A recent case study recommends that type B insulin resistance should be one of the differential diagnoses in patients with aquaporin-4 immunoglobulin G-positive neuromyelitis optica spectrum disorder presenting with altered glucose metabolism, regardless of the presence of systemic lupus erythematosus.
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune condition that is characterized by optic neuritis and myelitis, predominantly affecting the female population. The pathogenesis of this disease is based on aquaporin-4 immunoglobulin G (AQP4-IgG+) autoantibodies.
Among the various drugs that have been approved for the treatment of AQP4-IgG+ NMOSD, eculizumab is a complement (C)-5 antibody that is associated with decreased relapse risk in NMOSD patients. This study reported on the development of fatal septic complications following type B insulin resistance (TBIR) in a patient receiving eculizumab for the treatment of AQP4-IgG+ NMOSD. The study findings are published in the Journal of Neurology.
Baseline Characteristics
The study reported on a female patient who was diagnosed with AQP4-IgG+ NMOSD at 16 years of age. The diagnosis was preceded by attacks of optic neuritis, brainstem myelitis, and encephalitis. The disease was not responsive to medical treatment with immunosuppressive therapy, with subsequent occurrence of further episodes of myelitis and the development of multiple infections. The patient developed severe tetraparesis due to failure of treatment with disease-modifying drugs.
Eculizumab for AQP4-IgG+ NMOSD
The patient was administered eculizumab intravenously 6 years after the diagnosis of AQP4-IgG+ NMOSD, which led to the stabilization of the disease. Eight months following the initiation of treatment, the patient developed TBIR. This was characterized by recurrent hyperglycemic episodes, followed by postprandial hypoglycemia. The diagnosis of emerging TBIR was based on hyperinsulinemia, marked insulin resistance, hyperandrogenism, and higher levels of C peptide. The patient also suffered from severe pneumonia caused by Klebsiella pneumoniae. Despite treatment efforts, the patient died due to sepsis-related multiorgan failure approximately 18 months following the start of eculizumab treatment.
Eculizumab and Autoimmune Comorbidities
While eculizumab is an effective treatment for patients diagnosed with AQP4-IgG+ NMOSD, patients with autoimmune comorbidities are at increased risk for the development of severe infections and associated complications.
Source:
Doubrovinskaja, S., Korporal‐Kuhnke, M., Jarius, S., Haas, J., & Wildemann, B. (2023). Newly emerging type B insulin resistance (TBIR) during treatment with eculizumab for AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD): fatal outcome. Journal of Neurology. https://doi.org/10.1007/s00415-023-12071-9
