Stiff-person syndrome: an atypical presentation and a review of the literature
Hospital practice (1995)
Department of Emergency Medicine; Department of Medicine; Fellows and Residents
Stiff-person syndrome, anti-glutamic acid decarboxylase, general neurology, neuromuscular disorder, paraneoplastic syndrome, stiff-limb syndrome
: Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder associated with muscle rigidity and spasms. A number of antibodies have been associated with disorder, including anti-glutamic acid decarboxylase and anti-amphiphysin.; In this report, we present a rare case of a 79-year-old woman who presented with bilateral lower extremity weakness who was ultimately diagnosed with stiff-limb syndrome, a rare variant of SPS. Extensive laboratory and CSF studies were unrevealing. Electromyography showed significant peroneal motor neuropathy and complex repetitive discharges in the left tibialis anterior muscle. Antibodies to glutamic acid decarboxylase were significantly elevated at 124 units/mL. She was subsequently started on oral diazepam with significant improvement in her symptoms.: The presentation of SPS can vary based on epidemiologic factors, clinical symptoms, and associated disorders. These forms can have overlapping features which may make the categorization of patients into one of these forms challenging.
Lin, B. C., Johal, J., Sivakumar, K., Romano, A. E., & Yacoub, H. A. (2021). Stiff-person syndrome: an atypical presentation and a review of the literature. LVHN Scholarly Works. Retrieved from https://scholarlyworks.lvhn.org/research-historical-works/9