{"title":"P.084 Early malignant transformation of intracranial epidermoid cysts: a case report and systematic review","authors":"C Tsai, AD Rebchuk, J Oh, S Yip, M. Fatehi","doi":"10.1017/cjn.2024.189","DOIUrl":"https://doi.org/10.1017/cjn.2024.189","url":null,"abstract":"Background: Intracranial epidermoid cysts (IEC) are benign congenital intracranial lesions that rarely undergo malignant transformation. We report a case of IEC evolving into squamous cell carcinoma (SCC) 1-year post-resection. Further, we conducted a systematic review on cases of early malignant transformations of IECs. Methods: MEDLINE, EMBASE, and Scopus were searched from inception until December 2023 for studies reporting malignant transformations of IECs within 2 years of diagnosis. Results: A 48-year-old female underwent surgical resection of a cerebellopontine angle (CPA) IEC in May 2022. She re-presented in July 2023 with headaches, nausea, vomiting, right facial weakness, and rapid cyst progression. Repeat surgical resection revealed a high-grade SCC. Our systematic review identified 19 (10 females, 9 males) additional IEC cases undergoing malignant transformation within 2 years. The mean age at presentation was 57.6 years, most common location was CPA (n=13, 68.4%) and mean time between IEC to malignant transformation was 10.6 months. Eighteen (94.7%) cases transformed to SCC, of which 2 had leptomeningeal carcinomatosis, and 1 transformed to glioblastoma. Conclusions: While malignant transformations of IECs are rare, regular postoperative follow-up is crucial for early malignancy detection and treatment initiation. Further study is warranted to evaluate factors contributing to accelerated malignant progression of IECs.","PeriodicalId":9571,"journal":{"name":"Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques","volume":"23 6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141099900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"P.140 The clinical outcomes of patients with normal pressure hydrocephalus and fecal incontinence","authors":"HK Cheema, E. Torio","doi":"10.1017/cjn.2024.241","DOIUrl":"https://doi.org/10.1017/cjn.2024.241","url":null,"abstract":"Background: Normal Pressure Hydrocephalus (NPH) is characterized by the clinical triad of dementia, gait disturbance, and urinary incontinence. An initial case series by Hakim and Adams highlighted that all patients exhibited this triad, with only one presenting with fecal incontinence. This study aims to examine the outcomes of individuals experiencing fecal incontinence who have undergone ventriculoperitoneal shunting (VPS). Methods: A systematic review and surgical case series was conducted, involving consecutive adults diagnosed with NPH and treated with VPS between September 2016 and September 2022. Results: In the cohort of 85 patients, the median duration of NPH symptoms was 3.2 years. Gait and balance symptoms were prevalent in all patients, while cognitive, bladder, and bowel symptoms were observed in 85.9%, 91.8%, and 23.5% of cases. No significant differences were noted in age, sex, neurologic diseases presence, or lower gastrointestinal or pelvic pathology. The prevalence of fecal incontinence pre-surgery, within less than 3 months, and 3 months post-surgery were 23.5%, 32.9%, and 17.6%. The systematic review search yielded 515 articles, and 18 included patients with fecal incontinence. Conclusions: The insights gained from the systematic review and cohort offer a comprehensive understanding of the outcomes observed in patients with NPH and fecal incontinence following VPS.","PeriodicalId":9571,"journal":{"name":"Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques","volume":"17 6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141099938","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
L. Marulanda, R. Harrison, MM Alzahrani, L. Armstrong, J. Hukin, KM Chapman
{"title":"P.071 Improving care for patients with neurofibromatosis 1 in British Columbia","authors":"L. Marulanda, R. Harrison, MM Alzahrani, L. Armstrong, J. Hukin, KM Chapman","doi":"10.1017/cjn.2024.177","DOIUrl":"https://doi.org/10.1017/cjn.2024.177","url":null,"abstract":"Background: Neurofibromatosis 1 is a multisystem, neurocutaneous disorder with a predisposition for various malignancies. There is no established care pathway or multidisciplinary clinic for adult patients with NF1 in British Columbia (BC). Patients may miss timely screening or therapeutic interventions. The development of new therapies for NF1 highlights the urgency for coordinated care. Methods: A review of existing programs and guidelines was conducted. The estimated population with NF1 in BC was determined. A working group consisting of neuromuscular neurology, pediatric neuro-oncology, adult neuro-oncology, and medical genetics identified gaps in care. Results: Approximately 2200 adult individuals with NF1 are estimated to live in BC. A three-prong approach to address identified gaps was developed: A quarterly multidisciplinary NF Case Conference was initiated. The initial session was attended by 18 providers. Focus groups for patients and providers to enhance understanding of both perspectives are being conducted. Informed by the focus groups, an NF1 Care Pathway for BC will be developed. Conclusions: Advances in treatment for NF1 prompted the formation of the BC NF Working Group to develop a strategy to improve longitudinal, multidisciplinary care. The development of a care pathway, with patient input, will improve care coordination and access to care.","PeriodicalId":9571,"journal":{"name":"Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques","volume":"7 43","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141099016","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J. Kam, C. Hounjet, S. Makarenko, B. Brakel, A. Rebchuk, M. Castle-Kirszbaum, R. Akagami
{"title":"P.096 Hearing preservation and quality of life outcomes in partial labyrinthectomy petrous apicectomy for microsurgical resection of large posterior fossa skullbase tumors","authors":"J. Kam, C. Hounjet, S. Makarenko, B. Brakel, A. Rebchuk, M. Castle-Kirszbaum, R. Akagami","doi":"10.1017/cjn.2024.201","DOIUrl":"https://doi.org/10.1017/cjn.2024.201","url":null,"abstract":"Background: The Partial Labyrinthectomy Petrous Apicectomy (PLPA) aims to give transpetrosal access whilst preserving hearing for challenging tumors such as petroclival meningioma. There are few studies assessing resection and morbidity and no large studies that document hearing preservation and quality of life (QOL). We present the first large series to do so. Methods: A retrospective review was performed of all PLPA cases between 2005 and 2023 at a tertiary center. Demographics, tumor characteristics, neuromonitoring, hearing and surgical outcomes were collected. QOL was measured with the 36-item short form survey (SF-36). Results: Of 73 PLPAs, data for 56 patients undergoing 57 surgeries was obtained. Petroclival meningioma (57.8%) and epidermoid tumors (21.0%) were common indications . The mean patient age and tumor size were 51.6 years and 44mm. Gross total resection was achieved in 40.3%, near total in 15.8% and subtotal in 43.8% of cases with no perioperative mortality and was not influenced by attempted hearing preservation (p=0.183). Of 39 hearing preservation cases, 27 (69.2%) were preserved, 10 (25.6%) were lost and 2 had unclear outcomes. Conclusions: Improved microsurgery and neuromonitoring during PLPA leads to decreased mortality and morbidity compared to historical cohorts while achieving a high rate of resection, hearing preservation and maintained QOL.","PeriodicalId":9571,"journal":{"name":"Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques","volume":"85 10","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141101547","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Z. Siddiqi, A. Genge, C. Qi, A. Zhou, R. Kaprielian, J. Locklin, D. Garcia
{"title":"D.2 Cost-effectiveness analysis of efgartigimod vs chronic IVIg for treatment of patients with generalized myasthenia gravis in Canada","authors":"Z. Siddiqi, A. Genge, C. Qi, A. Zhou, R. Kaprielian, J. Locklin, D. Garcia","doi":"10.1017/cjn.2024.93","DOIUrl":"https://doi.org/10.1017/cjn.2024.93","url":null,"abstract":"Background: Efgartigimod is a human IgG1 antibody Fc fragment recently approved by Health Canada for patients with acetylcholine receptor antibody positive (AChR-Ab+) generalized myasthenia gravis (gMG). We assessed cost-effectiveness of efgartigimod vs chronic IVIg for adult patients with AChR-Ab+ gMG. Methods: A Markov model estimated costs (treatment and administration, disease monitoring, complications from chronic corticosteroid use, exacerbation and crisis management, adverse events, end-of-life care) and benefits (quality-adjusted life-years [QALYs]). The analysis was conducted from the Canadian healthcare system perspective. Health state transition probabilities were estimated using data from ADAPT, ADAPT+, and a network meta-analysis comparing efgartigimod with chronic IVIg. Utility values were obtained from MyRealWorld MG. Patients with MG-ADL ≥5 who did not die/discontinue were assumed to receive treatment every 4 weeks or every 3 weeks over the lifetime horizon. Results: Over the lifetime horizon, efgartigimod and chronic IVIg 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 IVIg with incremental QALYs of 3.45 and cost savings of $257,020 over the lifetime horizon. Conclusions: Efgartigimod may provide greater benefit at lower costs than chronic IVIg for Canadian patients with AChR-Ab+ gMG, with substantial healthcare system savings over the lifetime horizon.","PeriodicalId":9571,"journal":{"name":"Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques","volume":"41 6","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141102266","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
H Lee, IM Scott, A. Chatterjee, IR Mackenzie, MI Lapid, ED Huey, C. Tartaglia, K. Kantarci, KP Rankin, HJ Rosen, BF Boeve, AL Boxer, G. Hsiung
{"title":"P.002 Distinct neuropsychiatric symptom trajectories in frontotemporal dementia across genetic mutations","authors":"H Lee, IM Scott, A. Chatterjee, IR Mackenzie, MI Lapid, ED Huey, C. Tartaglia, K. Kantarci, KP Rankin, HJ Rosen, BF Boeve, AL Boxer, G. Hsiung","doi":"10.1017/cjn.2024.110","DOIUrl":"https://doi.org/10.1017/cjn.2024.110","url":null,"abstract":"Background: Frontotemporal dementia (FTD) often presents with varying neuropsychiatric symptoms (NPS), which may differ based on genetic mutations. We hypothesized distinct NPS trajectories in FTD progression among carriers of chromosome 9 open reading frame 72 (C9orf72), progranulin (GRN), and microtubule-associated protein tau (MAPT) mutations. Methods: We analyzed 1662 participants from ALLFTD, including 342 C9orf72, 148 GRN, 168 MAPT mutation carriers, and 1004 noncarriers. We categorized participants into four stages based on CDR plus NACC FTLD global scores: 1) Presymptomatic (consistent CDR=0), 2) Early conversion (CDR increasing from 0 to 0.5), 3) Advanced conversion (CDR increasing from 0.5 to ≥1.0), and 4) Symptomatic (CDR>1.0). The Neuropsychiatric Inventory-Questionnaire (NPI-Q) assessed NPS changes, analyzed using a mixed-effects model, accounting for age and baseline scores. Results: Our results indicated similar NPS trajectories in the presymptomatic stage for all groups. Notably, during early conversion, C9orf72 and GRN carriers exhibited significantly higher NPI-Q score increases than MAPT carriers, primarily in psychosis and hyperactivity domains. In later stages, increases in NPS were similar across groups. Conclusions: This study suggests familial FTD progression, particularly in TDP-43 pathology, may involve more severe NPS like psychosis or hyperactivity, differing from tau pathology or sporadic FTD. Further research is needed to explore these distinct trajectories.","PeriodicalId":9571,"journal":{"name":"Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques","volume":"4 7","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141101268","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
SG Buttle, D Martin, L. Foster, K. Woodward, M. Esser
{"title":"C.5 Altered inflammatory profiles in critically ill children with neurologic involvement","authors":"SG Buttle, D Martin, L. Foster, K. Woodward, M. Esser","doi":"10.1017/cjn.2024.90","DOIUrl":"https://doi.org/10.1017/cjn.2024.90","url":null,"abstract":"Background: More than 1 in 4 children admitted to the pediatric ICU (PICU) have suspected neuroinflammation for a variety of reasons. While often beneficial, uncontrolled inflammation can lead to secondary neurologic injuries and interfere with repair mechanisms. Methods: A prospective cohort study was initiated at Alberta Children’s Hospital to evaluate neuroinflammation in children admitted to the PICU. Forty-eight cytokines, chemokines and growth factors collected at multiple pre-determined timepoints were analyzed along with data on clinical trajectory. Preliminary exploratory analyses of patients enrolled January 2022-July 2023 were completed. Results: Fifty-three patients were included in the initial analysis. Encephalopathy (18.9%), hypoxia (17%) and TBI (15.1%) were the most common reasons for enrollment. All groups had temporal alterations in serum cytokines, with primary inflammatory brain diseases having the highest levels of innate inflammation (cytokine storm) on admission and day one compared to other subgroups. There was a trend towards normalization of cytokine levels over time. Conclusions: Temporal profiling of cytokine levels can inform on neuroinflammatory pathways contributing to the clinical course in critically ill children. Further analysis is ongoing with the entire cohort to evaluate longitudinal and between-group differences. Improved understanding of altered neuroinflammatory pathways in this population may assist with rationalizing targeted immunotherapies to improve outcomes.","PeriodicalId":9571,"journal":{"name":"Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques","volume":"7 36","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141099023","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"P.130 Endoscopic fenestration of trapped fourth ventricle","authors":"KM Libbus, SA Walling, ED Leck, PD McNeely","doi":"10.1017/cjn.2024.231","DOIUrl":"https://doi.org/10.1017/cjn.2024.231","url":null,"abstract":"Background: Trapped fourth ventricle (TFV) is a rare entity that occurs when the fourth ventricle is obstructed and isolated from the normal cerebrospinal fluid (CSF) circulation. While not always symptomatic, TFV can lead to compression of the cerebellum and brainstem, with potential for serious consequences. Treatment of TFV can be challenging, with options including CSF diversion via shunts versus open or endoscopic fenestrations. In this report, we describe a case of TFV that was managed endoscopically. Methods: A seven-year-old girl with a history of myelomeningocele and hydrocephalus, presented with a change in neurological status. Imaging of the brain and spine showed syringomyelia, markedly dilated ventricles, and a TFV. An endoscopic approach was used to fenestrate the wall of the fourth ventricle. Results: While there was an early favorable outcome, the first fenestration closed over within one month, requiring a repeat endoscopic fenestration. Both procedures were complicated by transient seizures, requiring a pediatric intensive care unit (PICU) admission after the second intervention. Pre- and post-operative clinical and diagnostic imaging findings are reported. Conclusions: Endoscopic fenestration can be an effective treatment option for management of TFV. The patient, family, and treating team should be prepared to deal with acute peri-operative complications that may require PICU management.","PeriodicalId":9571,"journal":{"name":"Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques","volume":"1 12","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141099239","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
E. Liu, N Tilbury, A. Zhou, J Su, A. Persad, B. Newton, U Ahmed, L Peeling, M. Kelly
{"title":"P.124 Assessing the fragility index of randomized controlled trials on carotid artery stenosis: systematic review","authors":"E. Liu, N Tilbury, A. Zhou, J Su, A. Persad, B. Newton, U Ahmed, L Peeling, M. Kelly","doi":"10.1017/cjn.2024.225","DOIUrl":"https://doi.org/10.1017/cjn.2024.225","url":null,"abstract":"Background: The fragility index (FI) is the minimum number of patients whose status would have to change from a nonevent to an event to turn a statistically significant result to a non-significant result. We used this to measure the robustness of trials comparing carotid endarterectomy (CEA) to carotid artery stenting (CAS). Methods: A search was conducted in MEDLINE, Embase, and PubMed on RCTs comparing CEA to CAS. The trials need to have statistically significant results and dichotomous primary endpoints to be included. Results: Our literature search identified 10 RCTs which included 9382 patients (4734 CEA, 4648 CAS). The primary end points of all included trials favoured CEA over CAS. The median FI was 9.5 (interquartile range 2.25 - 21.25). All of the studies that reported lost-to-follow-up (LTFU) had LTFU greater than its fragility index, which raises concern that the missing data could change the results of the trial from statistically significant to statistically insignificant. Conclusions: A small number of events (FI, median 9.5) were required to render the results of carotid artery stenosis RCTs comparing CEA to CAS statistically insignificant. All of the studies that reported LTFU had LTFU greater than its fragility index.","PeriodicalId":9571,"journal":{"name":"Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques","volume":"2 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141098896","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Rebchuk, K. Tosefsky, KC Martin, D. Chen, S. Yip, S. Makarenko
{"title":"P.082 Preoperative corticosteroids reduce diagnostic accuracy for primary central nervous system lymphoma biopsies: a meta-analysis","authors":"A. Rebchuk, K. Tosefsky, KC Martin, D. Chen, S. Yip, S. Makarenko","doi":"10.1017/cjn.2024.187","DOIUrl":"https://doi.org/10.1017/cjn.2024.187","url":null,"abstract":"Background: Primary central nervous system lymphoma (PCNSL) is highly sensitive to corticosteroid induced cell arrest, apoptosis and shrinkage. However, the precise impact of preoperative corticosteroid on accuracy of PCNSL diagnosis using tissue obtained from open or stereotactic biopsies remains debated. Methods: We conducted a systematic review and meta-analysis to determine the effect of preoperative corticosteroids on non-diagnostic biopsy rates for PCNSL in immunocompetent adults. Subgroup analyses explored whether non-diagnostic rates varied based on biopsy type. Results: Nineteen studies, comprising 1226 patients (55% male; mean age: 60.3 years), of which 679 (55.4%) received corticosteroids prior to biopsy were included. Overall, patients pretreated with corticosteroids were two times more likely to have a non-diagnostic biopsy compared to patients that were corticosteroid-naïve prior to biopsy (RR = 2.1 [95% CI: 1.1-4.1]). In the subgroup analysis limited to stereotactic biopsies, patient pretreated with corticosteroids were three times more likely to have a non-diagnostic biopsy (RR = 3.0 [95% CI: 1.2-7.5]). Whereas, in the open biopsy subgroup, there was no significant difference in non-diagnostic rates. Conclusions: Corticosteroids should be withheld, if clinically safe, prior to stereotactic biopsies in cases of suspected PCNSL. If corticosteroids are administered preoperatively, an open biopsy should be considered instead of stereotactic biopsy.","PeriodicalId":9571,"journal":{"name":"Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques","volume":"66 24","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141101662","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}