Background: Hemifacial spasm (HFS) is a disorder of unilateral facial myoclonus that can be psychosocially debilitating. It is generally caused by microvascular compression of the facial nerve and is amenable to potentially curative surgery. Unfortunately, many patients receive delayed surgical referrals and are instead managed with injections of botulinum toxin to varying benefit. The reason behind late referrals is unclear, but one factor may be misleading diagnostic information; specifically, MRI reports that indicate incorrect or absent neurovascular conflict. The goal of this study was to explore the association between MRI reports and operative findings in HFS patients, with the hopes of identifying opportunities for improved diagnosis and treatment. Methods: We performed a single-center, retrospective chart review of 30 consecutive patients who underwent surgery for HFS between January 2019 and January 2020. Descriptive data were extracted from pre-operative MRI reports and operative notes, then coded for statistical analysis. Results: Pre-operative MRIs were reported by radiologists from 6 provinces. Across all MRIs (n = 45), a positive finding was only identified in 51.1%. All 30 patients had clear neurovascular conflict noted in surgery. Conclusions: There is a need for improved diagnostic accuracy in HFS, with the goal of expediting surgical referrals and possible cure.

Background: Late-onset Pompe disease (LOPD) is caused by a deficiency of acid α-glucosidase (GAA), leading to progressive muscle and respiratory decline. Cipaglucosidase alfa (cipa), a recombinant human GAA naturally enriched with bis-mannose-6-phosphate, exhibits improved muscle uptake but is limited by inactivation at near-neutral blood pH. Miglustat (mig), an enzyme stabiliser, binds competitively and reversibly to cipa, enhancing its stability and activity. Methods: In dose-finding studies, Gaa-/- mice were treated with cipa (20 mg/kg) +/- mig (10 mg/kg; equivalent human dose ~260 mg). Clinical study methodologies have been published (Schoser et al. Lancet Neurol 2021:20;1027–37; Schoser et al. J Neurol 2024:271;2810–23). Results: In Gaa-/- mice, cipa+mig improved muscle glycogen reduction more than cipa alone and grip strength to levels approaching wild-type mice. LOPD patients (n=11) treated with cipa alone showed dose-dependent decreases in hexose tetrasaccharide (Hex4) levels by ~15% from baseline, decreasing another ~10% with added mig (260 mg). In a head-to-head study, cipa+mig had a similar safety profile to alglucosidase alfa. Among 151 patients (three trials), mig-related adverse events occurred in 21 (13.9%), none serious. Conclusions: Mig stabilised cipa in circulation, improving cipa exposure, further reducing Hex4 levels and was well tolerated in clinical studies in patients with LOPD. Sponsored by Amicus Therapeutics, Inc.

Over the years, cultural and linguistic diversity in schools across Europe has significantly increased due to migration and refugee flows. In response, international organizations, such as the Council of Europe and the European Commission, advocate intercultural education as both an educational strategy and a social policy tool to foster inclusion, address inequality, and build cohesive societies. This study contributes to the intercultural education literature by addressing an underexplored area: the process of translating intercultural policies into school practices. Using Street-Level Bureaucracy theory and qualitative research in Trento, Italy, it highlights the mechanisms and challenges shaping teachers’ practices and the extent of the policy–practice gap. Furthermore, the research also contributes to the Street-Level Bureaucracy theory. It shows that teachers can act as innovators in the policy implementation process. By engaging civil society members, notably students and members of migrant communities, as co-implementers, teachers reshape policy ecosystems through participatory and bottom-up approaches.

Five species of monorchiids are known from fishes of the family Gerreidae, of which one is from Australian waters. Here, we report it and two new monorchiids from three species of Gerres Quoy & Gaimard, 1824 from off Lizard Island, northern Great Barrier Reef, and Moreton Bay in south-eastern Queensland: Gerres oyena (Forsskål), Gerres oblongus Cuvier and Gerres subfasciatus Cuvier. One of the new species, found only in G. oblongus at Lizard Island, conforms most closely to the concept of Proctotrema Odhner, 1911. However, it differs from species of Proctotrema in oral sucker shape and location of intestinal bifurcation and termination. It is phylogenetically distinct from two sequenced species of Proctotrema; thus, we propose Obscuromonorchis ranae n. g., n. sp. The second new species infects all three gerreids, occurs at both Lizard Island and Moreton Bay, and is morphologically most similar to the concept of Monorchicestrahelmins Yamaguti, 1971. However, the combination of the length of the caeca, size of the testis and post-testicular region, and the form of spination in the genital atrium presents a clear genus-level distinction that warrants proposal of a new genus. There are no molecular data for the three recognised species of Monorchicestrahelmins. We propose Argenticola shuyinae n. g., n. sp. for this species. New specimens of Gerricola queenslandensis Wee, Cutmore & Cribb, 2021 were collected from off Lizard Island and Moreton Bay. The three species form a well-supported clade but with internal branch lengths and topology consistent with genus-level differentiation.

Background: Infundibular lesions are rare entities with variable clinical manifestations. Their detection during workup for endocrinologic abnormalities represents a decision-making challenge. We present a patient with ACTH-dependent hypercortisolemia found to have a stalk lesion, which was treated surgically. Methods: Information was gathered from electronic medical records. Results: A 57F underwent workup for Cushing’s syndrome, demonstrating evidence of ACTH-dependent hypercortisolemia. Imaging demonstrated a 4.7mm infundibular nodule. We elected to proceed with endoscopic endonasal approach for resection of the infundibular lesion with goal of biochemical cure. A satisfactory technical and radiographic resection of the infundibular lesion was achieved. However, the patient’s hypercortisolemia failed to resolve. Histopathologic analysis identified the lesion as a granulocytoma. Inferior petrosal sinus sampling further demonstrated evidence of ACTH-dependent central hypercortisolemia. She then underwent bilateral adrenalectomy for management of her persistent hypercortisolemia. Conclusions: This demonstrates a complex clinical picture in which our patient presented with biochemical results suggesting central ACTH-dependent hypercortisolemia with no identifiable glandular lesion. The presence of an infundibular lesion led to surgical intervention which unfortunately did not result in biochemical cure despite adequate technical results. The authors believe this case illustrates a challenging clinical conundrum which emphasizes the uncertainty that should be associated with management of stalk lesions.

Background: Transcranial doppler ultrasound (TCD) in a pediatric neurocritical setting can determine cerebral hemodynamics by assessing the blood flow velocity in main cerebral arteries. In large vessel occlusions (LVO) that require endovascular thrombectomy (EVT), TCD can monitor recanalization and arterial re-occlusion. We describe one case in a previously healthy 13-year-old girl with a right M1 middle cerebral artery occlusion. Methods: Analysis was done via a retrospective case review. Results: Our patient underwent a successful endovascular thrombectomy (EVT) six hours after symptom onset. Follow up TCDs done at 4, 8, and 24 hours showed stable peak systolic velocities (PSV) on the narrowing of right M1 ranging from 245 to 270 cm/s with stable pre-stenotic PSV around 110 cm/s, indicating focal and stable narrowing of M1 without reocclusion. No high transient signals (HITS) were identified on sub 10 minute TCDs. An urgent echocardiogram revealed a bicuspid aortic valve with vegetations, with later confirmation of infective endocarditis. The patient made an impressive recovery with only mild deficits. Conclusions: TCD can be an effective tool in a pediatric neurocritical setting in guiding initial recanalization after EVT and monitoring for arterial re-occlusion, HITS and hyperperfusion. TCD monitoring also decreases the amount of radiation exposure via CTA.

Background: Spinal cord injury (SCI) is a central nervous system injury that often leads to motor, sensory and autonomic dysfunction. Non-invasive trans-spinal electrical stimulation (ts-ES) has been shown to activate neural networks below the injury and improve motor function recovery after SCI. The objective of this study was to compare changes in motor and autonomic function attributable to ts-ES in individuals with incomplete SCI after 4 weeks of personalized training. Methods: Participants received 4 weeks of treadmill training with personalized step-cycle based PNS and FES with and without non-invasive lumbar ts-ES. Clinical outcome measures of motor function (2-minute walk test, Berg Balance and modified SCIM-Mobility) and metabolic analysis (heart rate and rate of oxygen consumption (VO2 sub-max)) were assessed before and after training. Non-invasive electromyography (EMG) and kinematic data assessed motor function. Results: Based on participant feedback and data, ts-ES with PNS/FES during training was tolerable, improved leg movement and facilitated muscle activity in knee extensors with 10-25% increased RMS amplitude of pre-training EMG activity during both forward and backward walking. Moreover, ts-ES tended to increase HR and VO2 sub-max within one session. Conclusions: Personalized rehabilitation strategies combining ts-ES with traditional physiotherapy exercises and locomotor training have the potential to improve recovery after SCI.

Background: CIDP is a rare immune-mediated demyelinating neuropathy that has significant phenotypic variability. A unifying immunopathological mechanism remains elusive, likely due to etiological heterogeneity among the variant presentations. This is best exemplified by the identification of nodal/paranodal antibodies, such as neurofascin 155, in a subgroup of CIDP patients who present with a distinct phenotype. Methods: We present the case of a 39-year-old male who presented with a 2-year history of progressive stocking-glove sensory loss and sensory ataxia. Electrodiagnostics confirmed an acquired demyelinating neuropathy, with serum anti-NF155 IgG4. His case was refractory to standard immunomodulatory therapy, including adequate trials of IVIG, steroids, azathioprine, and rituximab. He also had a non-therapeutic trial of PLEX, methotrexate, and tacrolimus. Results: After cessation of all immunomodulatory therapy for 2 years, he had spontaneous remission of his CIDP and near-complete resolution of electrodiagnostic/clinical abnormalities. Conclusions: This case provides insights into the natural history of NF155 “paranodalopathy” and highlights a unique case of supra-refractory CIDP which underwent spontaneous remission with near-complete resolution. Delayed effect from rituximab was posited as a contributor, however, the patient had no clinical or electrophysiological improvement 20-months after initiation of anti-CD20 therapy. Current data suggests the majority of CIDP patients respond to rituximab within 6-12 months.

Background: Giant olfactory groove meningiomas (OGMs), though rare, pose challenges due to their size. These slow-growing tumors often remain asymptomatic until exceeding 6 cm in diameter. While surgery has advanced, understanding long-term outcomes remains crucial. Methods: This retrospective study at a major medical center included all patients with giant OGMs (>6 cm) undergoing resection from 2000-2022.Data on visual status, recurrence, and functional status were collected. Multivariable logistic regression identified predictors of recurrence and functional outcome. Results: Thirty-two patients met the inclusion criteria for this study, with a mean age of 55.8years.The mean follow-up period was 62months. The majority of giant OGMs were classified as WHO grade 1(84.4%).Postoperatively, 19patients demonstrated improvement in visual acuity and visual field deficits.Radiological recurrence was observed in nine patients(28.1%) at a mean follow-up of 56months, with only three requiring reoperations for tumor resection.One patient developed a brain abscess, necessitating reoperation.Multivariable analysis identified patient age, Simpson grade of excision, and WHO grade as significant predictors of recurrence rate. Conclusions: This study demonstrates that surgery can improve visual deficits and functional outcomes. Postoperative outcomes were strongly predicted by age, resection extent, and histological grade. Developing a new predictive scale based on these parameters appears to strongly predict the Giant OGMs Long-Term outcome.

Coral reefs have been rapidly deteriorating, worldwide, due to global warming, ocean acidification, bleaching, diseases, and various local anthropogenic stressors, such as coastal development, habitat destruction, overfishing and eutrophication, all of which have significantly impacted the metabolic functions of corals and other marine organisms. Global warming has been identified as the main culprit in the decline of coral reefs. In response, we assessed the metabolic responses of one of the most iconic Caribbean corals to elevated temperatures. Accordingly, the proteomic profile of Acropora palmata was investigated during the cool dry and hot wet seasons of 2014 and 2015 in Puerto Rico using a combination of two-dimensional gel electrophoresis (2D-GE) and mass spectrometry. The study revealed that the average number of differentially abundant proteoforms between seasons was 527 in the inner-shelf reef at Enrique and 1,115 in the mid-shelf reef at San Cristobal, both located on the insular shelf of southwestern Puerto Rico. Our results show significant changes in A. palmata’s proteome, inducing alterations in key metabolic, enzymatic, translational, and apoptotic processes, between the cool dry and hot wet seasons. Quantitative real-time reverse transcription PCR (qRT-PCR) was used to validate the variation in the expression of five candidate stress-related genes under different seasonal temperatures. The findings highlight key proteoforms whose abundance varied with temperature, offering insight into A. palmata’s metabolic capacity to acclimate and respond to seasonal temperature fluctuations.

Background: Seronegative autoimmune encephalitis (SAE) in children is associated with cognitive deficits, particularly executive dysfunction. However, the relationship between cognitive impairment, disease severity, and lesion burden remains unclear. Identifying these associations could improve patient management and outcomes. This study characterizes neuropsychological symptoms in pediatric SAE and compares patients with and without formal neuropsychological assessments to determine factors influencing cognitive impairment. Methods: A retrospective review was conducted on 155 pediatric autoimmune encephalitis cases, including 80 with SAE. Eleven had neuropsychological evaluations. Statistical analyses assessed differences in age, disease severity, lesion characteristics, hospitalization, and treatment needs. Results: Executive dysfunction was present in 75% of SAE cases. Patients with neuropsychological evaluations were older (median: 8 vs. 3 years, p = 0.0115) and had more severe encephalitis at admission (p = 0.0391) and one year later (p = 0.0011). Lesion burden did not differ (p > 0.05), but patients with assessments had longer hospitalizations and required more intensive treatments (p < 0.005). Conclusions: Executive dysfunction in pediatric SAE is linked to disease severity rather than lesion burden. Systematic neuropsychological assessments should be integrated into patient care. Deeper phenotyping of cognitive profiles and identifying risk factors for poor prognosis will help personalize care in order to improve outcomes.

Background: Referrals to the Stroke prevention clinic with incomplete preliminary investigations decrease clinic capacity due to additional workload and the need for follow-ups. We aimed to improve the efficacy of the initial visit by increasing the completion rate of vascular imaging. Methods: Pre-post quasi-experimental study with three phases: Phase 1: Surveillance; Phase 2: Stakeholder feedback-informed intervention development (physicians and clinic staff); and Phase 3: Implementation. Interventions included a new referral order within the provincial EMR; a specific physician triage form listing required investigations (brain imaging, vascular imaging, cardiac tracing); and a nurse-led pre-visit via telephone. The primary outcome measure was the completion of vascular imaging - assessed with multivariable logistic regression Results: The study’s inclusion criteria were met by 383 patients, mean age of 67.6±13.2 years; 49% were female, 62.5% were diagnosed with vascular events. An increase in vascular imaging before the initial visit was found in Phase 3 (139/184, 75.5%) compared to Phase 1 (121/198, 61.1%, Odds ratio 1.96 95% CI 1.3-3.1; p=0.003). Fewer follow-up visits were required in Phase 3 (22.8%) compared to Phase 1 (31.8%, p=0.049). Conclusions: A uniform referral process, a standard triage process, and a nurse-led pre-visit may improve the completion of essential investigations before the patient visit.

Background: Accessible ambulatory neurology care can reduce the need for inpatient evaluation. Aligning patient demand (service requests) with provider and space resources can optimize ambulatory clinic flow. In response to increasing referral volumes and wait times for neurologist access, a quality improvement initiative was undertaken to address demand. Methods: Process mapping and root cause analysis demonstrated access challenges and referral processing errors. Audit of 968 accepted referrals revealed variation in triage processes and decisions for referral questions. Neurologists defined inclusion criteria to specialty programs, based on referral questions. Referral management transitioned to a central intake model, reducing intra- and inter-clinic triage variability. Guidelines were established to prevent triage duplication and standardize appointment management. The primary outcome was accepted referrals per month. Secondary outcomes were referral rejection rate and neurology wait times. Results: Significantly more referrals were received per month post intervention (987 vs. 859, p<0.000). The number of accepted referrals did not change (p=0.147). Referral rejection rate increased from 21% to 31 % (p<0.000). Wait times increased by 16% (p=0.003). Conclusions: Referral management helped respond to increased referral requests. Despite no change in accepted referrals, wait times increased, suggesting a significant capacity problem and focus for further work.

Background: Advancements in AVM surgical techniques for cerebral arteriovenous malformation (AVM) underscore its efficacy. Our research aims to showcase the positive outcomes of treating low-grade AVMs surgically, focusing on safety and effectiveness. Methods: We retrospectively reviewed 55 patients with Spetzler-Martin (S-M) grade 1 and 2 AVMs who underwent surgical resection between January 2009 and December 2024. Results: In our study, 55 patients with S-M grade 1 and 2 AVMs underwent surgical resection, evenly divided between grades 1 (50.9%) and 2 (49.1%). Intracranial hemorrhage was the primary symptom in 74.5% of cases. Pre-operative Glasgow coma scale (GCS) scores revealed 69.1% of patients scored above 13, with 18% below 8. Successful resection was achieved in 87.3%. Postoperatively, 95.5% of ruptured and 90.9% of unruptured AVM patients showed lower or same modified Rankin scale scores. Poorer outcomes were significantly linked to lower GCS scores and intranidal/flow-related aneurysms. Conclusions: Our findings indicate surgical resection as a beneficial treatment for low-grade AVMs, yielding high cure rates and positive functional outcomes in both ruptured and unruptured cases. Preoperative GCS scores and the presence of associated aneurysms are predictive of postoperative functional status. Additionally, managing postoperative seizures effectively is key to enhancing prognosis

Background: Biochemical cure in functional pituitary adenomas (FPA) is crucial for reducing patient morbidity and improving quality of life following endoscopic endonasal procedures (EEA). The extent of resection plays a key role in achieving these outcomes. However, even with the aid of intraoperative navigation, complete resection of tumor components can be challenging due to the difficulty in distinguishing them from normal pituitary tissue. Indocyanine green (ICG) fluorescence has been used effectively in various cranial and spinal procedures, but its role in endoscopic skull base surgery has not yet been routinely established Methods: In this study, we describe our experience using ICG during EEA for the resection of FPA. Results: We discuss the fluorescence profiles of both adenomas and normal gland tissue. ICG helped identify additional tumor tissue that was not initially detected after macroscopic adenoma resection. It also allowed for perfusion assessment of the pituitary gland and nasoseptal flaps. No complications were observed following the ICG injection, and biochemical cure was achieved in more than 90% of cases. Conclusions: Our experience suggests that ICG is a safe and promising tool, improving both the extent of resection and endocrinologic outcomes in patients undergoing EEA for FPA.

Background: Patients with severe traumatic brain injury (TBI) are at uniquely high risk of venous thromboembolism (VTE), but the benefits of VTE prophylaxis must be weighed against the risk of intracranial hemorrhage expansion. Current guidelines are heterogenous in their recommendations for chemical VTE prophylaxis (cVTEp) in this high-risk cohort. We conducted a systematic review to identify the optimal timing of cVTEp in severe TBI patients. Methods: We executed a systematic search of the literature to identify adult severe TBI patients treated with cVTEp. Results were pooled, analyzed using random-effects models, and presented as Forest plots and odds ratios. Results: We included 21 studies representing 322,735 patients. The odds of VTE were 0.47 (95% CI: 0.37,0.60) when using the authors’ own criteria for early initiation, and the odds of VTE remained significantly decreased in subgroup analysis (<24h, <48 and <72h). Early VTEp both as defined by authors and in subgroup analysis did not significantly impact the odds of hemorrhage progression or mortality; except for initiation <48h which showed a positive impact on mortality (OR: 0.74, 95% CI: 0.63-0.87). Conclusions: This study supports early initiation of cVTEp in reducing the odds of VTE events without significantly increasing the risk of adverse events.