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Paraneoplastic Stiff Person Syndrome Secondary to Pancreatic Adenocarcinoma

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Stiff person syndrome (SPS) is a rare neurological disorder, characterised by progressive fluctuating muscular rigidity and spasms. Although the exact pathogenesis is unclear, glutamic acid decarboxylase (GAD) antibody, the enzyme… Click to show full abstract

Stiff person syndrome (SPS) is a rare neurological disorder, characterised by progressive fluctuating muscular rigidity and spasms. Although the exact pathogenesis is unclear, glutamic acid decarboxylase (GAD) antibody, the enzyme responsible for the synthesis of γ-aminobutyric acid (GABA), is found in approximately 60 to 80% of SPS patients. Up to 5% of all SPS patients are associated with malignancies; however, paraneoplastic SPS is usually not associated with anti-GAD antibodies but with anti-amphiphysin or anti-gephyrin antibodies. We report the case of a patient who presented with paraneoplastic SPS secondary to pancreatic adenocarcinoma that resolved following a Whipple procedure. Subsequently, she had a recurrence of her SPS symptoms 15months after the Whipple procedure, which preceded clinically detected recurrence of pancreatic cancer by 3 months. Case Report

Keywords: person syndrome; pancreatic adenocarcinoma; stiff person; sps; secondary pancreatic

Journal Title: Journal of Gastrointestinal Surgery
Year Published: 2017

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