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...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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De Gruyter
2021-03-01
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| Series: | Open Medicine |
| Subjects: | |
| Online Access: | https://doi.org/10.1515/med-2021-0220 |
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| 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. |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-04-11T10:47:38Z |
| format | Article |
| id | doaj.art-69702ba55cbe4550b520c24ae3ddbd3d |
| institution | Directory Open Access Journal |
| issn | 2391-5463 |
| language | English |
| last_indexed | 2024-04-11T10:47:38Z |
| publishDate | 2021-03-01 |
| publisher | De Gruyter |
| record_format | Article |
| series | Open Medicine |
| spelling | doaj.art-69702ba55cbe4550b520c24ae3ddbd3d2022-12-22T04:29:00ZengDe GruyterOpen Medicine2391-54632021-03-0116152653110.1515/med-2021-0220Efficacy and safety of therapeutic plasma exchange in stiff person syndromeCzempik Piotr F.0Gawryluk Justyna1Wiórek Agnieszka2Krzystanek Ewa3Krzych Łukasz J.4Department of Anaesthesiology and Intensive Care, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Medyków 14, Katowice, 40-752, PolandDepartment of Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Medyków 14, Katowice, 40-752, PolandDepartment of Anaesthesiology and Intensive Care, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Medyków 14, Katowice, 40-752, PolandDepartment of Neurology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Medyków 14, Katowice, 40-752, PolandDepartment of Anaesthesiology and Intensive Care, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Medyków 14, Katowice, 40-752, PolandThe 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.https://doi.org/10.1515/med-2021-0220glutamic acid decarboxylaseprocedure efficacyprocedure safetystiff person syndrometherapeutic plasma exchange |
| spellingShingle | Czempik Piotr F. Gawryluk Justyna Wiórek Agnieszka Krzystanek Ewa Krzych Łukasz J. Efficacy and safety of therapeutic plasma exchange in stiff person syndrome Open Medicine glutamic acid decarboxylase procedure efficacy procedure safety stiff person syndrome therapeutic plasma exchange |
| title | 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_short | Efficacy and safety of therapeutic plasma exchange in stiff person syndrome |
| title_sort | efficacy and safety of therapeutic plasma exchange in stiff person syndrome |
| topic | glutamic acid decarboxylase procedure efficacy procedure safety stiff person syndrome therapeutic plasma exchange |
| url | https://doi.org/10.1515/med-2021-0220 |
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