Journal
JOURNAL OF NEUROMUSCULAR DISEASES
Volume 8, Issue 6, Pages 973-977Publisher
IOS PRESS
DOI: 10.3233/JND-200616
Keywords
Stiff Person Syndrome; immunodeficiency; immunoglobulin; subcutaneous
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Funding
- CSL Behring LLC, King of Prussia, PA, USA
- CSL Behring
- Takeda
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SCIgPro20 treatment in patients with Stiff Person Syndrome resulted in mild and local reactions, improvement in spasticity symptoms, and reduced seizure frequency in some patients.
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.
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