Krzystanek Ewa Krzych Łukasz J.
author_sort	Czempik Piotr F.
title	Efficacy and safety of therapeutic plasma exchange in stiff person syndrome
title_short	Efficacy and safety of therapeutic plasma exchange in stiff person syndrome
title_full	Efficacy and safety of therapeutic plasma exchange in stiff person syndrome
title_fullStr	Efficacy and safety of therapeutic plasma exchange in stiff person syndrome
title_full_unstemmed	Efficacy and safety of therapeutic plasma exchange in stiff person syndrome
title_sort	efficacy and safety of therapeutic plasma exchange in stiff person syndrome
publisher	De Gruyter
publishDate	2021
url	https://doaj.org/article/69702ba55cbe4550b520c24ae3ddbd3d
work_keys_str_mv	AT czempikpiotrf efficacyandsafetyoftherapeuticplasmaexchangeinstiffpersonsyndrome AT gawrylukjustyna efficacyandsafetyoftherapeuticplasmaexchangeinstiffpersonsyndrome AT wiorekagnieszka efficacyandsafetyoftherapeuticplasmaexchangeinstiffpersonsyndrome AT krzystanekewa efficacyandsafetyoftherapeuticplasmaexchangeinstiffpersonsyndrome AT krzychłukaszj efficacyandsafetyoftherapeuticplasmaexchangeinstiffpersonsyndrome
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Efficacy and safety of therapeutic plasma exchange in stiff person syndrome

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