Anesthetic management of a patient with Stiff-Person Syndrome and endometrial cancer for robotic surgery: A case report

Abstract

Stiff-person syndrome (SPS) is a rare disorder of the nervous system, characterized by muscle stiffness, rigidity, and painful spasms involving truncal and limb musculature that may severely limit mobility.Our case documents a 53-year-old patient with SPS and endometrial cancer who was positive for anti-GAD and paraneoplastic antibodies, who presented to our institution for robotic surgery. These patients are at high risk for prolonged hypotonia and mechanical ventilation. Our patient underwent general anesthesia without complications despite multiple comorbidities.

What to expect from paraneoplastic syndromes of the nervous system in uterine cancer: A review of the literature

“…It is defined by heightened muscle activity resulting from glutamic acid decarboxylase (GAD) blockade, usually mediated by anti-GAD antibodies, however, in most paraneoplastic SPS cases, anti-GAD antibodies are not present ( McKeon et al, 2012 ) and the syndrome is linked to anti-amphiphysin, anti-gephyrin, and anti-Ri antibodies ( Grimaldi et al, 1993 ). A report by Yeoh et al introduces a 53-year-old patient with anti-GAD (+) SPS diagnosed with endometrial cancer one year after the syndrome’s diagnosis ( Yeoh et al, 2020 ). The patient exhibited mild yet progressive symptoms and was treated with opioids.…”

An update on malignant tumor-related stiff person syndrome spectrum disorders: clinical mechanism, treatment, and outcomes

“…The major SPSSD subtype is classic SPS ( 11 , 68 , 69 , 73 , 77 , 80 , 92 – 98 , 134 , 135 ), followed by SPS with myasthenia gravis ( 21 – 25 , 29 , 31 , 34 – 36 , 86 , 89 , 90 , 135 , 136 ), PERM ( 91 ), SLS ( 57 ), progressive dizziness and unstable gait ( 87 ), and NMDAR-SPS ( 9 ), as shown in Table 5 . Moreover, the major carcinoma associated with SPSSD subtypes is breast cancer ( 21 , 39 , 42 , 47 , 50 , 51 , 56 , 57 , 137 ), followed by thymoma ( 11 , 80 , 91 – 98 , 104 – 106 , 134 , 135 ), lymphoma ( 77 , 80 ), lung cancer ( 68 , 69 , 73 ), and other carcinomas ( 21 – 25 , 29 , 31 , 34 – 36 , 86 , 89 , 90 , 135 , 136 ).…”

“…SPSSD is also associated with other carcinomas, such as mediastinal liposarcoma ( 22 ), metastatic adenocarcinoma ( 23 ), pancreatic adenocarcinoma ( 24 ), renal cell carcinoma ( 25 ), mediastinal cancer, undifferentiated carcinoma of an undetermined origin ( 26 ), multiple myeloma ( 86 ), embryonal carcinoma ( 27 ), malignant glioma ( 87 ), ovarian adenocarcinoma ( 88 ), prostate carcinoma ( 88 ), testicular seminoma and germ cell neoplasia ( 88 ), pancreatic cancer ( 88 ), melanoma ( 88 ), an invasive carcinoma of no special type ( 28 ), ovarian teratoma ( 9 ), small cell carcinoma of the bladder ( 31 ), pleuropulmonary blastoma ( 33 ), malignant mesothelioma ( 35 ), colon cancer, and Hürthle cell adenoma ( 36 ). It is also associated with overlapping cancers, such as breast cancer with colon cancer ( 30 , 39 ), chronic lymphocytic leukemia ( 81 ), thymoma and non-Hodgkin lymphoma ( 80 ), non-functioning pituitary microadenoma, and endometrial cancer ( 29 ) ( Table 4 ). Table 5 shows the other carcinomas included in 25 studies involving six SPSSD subtypes, namely, classic SPS ( 21 – 36 ), SLS ( 86 , 90 ), PERM ( 91 ), progressive dizziness and unstable gait ( 87 ), and NMDAR-SPS ( 9 ).…”