The
stiff person syndrome (SPS) is an extremely rare
neurological disorder with primarily immune-mediated etiology. The cardinal symptoms are progressive, fluctuating axial/proximal limb muscle stiffness and
spasms. The diagnosis is based on the clinical picture, electromyography examination and detection of
antibodies to
glutamic acid decarboxylase (anti-GAD). Adverse effects of medications might preclude its use or increase in dosing, therefore symptomatic and/or immunomodulatory medical
therapy might be ineffective in acute exacerbation of the disease. We present a case of a 49-year-old female with exacerbation of SPS, in whom some standard
pharmacotherapy could not be introduced (
clonazepam,
baclofen used in the past) and doses of existing standard medications could not be increased (
diazepam,
gabapentin, and
levetiracetam) due to adverse effects. Moreover, a newly introduced medication (
methylprednisolone) also led to a serious adverse effect (severe
hyperglycemia). The patient underwent therapeutic
plasma exchange (TPE) with good effect and no complications. We review the literature regarding the efficacy and safety profile of TPE in exacerbation of SPS unresponsive to medical
therapy. The procedure seems to have a good safety profile as an adjunct
therapy for exacerbation of SPS not responding to standard medical
therapy in this patient population.