Gad-Awful Spasms

Abstract

Stiff person syndrome (SPS) is a rare autoimmune neurological disease that causes progressive, fluctuating, painful muscle rigidity and spasms, generally beginning in the axial muscles, truncal region, and progressing to proximal musculature. A hyperlordotic posture is considered an early clinical hallmark of the condition. The most common pathological correlate is with anti-glutamic acid decarboxylase (GAD) 65 antibodies, but several other associated antibodies have been identified There is a clinical spectrum of disease with variants including stiff leg syndrome, progressive encephalomyelitis with rigidity and myoclonus (PERM), and a paraneoplastic variant, often associated with anti-amphiphysin antibodies. Electromyographic findings reveal continuous motor activity. Treatment modalities for SPS focus primarily on symptomatic relief to improve quality of life. Gamma amino butyric acid (GABA)ergic agonists, including benzodiazepines, such as diazepam and baclofen, are first-line therapies for muscle rigidity and spasms. Immunotherapies such as corticosteroids, intravenous immunoglobulin (IVIg), plasmapheresis, and rituximab have demonstrated benefit, As symptoms can be provoked by anxiety and emotional stressors, psychological support with cognitive behavioral therapy should be considered.