Affiliations: [a] Department of Pediatrics, Levine Children’s Hospital, Atrium Health, Charlotte, NC, USA
| [b] Division of Pediatric Allergy and Immunology, Mayo Clinic Children’s Center, Rochester, MN, USA
| [c] The Allergy, Asthma, and Sinus Center, Knoxville, TN, USA
| [d] Pulmonary and Critical Care and Infectious Disease, Wake Forest School of Medicine, Winston-Salem, NC, USA
Correspondence:
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Correspondence to: Niraj C. Patel, MD, MS, Department of Pediatrics, Section of Infectious Disease and Immunology, Levine Children’s Hospital, Atrium Health, PO Box 32861, Charlotte, NC, 28203, USA. Tel.: +1 704 381 6803; Fax: +1 704 381 6841; E-mail: Niraj.Patel@AtriumHealth.org.
Abstract: Stiff Person Syndrome (SPS), a rare autoimmune neurologic disorder characterized by fluctuating muscle spasms and rigidity, is mediated by autoantibodies to glutamic acid decarboxylase (GAD) antibodies. Symptoms of SPS have been shown to improve after administration of intravenous immunoglobulin (IVIG) however, there is a paucity of information regarding use of SCIg in SPS. Four patients with Stiff Person Syndrome were treated with SCIgPro20 for a period between 31 to 101 months. Most reactions were local and mild. All patients reported improvement in spasticity, and 2 patients reported improvement in seizure frequency. SCIgPro20 was well tolerated in patients with SPS and was associated with improvement in symptoms.
Keywords: Stiff Person Syndrome, immunodeficiency, immunoglobulin, subcutaneous
