Hanwen Zhang1, Wenshi Wei1. 1. Department of Neurology, Huadong Hospital Affiliated to Fudan University No. 221, West Yan An Road, Shanghai, China.
Abstract
BACKGROUND: Stiff-person syndrome (SPS) manifests by progressive rigidity along with muscle spasms that affect the axial and limb muscles. First discovered in 1956, significant progress has been made in its clinical characterization, comprehension of pathogenesis, as well as effective treatment therapy. CASE PRESENTATION: A 67-year old female patient presented with a 2-year history of progressive stiffness along with painful spasms in both legs, with her condition worsening over the previous year making it considerably difficult for her to stand and walk. Here, we report a Stiff-person syndrome patient (SPS) with lung adenocarcinoma who was positive for anti-glutamate decarboxylase (anti-GAD) antibodies. Treatment with hormones and gamma-globulin improved her symptoms. In addition, we present a literature review of SPS patients with tumors. CONCLUSIONS: The diagnosis of autoimmune SPS was on the basis of clinical, electrophysiological, as well as immunological findings. Early SPS detection is critical to preventing long-term disability. AJTR
BACKGROUND: Stiff-person syndrome (SPS) manifests by progressive rigidity along with muscle spasms that affect the axial and limb muscles. First discovered in 1956, significant progress has been made in its clinical characterization, comprehension of pathogenesis, as well as effective treatment therapy. CASE PRESENTATION: A 67-year old female patient presented with a 2-year history of progressive stiffness along with painful spasms in both legs, with her condition worsening over the previous year making it considerably difficult for her to stand and walk. Here, we report a Stiff-person syndrome patient (SPS) with lung adenocarcinoma who was positive for anti-glutamate decarboxylase (anti-GAD) antibodies. Treatment with hormones and gamma-globulin improved her symptoms. In addition, we present a literature review of SPS patients with tumors. CONCLUSIONS: The diagnosis of autoimmune SPS was on the basis of clinical, electrophysiological, as well as immunological findings. Early SPS detection is critical to preventing long-term disability. AJTR
Authors: Divyanshu Dubey; Jiraporn Jitprapaikulsan; Hongyan Bi; Rocio Vazquez Do Campo; Andrew McKeon; Sean J Pittock; Janean K Engelstad; John R Mills; Christopher J Klein Journal: Neurology Date: 2019-10-17 Impact factor: 9.910