Fulminant Acute Ascending Hemorrhagic Myelitis Treated with Eculizumab

Hsam, Nang Boe Ohnmar and Angstwurm, Klemens and Peters, Sebastian and Fuchs, Kornelius and Schuierer, Gerhard and Bogdahn, Ulrich and Weissert, Robert (2017) Fulminant Acute Ascending Hemorrhagic Myelitis Treated with Eculizumab. FRONTIERS IN NEUROLOGY, 8: 345. ISSN 1664-2295,

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Abstract

We describe an 18-year-old patient who developed back pain, rapidly ascending sensomotory deficits, bladder dysfunction, Lhermitte's sign, absent abdominal reflexes of all three levels, brisk tendon reflexes, and positive Babinski's sign. Magnetic resonance imaging of the spinal cord showed a long segment of cervical and thoracic intramedullary signal hyperintensity suggesting a longitudinally extensive transverse myelitis possibly within the course of a fast progressing ascending immune-mediated hemorrhagic myelopathy. Throughout his illness, the patient deteriorated with tetraplegia, cardiac arrest, and respiratory failure although he received, after exclusion of infective causes, therapy with steroids, immunoglobulins, plasmapheresis, and cyclophosphamide. Interestingly, treatment with the C5-inhibitor eculizumab to prevent complement-mediated spinal cord injury achieved an arrest of clinical deterioration. We propose eculizumab as treatment in fast progressive and potentially fatal immune-mediated spinal cord injury. Furthermore, this case raises awareness for the process of clinical decision-making in severe myelopathies.

Item Type: Article
Uncontrolled Keywords: EXTENSIVE TRANSVERSE MYELITIS; OPTICA SPECTRUM DISORDERS; NEUROMYELITIS-OPTICA; SPINAL-CORD; acute myelopathy; transverse myelitis; eculizumab; autoimmune myelitis; longitudinally extensive transverse myelitis; spinal cord hemorrhage; C5-inhibitor; interleukin-6
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Neurologie
Depositing User: Dr. Gernot Deinzer
Date Deposited: 14 Dec 2018 13:16
Last Modified: 22 Feb 2019 06:29
URI: https://pred.uni-regensburg.de/id/eprint/1522

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