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

Abstract

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