Background: Longitudinally extensive transverse myelitis (LETM) is a demyelinating condition that is associated with diseases such as neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis, collagen vascular disease, or can be idiopathic. LETM can be severe enough to cause quadraparesis, marked sensory dysfunction, and respiratory failure. Rarely, these patients are unresponsive to conventional immune therapy. Methods: We report two cases of severe LETM with acute development of quadraparesis and respiratory failure requiring intensive care admission and failure to respond to high-dose corticosteroids, plasma exchange, IVIg and rituximab. Disease cessation and ultimately, significant recovery, was achieved after an 8-day cyclophosphamide induction. Results: A 21 yo female with antibody positive NMOSD and a 19 yo male with idiopathic LETM remained quadraparetic and ventilator dependent with active MRIs despite multiple courses of intravenous methylprednisolone, plasma exchanges, and in the NMOSD patient, IVIg and a 4-week course of rituximab. Both patients ultimately improved significantly and are now ambulatory with subsequent cyclophosphamide induction. Conclusions: In patients with severe LETM of presumed immune origin, who fail to respond to corticosteroids and plasma exchange, cyclophosphamide induction should be considered. This agent provides a more robust immunosuppressive response and can be induced rapidly. Cyclophosphamide effects and supportive evidence are further discussed.

Background: The Gilenya® Go ProgramTM offers education and support services, including coordination of first dose observation (FDO) and follow-up contact to reinforce monitoring recommendations and compliance in fingolimod-treated relapsing-remitting multiple sclerosis (RRMS) patients. Methods: Data were analyzed for patients enrolled in the Canadian Gilenya® Go ProgramTM from March 2011 to January 2016. The retention to fingolimod therapy, reasons for treatment discontinuation and incidence of adverse events (AEs) during treatment are reported. Results: At data cut-off, 3956 patients had completed FDO; 3201 patients were being actively treated. Mean age at enrolment was 41.0 years; 74.9% patients were female. The overall fingolimod exposure was 7869 patient-years. Most recent previous therapies (n=3746) included interferons (43.3%) and glatiramer acetate (29.6%). Most common reasons for switching to fingolimod (n=3674) was lack of efficacy (31.8%). Retention to therapy at data cut-off was 81.3%. AEs (45.2%) were the most common reason (n=334) for treatment discontinuation and included low lymphocyte count/abnormal hematology values (13.8%), gastrointestinal disturbances (6.9%), and elevated liver enzyme levels (7.8%). Adherence to recommended ophthalmic examination was 92.4%. Conclusions: In real-world clinical practice in Canada, adherence to both fingolimod treatment and monitoring was high. The Gilenya® Go Program™ helps to meet the safety monitoring recommendations for fingolimod-treated RRMS patients.

Background: Despite the availability of cell-based assays for aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibodies provincially, outside confirmatory testing is often performed (typically Mayo Clinic Laboratories, USA) when results deviate from expected. It is unknown how often this costly undertaking (upwards of $1,200 CAN) alters diagnosis and management. Methods: We undertook a quality improvement project evaluating the concordance/discordance rate with select chart review in all patients who had cell-based AQP4 or MOG IgG antibody testing at Mitogen Diagnostics (MitogenDx; Calgary, Alberta) and subsequent testing at Mayo Clinic Laboratories from as early as 2010 to July 2020. Results: Preliminary review of data from January 2016 to July 2020 retrieved 145 paired tests; 10 of which were discordant (concordance rate: 93.1%). Chart review confirmed 9 truly discordant cases, often associated with AQP4 or MOG weak-positive results (7/9 cases) or presumed false negative AQP4 results in prototypical neuromyelitis optica spectrum disorder (2/9 cases). Conclusions: Discordant results were rare when comparing MitogenDx local AQP4/MOG antibody test results to those referred out to Mayo Clinic Laboratories, impacting diagnosis and treatment in only 3 patients out of the total. Our results suggest costly outside confirmatory testing of AQP4/MOG antibodies could be reduced.