Stiff-person syndrome is a rare disease characterized by muscle rigidity and painful spasms in the axial and limb muscles. The authors reported here a case of an axilally lymphadenectomy in a 46-year-old woman with stiff-person syndrome. With train of four ratio (TOFR) monitoring at the ulnar nerve, general anesthesia was induced and maintained with fentanyl, vecuronium and propofol with target controlled infusion. A TOFR, BIS monitor and invasive arterial pressure monitoring were employed. During the operation, there was no muscle rigidity and spasm. Ten minutes after the operation, she was fully awake and train of four ratio recovered to 95%, and extubated uneventfully. We chose propofol, because of previous reports about prolonged hypotonicity by interaction of baclofen and isoflurane. Preoperative good symptom control, choice of total intravenous anesthesia (TIVA), and application of the electrical nerve stimulator prevented postoperative hypotonia and resulted in safe anesthetic management.
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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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