Contributions
Abstract: EP1350
Type: ePoster
Abstract Category: Clinical aspects of MS - MS Variants
Introduction: There are only very few cases of tumefactive demyelination of the spinal cord reported in the literature. The course is frequently unfavourable due to diagnostic difficulties, limited therapeutic success, life-threatening complications and mechanical spinal cord injury.
Objectives: To report a case of steroid-unresponsive aquaporin-4 antibody negative tumefactive demyelination of the cervical cord with limited success of treatment escalation including plasma exchange and natalizumab.
Case presentation: We present the case of a 41-year-old previously healthy woman who developed subacute sensory loss in the upper extremities. MRI disclosed a longitudinally extensive lesion in the cervical cord (atlas to C5). Several brain MRI lesions as well as CSF-specific oligoclonal bands were detected. Repeated serological testing for aquaporin-4 antibody and other infectious/autoimmune aetiologies were negative. She improved on high-dose steroids but was readmitted three weeks later due to tetraparesis. On an additional course of steroids, she required admission to the ICU after clinical deterioration and requirement for mechanical ventilation. On MRI, the cervical lesion had expanded in all planes with evidence of extensive Gd-enhancement and edema, causing additional mechanical injury. Rapid escalation with plasma exchange and later natalizumab did not improve the course. Severe atrophy of the cervical cord was present on follow-up MRI. She remained at the ICU for 219 days before being transferred to a nursing home, where she died of basilar artery thrombosis.
Conclusions: This case of fulminant tumefactive demyelination in the cervical cord and mechanical injury due to spinal cord compression reinforces the need for a multidisciplinary care team in order to improve the outcome of non-traumatic myelopathies. Additional treatment strategies including rapid surgical decompression and early initiation immunosuppression may be considered for upcoming cases.
Disclosure: ABK, HFN, WK, MRM: nothing to disclose. ET: has received research funding from UCB Pharma, Biogen-Idec, Red Bull, Merck, the European Union, FWF Österreichischer Fond zur Wissenschaftsförderung, and Bundesministerium für Wissenschaft und Forschung and has acted as a paid consultant to Eisai, Takeda, Ever Neuropharma, Biogen, Bial, Sunovion, and UCB, and has received speakers" honoraria from Biogen, Bial, Eisai, GL Lannacher, GlaxoSmithKline, Boehringer, Sunovion, Newbridge Pharma, and UCB Pharma. JS: received research funding from the Paracelsus Medical University, Bayer, Biogen-Idec, Merck and Novartis, has acted as paid consultant to Novartis and Genzyme, and has received speakers" honoraria from Biogen-Idec, Ever Neuropharma, Genzyme, Novartis and Teva-Ratiopharm.
Abstract: EP1350
Type: ePoster
Abstract Category: Clinical aspects of MS - MS Variants
Introduction: There are only very few cases of tumefactive demyelination of the spinal cord reported in the literature. The course is frequently unfavourable due to diagnostic difficulties, limited therapeutic success, life-threatening complications and mechanical spinal cord injury.
Objectives: To report a case of steroid-unresponsive aquaporin-4 antibody negative tumefactive demyelination of the cervical cord with limited success of treatment escalation including plasma exchange and natalizumab.
Case presentation: We present the case of a 41-year-old previously healthy woman who developed subacute sensory loss in the upper extremities. MRI disclosed a longitudinally extensive lesion in the cervical cord (atlas to C5). Several brain MRI lesions as well as CSF-specific oligoclonal bands were detected. Repeated serological testing for aquaporin-4 antibody and other infectious/autoimmune aetiologies were negative. She improved on high-dose steroids but was readmitted three weeks later due to tetraparesis. On an additional course of steroids, she required admission to the ICU after clinical deterioration and requirement for mechanical ventilation. On MRI, the cervical lesion had expanded in all planes with evidence of extensive Gd-enhancement and edema, causing additional mechanical injury. Rapid escalation with plasma exchange and later natalizumab did not improve the course. Severe atrophy of the cervical cord was present on follow-up MRI. She remained at the ICU for 219 days before being transferred to a nursing home, where she died of basilar artery thrombosis.
Conclusions: This case of fulminant tumefactive demyelination in the cervical cord and mechanical injury due to spinal cord compression reinforces the need for a multidisciplinary care team in order to improve the outcome of non-traumatic myelopathies. Additional treatment strategies including rapid surgical decompression and early initiation immunosuppression may be considered for upcoming cases.
Disclosure: ABK, HFN, WK, MRM: nothing to disclose. ET: has received research funding from UCB Pharma, Biogen-Idec, Red Bull, Merck, the European Union, FWF Österreichischer Fond zur Wissenschaftsförderung, and Bundesministerium für Wissenschaft und Forschung and has acted as a paid consultant to Eisai, Takeda, Ever Neuropharma, Biogen, Bial, Sunovion, and UCB, and has received speakers" honoraria from Biogen, Bial, Eisai, GL Lannacher, GlaxoSmithKline, Boehringer, Sunovion, Newbridge Pharma, and UCB Pharma. JS: received research funding from the Paracelsus Medical University, Bayer, Biogen-Idec, Merck and Novartis, has acted as paid consultant to Novartis and Genzyme, and has received speakers" honoraria from Biogen-Idec, Ever Neuropharma, Genzyme, Novartis and Teva-Ratiopharm.