Prevention of an Acute Severe Exacerbation of Stiff-person Syndrome during Surgery

Therapeutic Plasma Exchange in an Uncommon Disease: Stiff-Person Syndrome: Case Report

“…1,2,9 Drugs that enhance GABA neurotransmission, such as diazepam, vigabatrin, and baclofen, provide mild to modest relief of clinical symptoms. 14 The rarity of this condition limits the feasibility of controlled clinical trials in the treatment of SPS, but the available evidence suggest that drugs that increase cortical and spinal inhibition such as benzodiazepines and drugs that provide immune modulation such as intravenous immunoglobulin, TPE, and prednisone are effective treatment options. 3,4,8,10,11,14,15 Treatment with IVIg, anti-anxiety drugs, muscle relaxants, anti-convulsants and pain relievers will improve the symptoms, but will not cure the disorder.…”

Stiff-Person Syndrome First Manifesting in Pregnancy

“…As our patient was administered low doses of symptomatic therapy (diazepam and baclofen), she probably had spontaneous postpartum remission. By analogy with postpartum administration of intravenous immunoglobulins to a patient with multiple sclerosis and a high risk of relapse, Gros et al [5] recommend intravenous immunoglobulin therapy prior to the scheduled operative procedure, to prevent SPS exacerbation. Great caution is warranted in anaesthesia for possible postoperative hypotonia due to cumulative GABA-ergic action of anaesthetics and GABA-mimetics generally used by SPS patients.…”