Generalized myasthenia gravis (gMG) is a chronic neuromuscular disease that causes muscle weakness and fatigue, severely impairing quality of life. Efgartigimod is a novel drug that is recently approved for treatment of acetylcholine receptor antibody-positive (AChR-Ab+) gMG patients in Canada. In clinical practice, it is expected to be used in AChR-Ab+ gMG patients who continue to experience symptoms despite conventional therapy and primarily replace chronic immunoglobulins.

A Markov model was developed to estimate costs and benefits (measured as quality-adjusted life years [QALYs]) of efgartigimod and chronic immunoglobulins for AChR-Ab+ gMG patients. The analysis was conducted from the perspective of the Canadian publicly funded healthcare system over a lifetime horizon. The model comprised six health states based on Myasthenia Gravis Activities of Daily Living (MG-ADL) scores: MG-ADL < 5, MG-ADL 5–7, MG-ADL 8–9, MG-ADL ≥ 10, myasthenic crisis or death. Health state transition probabilities were estimated from the ADAPT and ADAPT+ studies, plus a network meta-analysis that compared efgartigimod against chronic immunoglobulins. The MyRealWorld MG study informed utility values. Modeled costs included treatment and administration, disease monitoring, complications from chronic corticosteroid use, exacerbation/crisis management, adverse events and end-of-life care.

Over a lifetime horizon, efgartigimod and chronic immunoglobulins were predicted to have total discounted QALYs of 16.80 and 13.35 and total discounted costs of $1,913,294 and $2,170,315, respectively. Efgartigimod dominated chronic immunoglobulins with incremental QALYs of 3.45 and cost savings of $257,020.

Efgartigimod provides greater benefit in terms of lower costs than chronic immunoglobulins for AChR-Ab+ gMG patients in Canada.

White matter hyperintensities (WMH) on fluid-attenuated inversion recovery MRI sequence are regions where fluid from supplying vessels leaks into brain tissue. Some studies have demonstrated an association between WMH and cognitive decline. Given the common WMH risk factors in our local population, the aim of this study is to examine the relationship of overall and regional WMH with cognition in Hamilton, Canada.

Adults presenting to Hamilton General Hospital in 2020 with a head MRI and cognitive assessment within 6 months of the MRI were included in our cross-sectional study. MRIs were reviewed, assigning a periventricular (PV), a subcortical (SC) and an overall severity score to each based on the Fazekas scale, ranging from 0 to 3. Montreal Cognitive Assessment (MoCA) scores were used as a measure of cognitive function. Patients with confounding diagnoses were excluded. Multiple regression analyses were conducted between WMH and cognitive scores, adjusting for hypertension, diabetes and smoking.

Multiple regression models revealed R2 values of 0.097, 0.050 and 0.036 for overall, PV and SC WMH with MoCA, respectively. There were negative associations between overall Fazekas scores and MoCA (B = −2.11, p < 0.001), PV scores and MoCA (B = −1.46, p < 0.001) and SC scores and MoCA (B = −1.21, p = 0.002).

The association between MRI WMH and cognition supports prognostic use for cognitive decline to limit/delay deterioration. Specifically, stronger PV associations prompt research and perhaps development of revised scales prioritizing PV changes. Implementing this into the field of radiology whereby WMH severity and location assessment becomes a standard within brain MRI reports could improve patient outcomes.

To evaluate the impact of discharge destination on long-term functional outcomes in patients with aneurysmal subarachnoid hemorrhage (aSAH) and its association with achieving functional independence.

This retrospective cohort study included patients with aSAH treated endovascularly at a tertiary neurovascular center between October 2019 and November 2024. Patients who died before hospital discharge were excluded. Group comparisons were performed using one-way analysis of variance, Kruskal–Wallis, paired-sample t-tests and Pearson’s χ2. Multivariable logistic regression was used to assess the association between discharge site and functional independence achievement, adjusting for age, sex, World Federation of Neurosurgical Societies (WFNS) grade and modified Rankin Scale (mRS) score at discharge.

A total of 213 patients (mean age 55.9 ± 13.7 years; 77% female) were included. Hypertension (57.7%) and previous smoking (45.1%) were the most common risk factors. Significant differences in age, sex, WFNS grade and modified Fisher Scale scores were observed across discharge groups. While patients discharged home had better overall outcomes, those with poor WFNS grades showed greater reductions in mRS at 6 and 12 months when discharged to an acute rehabilitation center. Regression analysis demonstrated that discharge to an acute rehabilitation center was independently associated with higher odds of functional independence, while discharge to a primary care hospital decreased these odds.

Discharge to a primary care hospital after aSAH was associated with worse long-term outcomes. In contrast, early transfer to an acute rehabilitation center significantly improved functional independence, particularly among patients with poor baseline neurological status.

To quantify optic nerve hypoplasia/septo-optic-pituitary dysplasia (ONH/SOD) all-cause mortality rate and risk of death in Manitoba, Canada.

A retrospective population-based study with a case–control design was undertaken using the Manitoba Population Research Data Repository. Cases were 124 ONH/SOD patients diagnosed during 1990–2019, matched to 620 unrelated population-based controls on year of birth, sex and area of residence. Both cases and controls were followed until March 31, 2022, or until they moved out of the province or died. Crude mortality rate was estimated. Cox proportional hazards models were used to test for differences in all-cause mortality between cases and controls. Hazard ratios (HR) with 95% confidence intervals (CIs) were estimated.

Six of 124 (4.8%) cases with ONH/SOD and 8 of 620 (1.3%) controls died during the study’s follow-up period. The median (25th–75th percentiles) age of death of ONH/SOD patients was 4.6 (2.7–9.1) years. The median duration of follow-up was 12.0 years for the cases and 11.4 years for the controls. The crude mortality rate (95% CIs) was 3.7 (1.7–8.3) per 1000 person-years in patients with ONH/SOD and 1.0 (0.5–2.1) per 1000 person-years in unrelated matched controls. All-cause mortality was significantly higher in ONH/SOD patients compared to unrelated controls (HR = 3.7, 95% CI = 1.3–10.5).

Patients with ONH/SOD have a higher risk of death compared to unrelated controls. Healthcare professionals should be familiar with the morbidities and comorbidities associated with ONH/SOD and the complications that may lead to their demise, since they can be managed to reduce the mortality risk.

Giant cell arteritis (GCA), a form of large vessel vasculitis, carries the risk of irreversible loss of vision due to involvement of arteries supplying optic nerve, retina and choroid. As such, timely diagnosis of GCA is crucial. The study examines the presenting symptoms among suspected GCA patients with both positive and negative biopsy results and the association of computed tomography angiography (CTA) findings of the suspected GCA patients with superficial temporal artery (STA) biopsy results.

A retrospective study involving 22 patients who underwent STA biopsy and CTA at Kingston Health Science Center. The CTA images of the patients were analyzed for the following imaging signs: blurred STA wall, perivascular enhancement, presence of stenosis or occlusion of STA and calcification. The association between imaging signs and STA biopsy results was assessed using odds ratios (ORs). Additionally, the final alternative diagnosis for the patients with negative biopsy results was also investigated.

There was a strong association between the presence of two CTA imaging findings : blurred STA wall and perivascular enhancement, and positive STA biopsy results (OR: 29, p: 0.001). Suspected GCA patients with negative STA biopsy results had no single unifying alternative diagnosis.

This study provides evidence for the potential role of CTA in the initial assessment of suspected GCA patients.

Some patients with large vessel occlusion (LVO) are first evaluated at primary stroke centers (PSCs) before transfer to a comprehensive stroke center (CSC) for endovascular thrombectomy (EVT). A subgroup of these patients experiences rapid infarct growth, also known as “imaging decay,” during transfer, limiting the benefit from intervention. We evaluated the incidence, predictors and outcomes of imaging decay in this subgroup.

The present study was an analysis of all patients with anterior circulation LVO at PSCs in Northern Alberta and transferred to the CSC in the University of Alberta Hospital in Edmonton for EVT. The Alberta Stroke Program Early CT Score (ASPECTS) decay was defined as ≥ 2 ASPECTS points decrement at CSC compared to PSC. The primary outcome was 90-day home time.

182 patients were included. Median time between baseline and follow-up CTs was 250.5 (IQR 163–324.25) minutes. Out of the 182 patients, 66 patients (36%) had ASPECTS decay, and 32 of 66 patients (48%) underwent EVT. Poor collateral score was strongly associated with ASPECTS decay (OR = 0.35, [0.21–0.59], p < 0.001). Patients with ASPECTS decay have a significantly lower 90-day home time (β = –0.32, [–4.6 to –36.4], P < 0.001) and higher risk of 90-day mortality (OR = 4.9, [2.4–10.0], P < 0.001) and in-hospital death (OR = 3.8, [1.2–12.3], P = 0.03).

For patients with LVO transferred for thrombectomy, a third of our patients developed ASPECTS decay. Collateral blood flow was the main determinant of ASPECTS decay during interfacility transfers. Decay is strongly associated with poor functional outcomes.

Patients with stroke or transient ischemic attack (TIA) are at high early risk of mortality and morbidity. Current risk prediction tools focus on patients after hospital discharge but not on those surviving to outpatient follow-up. We examined whether demographic and medical history data could predict 1-year stroke recurrence and mortality, among those discharged alive and event-free for 90 days after stroke and 1 day after TIA.

Data were obtained from the Ontario Stroke Registry (13,848 stroke and 13,059 TIA patients) and linked to administrative databases. Two-thirds of each cohort were used for model derivation and one-third for validation. Multivariable regression models were used to predict stroke recurrence and all-cause mortality.

There were 238 (2.71%) recurrent strokes in the ischemic stroke and 298 (3.44%) in the TIA cohorts at one year. Increasing age and previous stroke/TIA were associated with an increased risk of recurrent stroke in both cohorts. A higher modified Rankin Scale and diabetes were associated with an increased risk of recurrent stroke in the stroke cohort and heart failure, smoking and discharge location in the TIA cohort. Time-dependent areas under the curve were modest, 0.59 (0.54–0.64) and 0.59 (0.55–0.64) for the stroke and TIA validation cohorts, respectively. C-statistics from derivation and validation cohorts for mortality ranged from 0.74–0.78.

The predictive accuracy of the models was quite low after accounting for several risk factors. Additional risk factors associated with stroke recurrence for people seen in outpatient stroke clinics, and innovative approaches to individualized secondary prevention are needed.

Achieving a first pass recanalization (FPR) improves clinical outcomes in patients with basilar artery strokes, but its association with initial infarct burden is unknown. We aimed to study the benefits of FPR for basilar artery strokes by initial infarct burden using the Posterior Circulation Alberta Stroke Program Early CT score (pc-ASPECTS).

We retrospectively analyzed the prospective multicentric Endovascular Treatment of Ischemic Stroke registry and included 194 patients diagnosed with an acute basilar artery occlusion who were treated with thrombectomy. Our primary outcome was a modified Rankin Scale (mRS) of 0–3 at 90 days, and our secondary outcomes were an mRS of 4–6 and mortality. We compared the 90-day clinical outcomes of achieving an FPR versus multiple thrombectomy passes based on patients’ initial infarct size on pretreatment MRI: small (pc-ASPECTS = 9–10), medium (pc-ASPECTS = 6–8) and large (pc-ASPECTS <6).

Patients with a medium or large infarct size had significantly better outcomes (mRS 0–3 at 3 months) if FPR was achieved than if multiple passes were required (RR = 1.61, 95% CI: 1.16, 2.24; p-value = 0.005; and RR = 3.41, 95% CI: 1.54–7.57; p-value = 0.003, respectively). No similar difference was seen among patients with small infarcts. Achieving an FPR was also associated with a significantly lower mortality risk among patients with a moderate infarct size (RR = 0.36, 95% CI: 0.17–0.79; p-value = 0.010) but not with those with small or large infarcts.

Achieving an FPR significantly improves clinical outcomes in acute stroke patients with basilar artery occlusions undergoing thrombectomy when their infarcts are medium or large. Ongoing research to develop surgical techniques to achieve FPR is crucial to improving patients’ prognoses.

Temporal arteritis (TA) is the most common vasculitis over age 50. Untreated, many patients will suffer blindness or stroke. Gold standard diagnosis is achieved by temporal artery biopsy. The aim of this study was to investigate the relevance of small vessel inflammation.

Our dataset was comprised of 72 temporal artery biopsies subjected to a blinded uniform re-examination paired with clinical data including demographics, history, physical examination and laboratory findings. Documented pathology variables included the presence or absence of TA, angiitis of vasa vasorum (AVV) and inflammation of small peri-adventitial vessels (small vessel vasculitis, SVV).

Clinical and pathological variables were subjected to multivariate analysis. In brief, 25% of cases were identified as TA, 20% as isolated AVV, 7% as isolated SVV and 5% as mixed isolated AVV/SVV, while 43% had no inflammation (NI). All cases of TA were accompanied by small vessel inflammation: 95% exhibited AVV with or without SVV, and 5% exhibited SVV alone, demonstrating a strong association between TA and small vessel inflammation. Of the 24 cases with isolated AVV/SVV, 26% received a clinical diagnosis of TA within one year in comparison to 13% of cases that had NI. Furthermore, isolated AVV/SVV was identified in 25% of patients with a high clinical probability for TA, 60% of whom acquired a diagnosis of TA on clinical grounds within one year of follow-up.

Our findings suggest that isolated AVV/SVV identifies a subgroup of patients with a higher risk of harboring or developing TA.