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Stiff Person Syndrome – Dual Treatment — SCE Neurology MCQ

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HardMovement DisordersStiff Person Syndrome – Dual TreatmentSCE Neurology

A 45-year-old woman with anti-GAD65 stiff-person syndrome has disabling stimulus-sensitive spasms and falls despite optimised diazepam and baclofen. What is the most appropriate next disease-modifying treatment?

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Correct answer: B — A course of intravenous immunoglobulin delivered under neuroimmunology supervision

For function-limiting stiff-person syndrome despite symptomatic GABAergic therapy, IVIg has the strongest trial evidence for improvement in stiffness and spasm burden. Other immunotherapies may be considered in refractory disease through a specialist service.

Reference: Dalakas et al., high-dose intravenous immune globulin for stiff-person syndrome: https://pubmed.ncbi.nlm.nih.gov/11756576/