: 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.
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http://dx.doi.org/10.1080/21548331.2021.1961456 | 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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