Role of plasma exchange in stiff person syndrome

Albahra, Said; Yates, Sean G.; Joseph, David; Simone, Nicole De; Burner, James; Sarode, Ravindra

doi:10.1016/j.transci.2019.03.015

Cited by 31 publications

(30 citation statements)

References 7 publications

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“…A retrospective analysis of 10 patients with anti-GAD65 positive stiff-person syndrome found that plasma exchange may help these patients, both for acute exacerbations and long-term maintenance 43…”

Section: Introductionmentioning

confidence: 99%

Plasma exchange in neurological disease

et al. 2019

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Plasma exchange is a highly efficient technique to remove circulating autoantibodies and other humoral factors rapidly from the vascular compartment. It was the first effective acute treatment for peripheral disorders such as Guillain-Barré syndrome and myasthenia gravis before intravenous immunoglobulin became available. The recent recognition of rapidly progressive severe antibody-mediated central nervous system disorders, such as neuromyelitis optica spectrum disorders and anti-N-methyl-D-aspartate-receptor encephalitis, has renewed interest in using plasma exchange for their acute treatment also. In this review we explain the principles and technical aspects of plasma exchange, review its current indications, and discuss the implications for its provision in the UK.

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Section: Introductionmentioning

confidence: 99%

Plasma exchange in neurological disease

et al. 2019

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“…Suppose one or both treatments fail; in that case, we suggest to start with IVIG over plasmapheresis [21]. Plasmapheresis, in our findings, had mixed results compared with IVIG, which had consistent effects and improved symptoms of SPS [24].…”

Section: Establishing a Treatment Protocolmentioning

confidence: 67%

“…In a clinical trial with 10 patients with SPS, six patients continue the treatment chronically on an outpatient basis. However, three patients have complete remission of their symptoms, while seven patients only partially relieve symptoms [24]. Studies suggest that plasma exchange may be useful as adjuvant therapy, mainly in patients with no IVIG regimen response.…”

Section: Plasma Exchange (Plasmapheresis) Therapymentioning

confidence: 96%

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Stiff-Person Syndrome: A Treatment Update and New Directions

Ortíz¹,

Ghani²,

Cox³

et al. 2020

Cureus

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“…For the treatment of symptoms following SPS, in mild cases, GABA receptor agonists, such as baclofen and benzodiazepines, can improve such symptoms. 5 However, in severe cases, immune therapies with intravenous immunoglobulin, corticosteroids, rituximab, or long-term immunosuppressive agents are required. 5,6 If hypercapnic respiratory failure is prolonged, intubation and tracheostomy may be needed.…”

Section: Introductionmentioning

confidence: 99%

Successful management of dyspnea in a patient with stiff person syndrome with noninvasive positive-pressure ventilation: A case report

Kim¹,

Chang²

2021

NeuroAsia

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Approximately half of patients with stiff person syndrome experienced dyspnea due to the involvement of the respiratory muscles. If these muscles are severely affected, respiratory failure can occur. Here, we describe a patient with stiff person syndrome whose nighttime dyspnea was successfully controlled by noninvasive positive-pressure ventilation (NIPPV). A 38-year-old female patient visited the emergency room due to nighttime dyspnea. Her O2 saturation was 93%, and PaCO2 increased to 53.4 mmHg on arterial blood gas analysis. No specific lesion was found on the chest radiograph. We considered that her dyspnea was caused by respiratory muscle stiffness following SPS. For the management of nighttime hypercapnia, we applied NIPPV with volume-targeted assist-control ventilation mode using an oronasal mask interface during sleep. After such treatment, PaCO2 was normalized, and nighttime dyspnea disappeared. We think that NIPPV would be a useful therapeutic option for managing dyspnea induced by stiff person syndrome.

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Role of plasma exchange in stiff person syndrome

Cited by 31 publications

References 7 publications

Plasma exchange in neurological disease

Plasma exchange in neurological disease

Stiff-Person Syndrome: A Treatment Update and New Directions

Successful management of dyspnea in a patient with stiff person syndrome with noninvasive positive-pressure ventilation: A case report

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