Anaesthetic management of a patient with a unique combination of anti-N-methyl-D-aspartate receptor encephalitis and stiff-person syndrome

Abstract

Stiff-person syndrome (SPS) and anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis are rare paraneoplastic syndromes caused by antibodies that target the central nervous system. Here, we describe a 26-year-old woman who presented with psychosis, amnesia, rigidity and fever. After extensive diagnostic and laboratory workup, she was diagnosed with an ovarian teratoma which was causing the symptoms of anti-NMDAR encephalitis and SPS. The patient was successfully treated with laparoscopic removal of the ovarian tumour under general anaesthesia. She was placed on immunosuppressant medications preoperatively and postoperatively, and her symptoms gradually resolved. Although there are case reports regarding the anaesthetic management of SPS and anti-NMDAR encephalitis, our study is the first report of a patient afflicted with both conditions.

Brain anomalies in early psychosis: From secondary to primary psychosis

“…Isolated frontal nonspecific white matter lesions have been described in anti-NMDAR encephalitis (Endres et al 2019;Gharedaghi et al 2018). A left frontal lobe expansive lesion with well-defined limits was seen in a grade II meningioma of the cerebral falx in a patient who had been followed in psychiatry for 25 years (since he was 24) with various diagnoses along the schizophrenia spectrum.…”

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

“…To date, SPSSDs include the following: (1) partial SPS, limited to extremities and often only one limb (stiff limb syndrome, SLS) or the torso; (2) SPS-plus, with classic SPS symptoms that exist in combination with cerebellar and/or brainstem findings; (3) PERM; and (4) some overlapping syndromes, such as classic SPS with epilepsy or limbic encephalitis (LE) ( 7 ), classic SPS with myasthenia gravis ( 8 ), classic SPS with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis (NMDARE-SPS) ( 9 ), classic SPS with central sleep apnea ( 10 ), and classic SPS with pure red blood cell aplasia ( 11 ). Most patients with SPSSD are middle-aged females; however, some patients with SPSSD are either pediatric individuals or adult males.…”

“…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 ). Furthermore, thyroid and renal cell cancers reportedly coexist with colon cancer and SPSSD ( 21 ).…”

“…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 ).…”