To save content items to your account,
please confirm that you agree to abide by our usage policies.
If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account.
Find out more about saving content to .
To save content items to your Kindle, first ensure no-reply@cambridge.org
is added to your Approved Personal Document E-mail List under your Personal Document Settings
on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part
of your Kindle email address below.
Find out more about saving to your Kindle.
Note you can select to save to either the @free.kindle.com or @kindle.com variations.
‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi.
‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
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.
To examine the added value of an elevated calculated peak right ventricular outflow tract pressure gradient as a surrogate for infective endocarditis diagnosis.
Methods:
A cohort study included patients admitted between 2003 and 2020 at a tertiary children’s medical centre. Patients with surgically inserted exogenous right ventricular outflow tract conduits and infective endocarditis were included. Infective endocarditis was diagnosed using the revised Duke criteria (2023). Controls had right ventricular outflow tract conduits and febrile illness from other causes. Clinical, laboratory, and echocardiographic findings, including calculated peak right ventricular outflow tract pressure gradient, were collected.
Results:
Among 26 febrile episodes (11 with infective endocarditis, 15 controls), the infective endocarditis group had a higher peak right ventricular outflow tract pressure gradient during acute illness (70 vs. 23 mmHg, p < 0.05). On admission, 18% of infective endocarditis patients had a definite diagnosis by Duke’s criteria, 45% had a probable diagnosis, and 36% lacked confirmation. Including peak right ventricular outflow tract gradient as a major criterion would yield a 90% diagnosis rate upon admission (45% definite, 45% possible).
Conclusions:
Increased right ventricular outflow tract pressure gradient in febrile patients with exogenous conduit in the right ventricular outflow tract is a potential marker for infective endocarditis. Including this gradient as a major Duke criterion enables earlier and more definitive diagnosis in debatable cases.
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.
Pulmonary artery capacitance is a relatively novel measurement associated with adverse outcomes in pulmonary arterial hypertension. We sought to determine if preoperative indexed pulmonary artery capacitance was related to outcomes in paediatric heart transplant recipients, describe the changes in indexed pulmonary artery capacitance after transplantation, and compare its discriminatory ability to predict outcomes as compared to conventional predictors.
Methods:
This was a retrospective study of paediatric patients who underwent heart transplant at our centre from July 2014 to May 2022. Variables from preoperative and postoperative clinical, catheterisation, and echo evaluations were recorded. The primary composite outcome measure included postoperative mortality, postoperative length of stay in the top quartile, and/or evidence of end organ dysfunction.
Results:
Of the 23 patients included in the analysis, 11 met the composite outcome. There was no statistical difference between indexed pulmonary artery capacitance values in patients who met the composite outcome [1.8 ml/mmHg/m2 (interquartile 0.8, 2.4)] and those who did not [1.4 (interquartile 0.9, 1.7)], p = 0.17. There were no significant signs of post-operative right heart failure in either group. There was no significant difference between pre-transplant and post-transplant indexed pulmonary artery capacitance or indexed pulmonary vascular resistance.
Conclusions:
Preoperative pulmonary artery capacitance was not associated with our composite outcome in paediatric heart transplant recipients. It did not appear to be additive to pulmonary vascular resistance in paediatric heart transplant patients. Pulmonary vascular disease did not appear to drive outcomes in this group.
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.
Background: Attitudes toward aging influence many health outcomes, yet their relationship with cognition and Alzheimer’s disease (AD) remains unknown. To better understand their impact on cognition and AD risk, we examined whether positive attitudes predict better cognition and diminished risk on AD biomarkers. Methods: A subsample of older adults with a family history of AD (n=54; women=39) from the McGill PREVENT-AD cohort participated in this study. Participants completed the Attitudes to Ageing Questionnaire (AAQ-24), providing three scores: psychosocial loss, psychological growth and physical change. Participants underwent cognitive testing (Rey Auditory Verbal Learning Test, RAVLT; Delis-Kaplan Executive Function System-Color Word Interference Test, D-KEFS-CWIT), and AD blood-based biomarker assessments (p-tau217, Aβ42/40). Regression models tested associations, adjusting for covariates (age, sex, education, depression, APOE4), and were Bonferroni corrected. Results: Positive attitudes were associated with better recall and recognition (RAVLT) and improved word reading, colour naming, switching, and inhibition (D-KEFS-CWIT) (p<0.00077), while negative attitudes showed the opposite pattern. Negative attitudes were correlated with lower Aβ42/40 ratios, while positive attitudes were linked to lower p-tau217 (p<0.0167). Conclusions: These findings demonstrate that positive attitudes predict better cognition and a lower risk profile for AD biomarkers, suggesting that life outlook may be an early disease feature or a risk factor.
Background: Reversible cerebral vasoconstriction syndrome (RCVS) is defined clinically by headaches associated sometimes with seizures and neurological deficits, and radiologically with intermittent spasms in cerebral arteries that would resolve in 3 months. It can present with multiple bleeding patterns. Intraventricular hemorrhage (IVH) is a rare presentation for RCVS as there are 4 reported cases. Methods: This is a case report and review of literature. Results: A 36-year-old patient sought medical attention due to the acute onset of thunderclap headache. A computed tomography (CT), and CT angiogram (CTA) of his head and neck were done. It showed large IVH associated with hydrocephalus and no underlying vascular abnormalities. An external ventricular drain was placed and he was on nimodipine. A CTA was done on day 17 because he had acute global aphasia, right hemibody weakness, and right homonomous hemianopia, which showed severe diffuse intracranial stenosis involving the circle of Willis. He was taken to the angiography suite for chemical spasmolysis with verapamil. There was radiographic improvement and clinical resolution. Conclusions: This case highlights the rarity of RCVS presentations and further signifies the utility of intraarterial spasmolytics as an adjunct in the diagnosis of difficult cases. Further retrospective and prospective data are needed.
Background: The increasing use of anti-B cell therapies in managing multiple sclerosis(MS) around the time of conception has raised important considerations for neonates exposed in utero. International recommendations suggest assessing neonatal B cell count in potentially exposed neonates. Practical implementation of cord blood collection at birth requires coordinated care across specialties, including paediatric haematology, neurology and obstetrics. Methods: This workshop, scheduled for 01/30/2025, will address clinical and logistical challenges of neonatal B cell assessments following in utero exposure to anti-B cell therapies. Presentations by MS pregnancy specialists from Toronto,Ontario, will be complemented by collaborative problem-solving among participants, including a paediatric haematologist, MS neurologists, obstetricians, paediatricians, and a quality specialist. A patient with lived experience will contribute to discussions. Results: The workshop will develop a care pathway for cord blood B cell testing, optimizing vaccine scheduling at London Health Sciences Center (LHSC) in London Ontario. Outcomes will include enhanced multidisciplinary collaboration, participant feedback, development of a practical clinical care plan for B cell collection and interpretation and measures of the pathway’s impact on patient satisfaction and clinical decisions. Conclusions: This initiative will improve care for mothers and neonates exposed to anti-B cell therapies, addressing critical gaps in clinical practice through collaboration and a standardized approach.
Previous studies on the association between fruit juice consumption and type 2 diabetes remain controversial, which might be due to heterogeneity in the polygenic risk score (PRS) for type 2 diabetes. We examined the association between fruit juice and type 2 diabetes by PRS for type 2 diabetes. We investigated whether fruit juice influences type 2 diabetes risk differently among individuals with varying genetic risks. Data from the Japan Multi-Institutional Collaborative Cohort (J-MICC) study, a cross-sectional study of 13 769 Japanese individuals was used for our analysis. The primary exposure was the frequency of fruit juice, categorised as do not drink, less than 1 cup per day or more than 1 cup per day. We selected PGS002379, a PRS for type 2 diabetes developed using East Asian populations. The primary outcome was physician-diagnosed type 2 diabetes, reported by participants. The consumption of fruit juice was significantly inversely associated with type 2 diabetes in the group with a high PRS for type 2 diabetes (OR: 0·78, 95 % CI: 0·65, 0·93 for < 1 cup/d and OR: 0·54, 95 % CI: 0·30, 0·96 for > 1/d), but this association was not observed in the low PRS group. Fruit juice consumption was inversely associated with type 2 diabetes, especially in genetically high-risk populations for type 2 diabetes.