Faculty Bibliography

Background. Brain lesions are common in neuromyelitis optica spectrum disorder (NMOsd) and may resemble lesions of multiple sclerosis (MS). Objectives. To describe the imaging characteristics of supratentorial lesions in NMOsd on ultrahigh-field (7 T) MRI with special attention to vessel-lesion relationship. Methods. Ten NMOsd patients, all women and all seropositive for NMO IgG, with mean age of 51.3 +/- 15.4 years and disease duration of 9.2 +/- 6.4 years, were scanned at a 7 T whole-body human MR system with high-resolution 2D gradient echo sequence optimized to best visualize lesions and venous structures, T2- and T1-weighted imaging. Results. In 10 patients with NMOsd, a total of 92 lesions were observed (mean: 9.2 +/- 8.8; range: 2-30), but only 8 lesions (9%) were traversed by a central venule. All lesions were <5 mm in diameter, and 83% were located in subcortical white matter. There were no lesions in the cortex or basal ganglia. Two patients exhibited diffuse periependymal abnormalities on FLAIR. Conclusions. Small, subcortical lesions without a central venule are the most consistent finding of NMOsd on 7 T MRI of the brain. Ultrahigh-field imaging may be useful for differentiating between NMOsd and MS.

The North American Research Committee on Multiple Sclerosis (NARCOMS) Registry is a database that contains information from over 35,000 patient volunteers on symptom severity in 11 domains commonly affected in multiple sclerosis (MS): mobility, hand function, vision, fatigue, cognition, bowel/bladder function, sensory, spasticity, pain, depression, and tremor/coordination. The Registry affords a unique opportunity to study the frequency and severity of domain-specific impairment in a contemporary, mostly treated MS cohort over the course of the disease. The objective of this work was to calculate symptom prevalence in each of the 11 domains for years 0 to 30 from symptom onset. The resulting "symptom prevalence tables" demonstrate that a majority of participants perceive at least some degree of impairment in most domains as early as the first year of disease. The severity of impairment increases with disease duration across all domains, but the patterns of disability accumulation differ. The symptom prevalence tables illustrate the magnitude of perceived impact of the disease and highlight the extent of unmet need in symptomatic management. The tables are easy to use and allow MS patients and their clinicians to compare an individual's own impairment in any of the 11 domains to that of NARCOMS participants with the same disease duration.

Background: Multiple Sclerosis Severity Score (MSSS; Roxburgh et al, Neurology; 64:1144) is a mean rank of Expanded Disability Status Scale (EDSS) among patients with same disease duration. MSSS has been used to compare disease severity in different patient populations and for monitoring treatment response in groups of patients. Clinical utility of MSSS is limited by the requirement for objective assessment of EDSS score by a trained MS specialist. Patient-Determined Disability Steps (PDDS).is a validated, nine-point disability scale that does not require clinician input. A ranking scale of PDDS, an analogue of MSSS, may be a practical and cost-effective tool for monitoring long-term outcomes in groups of patients. Objective: To develop a ranking scale of Patient-Determined Disability Steps (PDDS) using the North American Research Committee on Multiple Sclerosis (NARCOMS) Registry as the reference population. Design/Methods: All NARCOMS registrants with known PDDS and disease duration of 1-45 years at PDDS were included in the study. Frequency-ranking methodology (Roxburgh, et al. Neurology; 64:1144) was applied to the NARCOMS dataset to derive disease duration-adjusted mean ranks of PDDS scores. We termed the new measure, 'Patient-derived MS Severity Score' (P-MSSS). We also calculated disease duration-adjusted maximum rank (or prevalence) of each PDDS. The data for mean rank (P-MSSS) and maximum rank (prevalence) of all PDDS scores for the first 45 years of disease was graphically represented in the form of P-MSSS Reference Table. Results: The cohort was comprised of 27,918 NARCOMS enrollees, of whom 72.7% were female and 90.1% were White. Mean age of symptom onset was 30.1 (+10.1) years, and age at enrollment was 47.1 (+11.0) years. P-MSSS Reference Table represents frequency-rank of disability for each of the first 45 years since symptom onset. About half of the enrollees described their disability as "limiting daily activities" or worse just one year after disease, and two-thirds - after 5!