Faculty Bibliography

OBJECTIVE: To quantify the periventricular venous density in neuromyelitis optica spectrum disease (NMOSD) in comparison to that in patients with multiple sclerosis (MS) and healthy control subjects. MATERIALS AND METHODS: Sixteen patients with NMOSD, 16 patients with MS and 16 healthy control subjects underwent 7.0-Tesla (7T) MRI. The imaging protocol included T2*-weighted (T2*w) fast low angle-shot (FLASH) and fluid-attenuated inversion recovery (FLAIR) sequences. The periventricular venous area (PVA) was manually determined by a blinded investigator in order to estimate the periventricular venous density in a region of interest-based approach. RESULTS: No significant differences in periventricular venous density indicated by PVA were detectable in NMOSD versus healthy controls (p = 0.226). In contrast, PVA was significantly reduced in MS patients compared to healthy controls (p = 0.013). CONCLUSION: Unlike patients with MS, those suffering from NMOSD did not show reduced venous visibility. This finding may underscore primary and secondary pathophysiological differences between these two distinct diseases of the central nervous system.

The discovery of a highly specific biomarker of neuromyelitis optica (NMO)-the anti-aquaporin-4 (AQP4) antibody-has opened new paths to understanding disease pathogenesis and afforded a way to confirm the diagnosis in clinical practice. An important consequence of the discovery is the broadening of the spectrum of syndromes seen in the context of AQP4 autoimmunity. These syndromes have been subsumed under the rubric of NMO spectrum disorder (NMOSD). The current classification recognizes not only optic neuritis and myelitis as core syndromes of NMOSD but also cerebral, diencephalic, brainstem, and area postrema syndromes. These neurologic syndromes are the focus of our review. AQP4 is also expressed in many organs outside of the central nervous system, and this may explain some of the unusual, non-neurologic features that have been occasionally reported in NMOSD. Our review catalogues non-neurologic manifestations seen in NMOSD and concludes with a discussion of frequently associated autoimmune and neoplastic comorbidities of NMOSD.

OBJECTIVE: We aimed to analyse the effect of the introduction of fingolimod, the first oral disease-modifying therapy, on treatment utilisation and persistence in an international cohort of patients with multiple sclerosis (MS). METHODS: MSBASIS, a prospective, observational sub-study of the MSBase registry, collects demographic, clinical and paraclinical data on patients followed from MS onset (n=4718). We conducted a multivariable conditional risk set survival analysis to identify predictors of treatment discontinuation, and to assess if the introduction of fingolimod has altered treatment persistence. RESULTS: A total of 2640 patients commenced immunomodulatory therapy. Following the introduction of fingolimod, patients were more likely to discontinue all other treatments (hazard ratio 1.64, p<0.001) while more patients switched to fingolimod than any other therapy (42.3% of switches). Patients switched to fingolimod due to convenience. Patients treated with fingolimod were less likely to discontinue treatment compared with other therapies (p<0.001). Female sex, country of residence, younger age, a high Expanded Disability Status Scale score and relapse activity were all independently associated with higher rates of treatment discontinuation. CONCLUSION: Following the availability of fingolimod, patients were more likely to discontinue injectable treatments. Those who switched to fingolimod were more likely to do so for convenience. Persistence was improved on fingolimod compared to other medications.