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01-01-2018 | Case Report

Paraneoplastic Stiff Person Syndrome Secondary to Pancreatic Adenocarcinoma

Authors: Stephanie Yu Shan Yong, Jin Yao Teo, Kok Pin Yong, Brian K. P. Goh

Published in: Journal of Gastrointestinal Surgery | Issue 1/2018

Excerpt

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.1,2 Up to 5% of all SPS patients are associated with malignancies;3 however, paraneoplastic SPS is usually not associated with anti-GAD antibodies but with anti-amphiphysin or anti-gephyrin antibodies.4,5 …

Solimena M, Folli F, Denis-Donini S, Comi GC, Pozza G, De Camilli P et al. Autoantibodies to glutamic acid decarboxylase in a patient with stiff-man syndrome, epilepsy, and type I diabetes mellitus. The New England journal of medicine. 1988;318(16):1012–20. https://doi.org/10.1056/nejm198804213181602.

Dalakas MC, Fujii M, Li M, McElroy B. The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome. Neurology. 2000;55(10):1531–5.CrossRefPubMed

Hadavi S, Noyce AJ, Leslie RD, Giovannoni G. Stiff person syndrome. Practical Neurology. 2011;11(5):272–82. https://doi.org/10.1136/practneurol-2011-000071.CrossRefPubMed

De Camilli P, Thomas A, Cofiell R, Folli F, Lichte B, Piccolo G et al. The synaptic vesicle-associated protein amphiphysin is the 128-kD autoantigen of stiff-man syndrome with breast cancer. The Journal of experimental medicine. 1993;178(6):2219–23.

Butler MH, Hayashi A, Ohkoshi N, Villmann C, Becker CM, Feng G et al. Autoimmunity to gephyrin in stiff-man syndrome. Neuron. 2000;26(2):307–12.CrossRefPubMed

Brizzi MP, Sonetto C, Tampellini M, Di Maio M, Volante M, Scagliotti GV. Unusual paraneoplastic neurological syndrome secondary to a well differentiated pancreatic neuroendocrine tumor: a case report and review of the literature. BMC Cancer. 2015;15. https://doi.org/10.1186/s12885-015-1923-4.

Graus F, Delattre J, Antoine J, Dalmau J, Giometto B, Grisold W et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. Journal of Neurology, Neurosurgery, and Psychiatry. 2004;75(8):1135–40. https://doi.org/10.1136/jnnp.2003.034447.CrossRefPubMedPubMedCentral

Title: Paraneoplastic Stiff Person Syndrome Secondary to Pancreatic Adenocarcinoma
Authors: Stephanie Yu Shan Yong
Jin Yao Teo
Kok Pin Yong
Brian K. P. Goh
Publication date: 01-01-2018
Publisher: Springer US
Published in: Journal of Gastrointestinal Surgery / Issue 1/2018
Print ISSN: 1091-255X
Electronic ISSN: 1873-4626
DOI: https://doi.org/10.1007/s11605-017-3611-y

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Paraneoplastic Stiff Person Syndrome Secondary to Pancreatic Adenocarcinoma

Excerpt

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