Efficacy and safety of therapeutic plasma exchange in stiff person syndrome

Czempik, Piotr F.; Gawryluk, Justyna; Wiórek, Agnieszka; Krzystanek, Ewa; Krzych, Łukasz J.

doi:10.1515/med-2021-0220

Cited by 10 publications

(4 citation statements)

References 32 publications

(7 reference statements)

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“…However, there is a lack of consensus on the immunotherapy regimen. A study showed that IVIG had a better therapeutic effect than IVMP for patients with SPS and ACA ( 24 ). Another study found that corticosteroids were the best regimen for ACA ( 25 ).…”

Section: Discussionmentioning

confidence: 99%

Neurological disorders associated with glutamic acid decarboxylase 65 antibodies: Clinical spectrum and prognosis of a cohort from China

et al. 2022

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ObjectiveTo describe clinical phenotypes and prognosis of neurological autoimmunity related to glutamic acid decarboxylase 65 (GAD65) antibodies in China.MethodIn this retrospective observational study from Peking Union Medical College Hospital, we identified patients with neurological disorders related to GAD65 antibodies (cell-based assay) from May 2015 to September 2021. Clinical manifestations, immunotherapy responsiveness, and outcomes were collected after obtaining informed consent from all patients.ResultsFifty-five patients were included: 40 (72.73%) were women and initial neurological symptoms developed at 42(34-55) years of age. The median time to the nadir of the disease was 5 months (range from 1 day to 48 months). The clinical syndromes included limbic encephalitis (LE) or epilepsy (Ep) (n = 34, 61.82%), stiff-person syndromes (SPS) (n = 18, 32.73%), autoimmune cerebellar ataxia (ACA) (n = 11, 20%), and overlap syndrome in eight (14.55%) patients. Thirty-two (58.2%) patients had comorbidities of other autoimmune diseases, including Hashimoto thyroiditis (n = 17, 53.13%), T1DM (n = 11, 34.78%), vitiligo (n = 6, 18.75%), and others (n=5, 15.63%). Two (3.64%) patients had tumors, including thymoma and small cell lung cancer. Fifty-one (92.7%) patients received first-line immunotherapy (glucocorticoids and/or IV immunoglobulin), and 4 (7.3%) received second-line immunotherapy (rituximab). Long-term immunotherapy (mycophenolate mofetil) was administered to 23 (41.8%) patients. At the median time of 15 months (IQR 6–33.75 month, range 3–96 month) of follow-up, the patients' median modified Rankin Score (mRS) had declined from 2 to 1. Thirty-eight (70.4%) patients experienced clinical improvement (mRS declined ≥1), 47 (87%) had favorable clinical outcomes (mRS ≤2), and nine were symptom-free (16.7%). The sustained response to immunotherapy ranged from 7/15 (63.63%) in ACA patients and 22/34 (64.7%) in LE/Ep patients to 14/17 (82.35%) in SPS patients.ConclusionsLE/Ep was the most common neurological phenotype of GAD65 antibody neurological autoimmunity in our cohort. Most patients had comorbidities of other autoimmune diseases, but underlying tumors were rare. Most patients responded to immunotherapy. However, the long-term prognosis varied among different clinical phenotypes.

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Section: Discussionmentioning

confidence: 99%

Neurological disorders associated with glutamic acid decarboxylase 65 antibodies: Clinical spectrum and prognosis of a cohort from China

et al. 2022

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“…Our review showed that 83.16% of the patients receiving IVIG had some form of improvement demonstrated either through various scoring systems or subjectively. While comparing the efficacy of IVIG therapy with plasma exchange, a review by Czempik et al revealed that 70% (n = 28) showed marked improvement, while 30% showed no improvement, among 40 patients receiving therapeutic plasma exchange 69 . GABA is involved in a number of synaptic transmissions in the brain that control muscle tone, autonomic responses, fear, arousal, and behavior 58 .…”

Section: Discussionmentioning

confidence: 99%

“…showed no improvement, among 40 patients receiving therapeutic plasma exchange. 69 GABA is involved in a number of synaptic transmissions in the brain that control muscle tone, autonomic responses, fear, arousal, and behavior. 58 Increased GABAergic transmission due to the use of IVIG results in decreased muscle stiffness as well as a decrease in the frequency of spasms triggered by fear and emotional upsets in patients.…”

Section: Discussionmentioning

confidence: 99%

Intravenous immunoglobulin in the management and outcome of Stiff‐Person syndrome: A systematic review

Aryal

Shrestha

Homagain

et al. 2022

Clinical & Exp Neuroim

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Background: Stiff Person Syndrome (SPS), a rare immune-mediated neuromuscular disorder, is characterized by rigidity, stiffness, and intermittent spasms of axial and extremity muscles. Various immunotherapies including intravenous immunoglobulin (IVIG) have been used for this autoimmune condition. Here we aim to review the role of IVIG in the treatment of SPS along with its outcome.Methods: A systematic literature search of PubMed and Embase was conducted to identify the relevant published articles against the predefined criteria using suitable keywords combinations till September 20, 2021. Data were extracted to produce descriptive information of SPS patients on demographics, diagnostics, treatment with IVIG, and outcome.Results: Twelve studies with 216 patients were included in the review and 63.89% of them had classical SPS. Glutamic acid decarboxylase (GAD) autoantibodies were present in 72.68% of the patients and 57.89% in whom electromyography (EMG) was performed had continuous motor activity. IVIG therapy was given to 95 patients in different regimens in various studies and varying scoring systems were used to assess the outcome, and 83.16% showed some form of improvement, 14.74% showed no improvement, while 2.10% worsened. None of the included studies mentioned an adverse effect of IVIG in the patients. Conclusion: IVIG may benefit patients with SPS along with other medications. Owingto the rarity of the disease and insufficient studies on the assessment of immunotherapy in SPS, longitudinal studies with a sizable number of patients are required to clarify clinical course, treatment, and outcome in SPS with the use of IVIG.anti-glutamic acid decarboxylase antibodies, immunotherapy, intravenous immunoglobulin, stiff limb syndrome, stiff person syndrome | INTRODUCTIONStiff Person Syndrome (SPS) is a rare and incapacitating neuromuscular disorder characterized by muscle rigidity, stiffness, and intermittent spasms involving the muscles of axial and extremities. 1,2 Activation of agonist and antagonist muscles results in stiffness, due to which patients have frequent falls, muscle spasms, and chronic muscle pain. 3,4 Originally described by Moersch and Woltman, the etiology of SPS is not known but upcoming evidence suggests a likely autoimmune cause. 2,5 It is associated with a high level of anti-glutamic acid decarboxylase (GAD) antibodies, an enzyme controlling the formation of gamma-amino butyric acid (GABA). 6,7 With no structural damage

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“…In a small randomized controlled trial with 16 patients who had SPS and anti-GAD Abs, intravenous immunoglobulins (IVIGs) were effective in reducing spasms and improving functional outcomes (13). A recent study suggested the safety and efficacy of therapeutic PLEX as an adjunct to immunosuppressive therapy in GAD Ab-associated SPS (14). However, in paraneoplastic SPS, randomized controlled trials are lacking due to the rarity of the disease.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Amphiphysin Antibody-Associated Stiff-Limb Syndrome and Myelopathy: An Unusual Presentation of Breast Cancer in an Elderly Woman

et al. 2021

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Background: Paraneoplastic stiff-limb syndrome (SLS) is a rare manifestation of underlying malignancy and could have distinctive features different from the classic stiff-person syndrome (SPS).Case Description: We present a case of anti-amphiphysin antibody (Ab)-associated paraneoplastic SLS, in an 83-year-old woman with invasive ductal carcinoma of the breast. She presented with stiffness, painful spasms of the distal legs, and asymmetrical fixed posturing of the foot. There are coexisting long-tract disturbance and lower-extremity weakness. Treatment with diazepam provided symptomatic relief while plasma exchange (PLEX) did not lead to significant clinical improvement. The patient was bedridden within 3 months and passed away within 6 months from symptom onset.Conclusion: This case highlights the importance of recognition of uncommon presentation of SPS and its oncological significance. This entity requires a high degree of suspicion for initiation of the proper workup. The rapid identification and treatment of the underlying tumor might offer the best chance for recovery.

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Efficacy and safety of therapeutic plasma exchange in stiff person syndrome

Cited by 10 publications

References 32 publications

Neurological disorders associated with glutamic acid decarboxylase 65 antibodies: Clinical spectrum and prognosis of a cohort from China

Neurological disorders associated with glutamic acid decarboxylase 65 antibodies: Clinical spectrum and prognosis of a cohort from China

Intravenous immunoglobulin in the management and outcome of Stiff‐Person syndrome: A systematic review

Case Report: Amphiphysin Antibody-Associated Stiff-Limb Syndrome and Myelopathy: An Unusual Presentation of Breast Cancer in an Elderly Woman

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