Background: Congenital myopathies (CM) are inherited muscle disorders historically classified according to features seen on muscle biopsy and congenital-onset weakness and hypotonia. The aim of our study was to evaluate the benefit of genetic testing, muscle biopsy, NCS/EMG and muscle MRI in obtaining a definite diagnosis for these patients. Methods: A retrospective chart review of all patients diagnosed at a single tertiary-care pediatric hospital over 15 years (2008-2022). REB approval was obtained. Results: Over a period of 15 years, 42 patients with CM were included. All (100%) had genetic testing (i.e. gene panel, WES), 65.9% had muscle biopsy, 67.5% had NCS/EMG and 20% had a muscle MRI. Definite diagnosis was obtained in 38% by genetic testing only, while 42.8% had a diagnosis made by genetic testing supported by the findings of one or more of the other diagnostic tools. Conclusions: Early diagnosis of CM is still essential in congenital myopathies to provide optimal care. Genetic testing is the gold standard for diagnosis, but other diagnostic tools remain valuable in the case of variants of unclear significance.

Background: Infections are hypothesized to trigger certain autoimmune diseases; however, there is a lack of epidemiologic data surrounding pediatric neuro-autoimmune disorders during the COVID-19 pandemic. Our retrospective study assessed the incidence of pre-defined autoimmune disorders at the Children’s Hospital of Eastern Ontario from October 2017-June 2024. Methods: Inpatient/outpatient charts were queried to identify subjects with neuro-autoimmune disorders or type 1 diabetes (T1D) as a non-neurological autoimmune comparison group. Monthly incidences were compared between three COVID-19 pandemic restriction periods: the pre-restrictions (October 2017-March 2020), intra-restrictions (April 2020-June 2022), and post-restrictions periods (July 2022-June 2024). Poisson regression models were fit to the incidence data. To evaluate incidence of specific neuro-autoimmune disorders, crude monthly incidences of six diagnosis categories were compared: ‘Guillain-Barré syndrome’, ‘anti-NMDAR encephalitis’, ‘juvenile dermatomyositis’, ‘multiple sclerosis (MS)’, ‘acute demyelinating disorders’, and ‘other’. Results: Incidence of neuro-autoimmune disorders, but not T1D, decreased during the intra-restrictions period compared to the pre-restrictions period (IRR=0.57, 95% CI: 0.33-0.95, P<0.05). Grouping neuro-autoimmune subjects by diagnosis category showed a trend towards decreased incidence during the intra-restrictions versus pre-restrictions periods for all groups except MS. Conclusions: Incidence of certain neuro-autoimmune disorders, but not MS and T1D, decreased during pandemic restrictions, which may be due to reduced transmission of key infectious triggers.

Background: Ischemic stroke increases the number of glial cells, such as astrocytes, and causes neuronal death, disrupting the neuron-to-glia balance, contributing to neurodegeneration. Treatment with NeuroD-adeno-associated virus (NeuroD1-AVV) may enhance neuronal transdifferentiation and improve motor function, but the optimal administration protocol for the drug has yet to be determined. Methods: Non-human primates (NHPs) underwent middle cerebral occlusion surgery. Fourteen days poststroke, subjects received NeuroD1-AVV according to two distinct protocols: Three high doses and three low doses. Neurological deficits and cognitive performance were measured using the NHP stroke scale and coloured glove shift of set task, respectively. Nine months post-stroke, NHPs were euthanized. Brains were harvested and stained for neuronal (NEUN and MAP2) and glial (GFAP, IBA1) markers using immunofluorescence techniques. Results: Our results indicate that both protocols effectively rebalance the neuron-to-glia cell ratio by decreasing GFAP+ cells in the P1 and P2 NHPS ipsilateral hemispheres. No cognitive performance differences were found across groups; however, P2 had better NHPSS outcomes from months 2 to 9. Conclusions: The findings support both injection protocols in restoring histological balance, with P2 being more effective for motor function rehabilitation. Investigations into neuronal functionality and development levels continue.

Background: Intracranial extension of temporomandibular joint (TMJ) lesions is uncommon and may lead to radiological misinterpretation. This review aimed to identify clinical and radiological features of these lesions and whether radiological misinterpretation contributed to delayed or incorrect intervention. Methods: A comprehensive search of MEDLINE, SCOPUS, and Embase identified 2,256 records. Studies with clinical and imaging details of TMJ lesions extending intracranially were included. Reviews and non-English studies were excluded. After screening, 113 studies involving 132 patients were included. Results: Patients had an average symptom duration of 32 months until diagnosis (47% female, mean age 50±15 years). The most common diagnoses were pigmented villonodular synovitis/tenosynovial giant cell tumor (46%) and synovial chondromatosis (24%). Neurological symptoms were reported in 48% of cases, most frequently hearing loss (35%). Diagnostic accuracy increased from 38% to 62% when both CT and MRI were used. Most lesions were non-enhancing on CT (85%) and MRI (74%), and demonstrated no edema (96%). In one case, a ganglion cyst was misdiagnosed as a cystic brain tumor, leading to neurosurgical resection. Conclusions: TMJ lesions extending intracranially have neurological symptoms in less than half of cases and demonstrate no enhancement or edema. Familiarity with these characteristics is essential to avoiding misdiagnoses and ensuring timely management.

Background: Glial fibrillary acidic protein (GFAP), a brain-specific biomarker, shows promise in differentiating intracerebral hemorrhage (ICH) from acute ischemic stroke (IS) and stroke mimics (SM). A novel point-of-care platform measures GFAP in minutes, yet requires centrifugation to obtain plasma. We aim to determine whether participants recruited in an ongoing prospective biomarker study (during working hours) differ from non-recruited patients. Methods: An exploratory analysis of undifferentiated stroke <24h from onset, where plasma GFAP levels (pg/ml) are measured (i-STAT Alinity) at hospital arrival. Clinical characteristics are compared among recruited and non-recruited patients. Results: Among the first 101 patients recruited, mean (±SD) age (70.8±14.5 years), % females (48%), and median (IQR) NIHSS (9(3-20) were similar to the 270 non-recruited patients (70.3±16.3 years, 51% females, NIHSS 7 (3-17), respectively) in the same time period. Median ASPECTS was slightly lower in recruited patients (10(9-10) vs (10(10-10)) (p=0.03). ICH and SM were more common among non-recruited (52% IS/13% ICH/32% SM) compared to recruited patients (67% IS/5% ICH/29% SM, p=0.002), while large-vessel occlusion was more common among those recruited (44% vs 19%, p=0.001). Conclusions: Clinical characteristics do not differ among recruited vs. non-recruited patients in an ongoing biomarker study, yet sampling bias exists regarding underlying stroke condition, with efforts to mitigate this going forward.

Arcaded houses forming porticoes are characteristic of many medieval European towns in Spain, France, Switzerland, Italy, Germany, Czechia, Poland, Silesia and Ukraine. They are a unique feature at the border between public and private space, used for commerce, crafts and communication. Taking the arcaded houses of Silesia as a case-study, this article focuses on the legal and urban contexts in which they were constructed and removed. Comparison with the broader European context suggests that the form and appearance of arcaded houses in Silesia follow similar patterns and chronology to those that have been observed in much better-studied regions.

Background: The complement component C5 inhibitor, ravulizumab, is approved in Canada for the treatment of adults with AQP4-Ab+ NMOSD. Updated efficacy and safety results from the ongoing CHAMPION-NMOSD (NCT04201262) trial are reported. Methods: Participants received IV-administered, weight-based dosing of ravulizumab, with loading on day 1 and maintenance doses on day 15 and every 8 weeks thereafter. Following a primary treatment period (PTP; up to 2.5 years), patients could enter a long-term extension (LTE). Outcome measures included safety, time to first adjudicated on-trial relapse (OTR), risk reduction, and disability scores. Results: 56/41 patients entered/completed the LTE as of June 14, 2024. Median follow-up was 170.3 weeks (186.6 patient-years). No patients experienced an OTR. 94.8% (55/58 patients) had stable or improved Hauser Ambulation Index scores. 89.7% (52/58 patients) had no clinically important worsening in Expanded Disability Status Scale scores. Treatment-emergent adverse events (98.4%) were predominantly mild and unrelated to ravulizumab. Serious adverse events occurred in 25.9% of patients. Two cases of meningococcal infection occurred during the PTP, and none in the LTE. One unrelated death (cardiovascular) occurred during the LTE. Conclusions: Ravulizumab demonstrated long-term clinical benefit in AQP4-Ab+ NMOSD relapse prevention while maintaining or improving disability measures, with no new safety concerns.

This paper examines the impact of COVID-19 on labour governance and legal struggles faced by overseas Chinese workers. Drawing on migration studies and legal research, it explores the intersections of state, labour and law in the context of transnational mobility and dispute resolution. Through critical analysis of policy directives and court rulings, the paper highlights the Chinese government’s dual challenge in the wake of the pandemic: maintaining the continuity of overseas business operations to safeguard corporate profitability and China’s international image, while also protecting workers’ rights to uphold social stability. The findings reveal that overseas workers were at times overlooked in central government policy guidelines, despite facing unique legal, spatial and logistical challenges owing to the transnational and trans-jurisdictional nature of their employment. This lack of tailored policy attention has resulted in inconsistencies and disparities in how domestic courts adjudicate their legal claims. Gaps in overseas labour governance during times of crisis underscore the need for clearer legal stipulations and more inclusive judicial protections to address the complexities of transnational labour disputes under “Global China.”

Background: Occipital nerve stimulation (ONS) is a promising therapy for craniofacial pain syndromes refractory to conventional treatments. This study evaluates the long-term efficacy and safety of ONS over a 15-year period at Hôpital Enfant-Jésus, CHU de Québec, based on patient-reported outcomes and complication rates. Methods: A retrospective cohort analysis was conducted on patients with occipital neuralgia, cluster headaches, migraines, and other craniofacial pain syndromes who underwent ONS implantation. Pain intensity, quality of life, and patient-reported improvements were assessed at baseline, one year, and the latest follow-up. Trial stimulation was performed before permanent implantation. Results: Among 45 patients (mean age: 53.7 years), diagnoses included occipital neuralgia, migraines, cluster headaches, cervicogenic pain, and other pain syndromes. At follow-up (mean: 4.72 ± 1.02 years), NPRS scores decreased by 3.8 ± 1.7 points, BPI showed a 45% ± 18% reduction, and SF-36 revealed improvements in physical (20% ± 25%) and social functioning (30% ± 22%). Adverse events occurred in 27% of patients, with surgical revisions required in 14%. Conclusions: ONS provides significant, long-term pain relief and quality-of-life improvements in refractory craniofacial pain, particularly for occipital neuralgia and cluster headaches. While complications remain notable, advancements in techniques and technology may improve outcomes, supporting ONS in tailored pain management protocols.

Background: Accurate aquaporin-4 (AQP4) and Myelin Oligodendrocyte Glycoprotein (MOG) assays are essential for effectively diagnosing neuromyelitis Optica spectrum disorder and MOG antibody-associated disease. The Live Cell-Based Assay (L-CBA) is the gold standard laboratory test for detecting these antibodies. We studied the profiles of these antibodies, in samples of patients with relevant neurological conditions. Methods: Between January 2021 and December 2024, a total of 6673 samples of serum and/ or CSF were tested at BC Neuroimmunology Lab, Vancouver. We performed in-house L-CBA for the AQP4 and MOG Abs identification. We analyzed the demographics and characteristics of the positive Abs results. Results: We identified 7.8% positive results for anti-MOG and 2.7% for anti-AQP4 antibodies. Both antibodies were more frequent in females (AQP4: 76.9%, MOG: 65.1%). The average age of patients was 49.2 ± 18.8 years, ranging from 9 to 88 years for AQP4 antibodies, and 40.9 ± 19.5 years, ranging from 10 months to 89 years for MOG antibodies. Conclusions: Both anti-MOG and anti-AQP4 Abs are prevalent in females. Moreover, anti-MOG Abs are present across a wider age range from infancy to the elderly, and anti-AQP4 Abs are typically found in later ages, between 10 and 90 years.

Background: Leadership drives innovation, patient care and resident education in neurosurgery. This study aims to quantitatively analyze the demographic and professional characteristics of leaders in Canadian academic neurosurgery. Methods: Leaders in remunerated positions, such as department chairs and program directors, from the 14 Canadian Royal College of Physicians and Surgeons-accredited neurosurgery programs were identified using publicly available online resources, chain-referral sampling, and personal communications. Demographic and professional data were collected and analyzed using frequency analyses and exploratory chi-square tests. Results: Thirty-four neurosurgical leaders were identified, predominantly in Québec (29.4%) and Ontario (26.5%). Pediatrics (26.5%) was the most common subspecialty. Over half of leaders held positions in the institution where they trained (52.9%). Among non-Quebec programs, male neurosurgeons were less likely than females to hold leadership positions (p = 0.040, OR = 0.344, 95% CI 0.12-0.99), although males still predominated (18 males vs. 6 females). In Quebec, no gender association was found (p = 0.652). Leaders averaged 76.4 (±81.49) publications and an h-index of 19.71 (±15.12) with nearly two-thirds holding advanced degrees. Conclusions: This study establishes the current landscape of leaders in Canadian academic neurosurgery. Further research is needed to explore career trajectory and barriers to leadership in the field.

Background: This retrospective cohort study investigates radiographic factors linked to the success of Endoscopic Third Ventriculostomy (ETV) for hydrocephalus. Methods: We examined 48 patients who underwent ETV between August 2011 and March 2023. Radiographic factors analyzed included the basal skull angle, modified basal skull angle, interpeduncular cistern diameter, prepontine diameter, and approach angle to the third ventricle floor. Statistical analysis was performed using R studio. Results: The cohort had a median age of 41 years, with 22 females. Pathologies included aqueductal stenosis (21 cases), tectal tumors (7), and IVH (5). The mean ETV Success Score (ETVSS) was 76.7. Of the 21 failures, 16 required a shunt. A strong correlation was found between ETVSS and procedure success (p<0.001). Modified basal skull angle (p=0.028), interpeduncular cistern diameter (p<0.001), and approach angle (p<0.001) were all associated with ETV success. Decision tree analysis showed that the inclusion of approach angle to ETVSS improved sensitivity and specificity, reaching 1.0 for both. Conclusions: In conclusion, the study highlights that radiographic factors, particularly the modified basal skull angle, interpeduncular cistern diameter, and approach angle, are key predictors of ETV success. This information can assist neurosurgeons in planning cases more effectively.

Background: Hyperosmotic hyperglycemic nonketotic state (HHS) is associated with myriad neurological complications such as seizures. Methods: We report a case presenting with visual hallucinations due to occipital lobe epilepsy. Results: A 67-year old woman with chronic hypertension, hyperlipidemia and diabetes mellitus non-compliant to medication presented with a 10-day history of recurrent visual phenomena in the left visual field. She described stationery multi-coloured flashing lights which decreased in intensity, brightness and size after 3 minutes. She was alert and conscious during attacks. There was no limb jerking. Neurological examination was normal with no visual field defect. Capillary glucose was 28.1 mmol/L, Hba1c 9% and B-hydroxybutyrate < 0.1. She was treated with actrapid 8 units, glipizide 5 mg BD and empagliflozin 12.5 mg OM. Interictal electroencephalogram was normal with no epileptiform activity. Brain magnetic resonance imaging revealed restricted diffusion in the right occipital cortex with corresponding cortical thickening and increased FLAIR signal with subtle hypodensity on GRE sequence. Her visual symptoms improved dramatically with hydration and diabetic control. She was treated with a short course of keppra. Conclusions: Visual hallucinations are an uncommon but well recognised and fully reversible complication of HHS. Clinicians should not forget HHS in the workup of occipital lobe epilepsy.

Background: Stroke therapies remain an area of ongoing research. Gene therapies offer a novel approach to repair tissue damage, particularly NeuroD1-mediated astrocyte-to-neuron conversion, which regenerates functional neurons after ischemic injury. Here, we applied NeuroD1 therapy in a non-human primates (NHPs) stroke model to evaluate its effects on corticospinal tract (CST) recovery and motor performance. Methods: Eight NHPs underwent middle cerebral artery occlusion (MCAO). Fourteen days later, six animals received intracranial NeuroD1 treatment (three high-dose, three low-dose), while two received a control solution. Neurological and functional performance were assessed daily. MRI scans were performed at baseline and at 7, 30, 90, 120, and 240 days post-MCAO, with the bilateral CST reconstructed at each time point. All procedures followed Canadian Council of Animal Care guidelines and were approved by Queen’s University’s Animal Use Subcommittee. Results: We found that NHPs receiving the control solution exhibited poorer motor recovery and minimal CST reconstruction. In contrast, those treated with a low dose of NeuroD1 demonstrated motor and functional recovery along with CST reconstruction. Notably, animals receiving the higher dose showed the most significant overall recovery including a greater CST integrity. Conclusions: NeuroD1 treatment promotes white matter tract restoration and facilitates motor recovery following stroke.

Magnetic reconnection, a fundamental plasma process, is pivotal in understanding energy conversion and particle acceleration in astrophysical systems. While extensively studied in two-dimensional (2-D) configurations, the dynamics of reconnection in three-dimensional (3-D) systems remains under-explored. In this work, we extend the classical tearing mode instability to three dimensions by introducing a modulation along the otherwise uniform direction in a 2-D equilibrium, given by $g(y)$, mimicking a flux-tube-like configuration. We perform linear stability analysis (both analytically and numerically) and direct numerical simulations to investigate the effects of three-dimensionality. Remarkably, we find that a tearing-like instability arises in three dimensions as well, even without the presence of guide fields. Further, our findings reveal that the 3-D tearing instability exhibits reduced growth rates compared with two dimensions by a factor of $\int g(y)^{1/2} {\rm d}y\,/\int {\rm d}y$, with the dispersion relation maintaining similar scaling characteristics. We show that the modulation introduces spatially varying resistive layer properties, which influence the reconnection dynamics.