Stiff-person syndrome (SPS) is a rare neurological condition consisting of progressive and fluctuating rigidity of the axial muscles combined with painful spasms. The pathophysiology of SPS is not fully understood, but there seems to be an autoimmune component. The use of rituximab, a chimeric monoclonal antibody targeting CD20 protein in the surface of mature B cells, for the treatment of SPS is a recent therapeutical approach showing promising results. The authors present a case report of a 41-year-old female patient diagnosed with SPS who was treated with rituximab in a public hospital in Brasília, Brazil, showing a good and safe response to the treatment so far. Our data go along with some recent articles published in the literature.
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http://dx.doi.org/10.1136/bcr.05.2010.3021 | DOI Listing |
Ann Thorac Surg Short Rep
September 2024
Department of General Thoracic Surgery, Juntendo University School of Medicine, Tokyo, Japan.
Stiff person syndrome (SPS) is a neurologic disorder, some cases of which are associated with malignant disease. Here, we report a case of thymoma-associated SPS that was successfully treated with surgical resection. A 57-year-old man with progressive muscle stiffness and weakness was diagnosed with thymoma-related SPS.
View Article and Find Full Text PDFFront Immunol
December 2024
Department of Neurology, Specialized Rehabilitation Hospital/Capital Health, Abu Dhabi, United Arab Emirates.
Med Acupunct
October 2024
Department of Family Medicine, West Virginia University, Morgantown, West Virginia, USA.
Objective: Stiff person syndrome (SPS) is a rare neurological disorder. Treatments are limited, and non-pharmacologic therapies are recommended based on symptomatology. A G2P2002 post-menopausal 60-year-old female with hypertension, obesity, and type II diabetes, and SPS secondary to a paraneoplastic process cause by endometrioid ovarian adenocarcinoma who presented to acupuncture clinic seeking treatment for SPS and its sequela.
View Article and Find Full Text PDFPract Neurol
December 2024
Neurology Department, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, UK.
We describe a woman with stiff-person syndrome (SPS), whose muscle spasms resulted in sequential bilateral femoral neck fractures. Orthopaedic fixation of the first fracture was complicated by increased muscle spasm, fracture nonunion and ultimately metalwork fracture. SPS was diagnosed following the fracture of the contralateral femoral neck, neurology assessment and detection of high-titre antibodies to glutamic acid decarboxylase.
View Article and Find Full Text PDFBMJ Case Rep
December 2024
Internal Medicine, Hirslanden Klinik Sankt Anna, Luzern, Switzerland
A patient with reactivated varicella zoster virus (VZV) manifesting in the left-sided dermatome L3 and S2-S4 developed tonic spasms which morphed into myoclonic jerks, paresis, rigidity and hypoesthesia of the left leg. Later, stimuli-sensitive myoclonus progressed to affect the upper body and was accompanied by fever surges with high-frequency myoclonus, hypertensive derailment, dysphagia and other features of the brainstem with autonomic dysfunction. Cerebrospinal fluid tested positive for VZV, MRI showed no signs of myelitis and EEG was negative for epilepsy.
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