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Folia Neuropathologica
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vol. 61
Case report

Off-label use of eculizumab for neurological symptoms and progressive vision loss

Alicja Kuźniewska
Marta Jaskólska
Mariusz Kwarciany
Aleksandra Ciarka
Rafał Pęksa
Zbigniew Zdrojewski
Marcin Okrój

Department of Cell Biology and Immunology, Intercollegiate Faculty of Biotechnology, University of Gdańsk and Medical University of Gdańsk, Poland
Department of Rheumatology, Clinical Immunology, Geriatrics and Internal Medicine, Medical University of Gdańsk, Poland
Department of Neurology, Division of Adult Neurology, Medical University of Gdańsk, Poland
Department of Pathomorphology, Medical University of Gdańsk, Poland
Folia Neuropathol 2023; 61 (4):442-447
Online publish date: 2023/12/29
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We describe the results of eculizumab treatment of a patient with pachymeningitis, inflammatory infiltration of the left frontal lobe, and cerebral hematoma, who presented with progressive vision loss, epileptic seizures, and abnormal pattern of the complement system parameters. A 30-year-old female patient, initially diagnosed with hypereosinophilia and a tumour of the left orbit, developed a significant visual impairment in the left eye, progressive vision loss in the right eye, and neurological symptoms in the form of epileptic seizures and behavioural changes. Magnetic resonance imaging (MRI) revealed thickening of the dura mater in the left frontal area, slight oedema of the cortex, and subcortical white matter. Orbit biopsy showed non-specific inflammatory infiltrates. Despite the initial good response, symptoms progressed during treatment with glucocorticoids and immunosuppressants. Increased activity of the alternative complement pathway accompanied by a low level of its main inhibitor, factor H (FH), and the presence of anti-FH autoantibodies, was found. Genetic analysis revealed several missense variants of complement proteins, including two disease-linked mutations in FH (p.H402Y) and FI (T300A). An attempt to apply a complement C5 blocker, eculizumab, has been made. Neurological symptoms subsided, vision loss was inhibited, laboratory parameters improved, and discontinuation of steroid therapy was possible. The case underlines the role of complement system dysregulation in neurological distress.

eculizumab, complement system, epilepsy, cerebral hematoma, vision loss

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