Causalgia and complex regional pain syndrome (CRPS) type II with nerve injury can be difficult to treat. Surgical peripheral nerve denervation for causalgia has been largely abandoned by pain clinicians because of a perception that this may aggravate a central component (anesthesia dolorosa).

We selectively searched Pubmed, Cochrane, MEDLINE, EMBASE, CINAHL Plus, and Scopus from 1947 for articles, books, and book chapters for evidence of surgical treatments (nerve resection and amputation) and treatment related to autoimmunity and immune deficiency with CRPS.

Reviews were found for the treatment of causalgia or CRPS type II (n = 6), causalgia relieved by nerve resection (n = 6), and causalgia and CRPS II treated by amputation (n = 8). Twelve reports were found of autoimmunity with CRPS, one paper of these on associated immune deficiency and autoimmunity, and two were chosen for discussion regarding treatment with immunoglobulin and one by plasma exchange. We document a report of a detailed and unique pathological examination of a CRPS type II affected amputated limb and related successful treatment with immunoglobulin.

Nerve resection, with grafting, and relocation may relieve uncomplicated causalgia and CRPS type II in some patients in the long term. However, an unrecognized and treatable immunological condition may underly some CRPS II cases and can lead to the ultimate failure of surgical treatments.

Embolization of middle meningeal artery (EMMA) is a relatively new treatment for chronic subdural hematoma (CSDH). To date, an objective method that assesses or describes the extent of EMMA for the treatment of CSDH does not exist. Recently, the concept of a novel grading scale for EMMA in patients with CSDH has emerged. However, this has not been applied to a clinical case setting and inter-rater reliability has not yet been studied. The purpose of this study was to validate the grading scale in clinical practice and to assess for inter-rater reliability.

We retrospectively examined consecutive patients who underwent EMMA for CSDH. Patients were included if the whole head angiogram from common carotid as well as external carotid arteries before and after EMMA were available in the arterial, capillary as well as venous phases. Two independent readers, each with more than 5 years of experience in independent practice, assessed the angiograms for the grading of EMMA and assigned a score ranging between 0 and 3. The grading score between the two readers were compared using Cohen’s Kappa score to assess the inter-rater reliability.

In 19 patients, we found that EMMA had no periprocedural morbidity and mortality. The number of cases in each EMMA grading score category are as follows: 0 n =1; 1 n =3; 2 n =1; and 3 n =10. There was substantial inter-rater reliability for the assessment of grading of EMMA (Kappa = 0.74).

The novel EMMA grading scheme demonstrated substantial inter-rater reliability and appears promising.

Isocitrate dehydrogenase (IDH) mutation status is a key diagnostic and prognostic feature of gliomas. It is thought to occur early in glioma tumorigenesis and remain stable over time. However, there are reports documenting a loss of IDH mutation status in a subset of patients with glioma recurrence. Here, we identified patients with a documented loss of IDH mutation status longitudinally and performed multi-platform analysis in order to determine if IDH mutations are stable throughout glioma evolution.

We retrospectively identified patients from our institution from 2009 to 2018 with immunohistochemistry (IHC)-recorded IDH mutation status changes longitudinally. Archived formalin-fixed paraffin-embedded and frozen tissue samples from these patients were collected from our institution’s tumour bank. Samples were analysed using methylation profiling, copy number variation, Sanger sequencing, droplet digital PCR (ddPCR) and IHC.

We reviewed 1491 archived glioma samples including 78 patients with multiple IDH mutant tumour samples collected longitudinally. In all instances of documented loss of IDH mutation status, multi-platform profiling identified a mixture of low tumour cell content and non-neoplastic tissue including perilesional, reactive or inflammatory cells.

All patients with a documented loss of IDH mutation status longitudinally were resolved through multi-platform analysis. These findings support the hypothesis that IDH mutations occur early in gliomagenesis and in the absence of copy number changes at the IDH loci and are stable throughout tumour treatment and evolution. Our study highlights the importance of accurate surgical sampling and the role of DNA methylome profiling in diagnostically uncertain cases for integrated pathological and molecular diagnosis.

Frontotemporal dementia (FTD) patients frequently present with psychosis, which complicates diagnosis and management. In this study, we aim to examine the relationship between psychosis and the most common genetic mutations predisposing to FTD, and in the different pathological subtypes of FTD.

We conducted a systematic review, searching the literature up to December 2022, and reviewed 50 articles that met our inclusion criteria. From the reviewed articles, we extracted and summarized data regarding the frequency of psychosis and patient characteristics in each major genetic and pathological subtype of FTD.

Among FTD patients with confirmed genetic mutations or pathological diagnosss, the frequency of psychosis was 24.2%. Among the genetic mutation carriers, C9orf72 mutation carriers had the highest frequency of psychosis (31.4%), whereas GRN (15.0%) and MAPT (9.2%) mutation carriers had lower frequencies of psychosis. MAPT mutation carriers notably developed psychosis at a younger age compared to other genetic groups. The most common psychotic symptoms were delusions among C9orf72 carriers and visual hallucinations among GRN mutation carriers. Among the pathological subtypes, 30% of patients with FUS pathology, 25.3% of patients with TDP-43 pathology, and 16.4% of patients with tau pathology developed psychosis. In the TDP-43 group, subtype B pathology was the most common subtype reported in association with psychosis.

Our systematic review suggests a high frequency of psychosis in specific subgroups of FTD patients. Further studies are required to understand the structural and biological underpinnings of psychosis in FTD.

Levodopa-carbidopa intestinal gel (LCIG) therapy has been shown to be a safe and effective treatment for advanced Parkinson’s disease (PD). Limited data are available regarding long-term benefits and complications in Canada. Objective of the study was to review long-term experience and clinical outcomes in PD patients with LCIG therapy over 11 years in a multidisciplinary University clinic setting.

Chart review was done on PD patients with LCIG from 2011 to 2022. Data collected: dosing, UPDRS-III motor scores, OFF times, hours with dyskinesias, MoCA, complications, discontinuation reasons, and nursing time requirements.

Thirty-three patients received LCIG therapy with a mean follow-up of 3.25±2.09 years. UPDRS-III scores showed reduction of 15% from baseline (mean 35.9) up to 4 years (mean 30.4). Daily OFF time improved from baseline (mean 7.1 ± 3.13 hours) up to 5 years (mean 3.3 ± 2.31 hours; −53.5%; p < 0.048), and dyskinesias remained stable. Nursing time averaged 22 hours per patient per year after PEG-J insertion and titration. Most common complications were PEG-J tube dislodgement and stoma site infection (0–3zero to three events/patient/year). Serious side effects were seen in four (12%) patients resulting in hospitalization and/or death. Nine patients (27.2%) discontinued the treatment due to lack of improved efficacy over oral therapy or development of dementia and 10 (30%) died of causes unrelated to LCIG infusion.

Patients on LCIG showed improved motor function over 5-year follow-up. Serious complications were uncommon. Dedicated nursing time is required by LCIG-trained nurses in a multidisciplinary setting for optimum management.

To conduct feasibility and cost analysis of portable MRI implementation in a remote setting where MRI access is otherwise unavailable.

Portable MRI (ultra-low field, 0.064T) was installed in Weeneebayko General Hospital, Moose Factory, Ontario. Adult patients, presenting with any indication for neuroimaging, were eligible for study inclusion. Scanning period was from November 14, 2021, to September 6, 2022. Images were sent via a secure PACS network for Neuroradiologist interpretation, available 24/7. Clinical indications, image quality, and report turnaround time were recorded. A cost analysis was conducted from a healthcare system’s perspective in 2022 Canadian dollars, comparing cost of portable MRI implementation to transporting patients to a center with fixed MRI.

Portable MRI was successfully implemented in a remote Canadian location. Twenty-five patients received a portable MRI scan. All studies were of diagnostic quality. No clinically significant pathologies were identified on any of the studies. However, based on clinical presentation and limitations of portable MRI resolution, it is estimated that 11 (44%) of patients would require transfer to a center with fixed MRI for further imaging workup. Cost savings were $854,841 based on 50 patients receiving portable MRI over 1 year. Five-year budget impact analysis showed nearly $8 million dollars saved.

Portable MRI implementation in a remote setting is feasible, with significant cost savings compared to fixed MRI. This study may serve as a model to democratize MRI access, offer timely care and improved triaging in remote areas where conventional MRI is unavailable.

There is a paucity of data on the obstetrical outcomes of Canadian pregnant patients with epilepsy, which may differ from the average Canadian pregnancy and from other populations of pregnant patients with epilepsy.

Pregnant patients with epilepsy were identified from a prospectively collected database of patients seen at the maternal-fetal medicine obstetrics program of Mount Sinai Hospital (Toronto, Canada) between January 1, 2014, and November 20, 2020. Pregnancy, delivery, and neonatal outcome data were retrieved from this database and described using 95% binomial confidence intervals. Comparisons of obstetrical outcomes over the same period among the Canadian population average, obtained from publicly available national health data, were done using one-proportion Z-tests for nominal variables and one-sample t-tests for continuous variables.

In total, 282 pregnancies, from 224 patients, were included, which resulted in 274 live births. Mean maternal age was 32.8 years (s.d. = 4.6; population average [μ] = 30.9; p < 0.01), and 53% were primiparous (CI95% = 49%–61%; μ = 43%; p < 0.01). The observed rates of obstetrical complications were gestational hypertension 9% (CI95%=6%–13%; μ=7%; p=0.19), gestational diabetes 5% (CI95% = 3%–8%; μ = 9%; p = 0.02), cesarean section 44% (CI95% = 38%–50%; μ = 28%; p < 0.01), postpartum hemorrhage 5% (CI95% = 3%–8%; μ = 0.5%; p < 0.01), stillbirth 1% (CI95% = 0%–2%; μ=1%; p > 0.99), and prematurity 9% (CI95% = 6%–13%; μ = 8%; p = 0.44).

In this cohort of Canadian pregnant patients with epilepsy from an urban tertiary care center, observed rates of obstetrical complications were rare and no higher than in the Canadian population over the same period, with the exception of cesarean section and postpartum hemorrhage. Future prospective studies that include primary care and rural settings are needed to increase the generalizability of those results.

Objective, evidence-based neuroprognostication of postarrest patients is crucial to avoid inappropriate withdrawal of life-sustaining therapies or prolonged, invasive, and costly therapies that could perpetuate suffering when there is no chance of an acceptable recovery. Postarrest prognostication guidelines exist; however, guideline adherence and practice variability are unknown.

To investigate Canadian practices and opinions regarding assessment of neurological prognosis in postarrest patients.

An anonymous electronic survey was distributed to physicians who care for adult postarrest patients.

Of the 134 physicians who responded to the survey, 63% had no institutional protocols for neuroprognostication. While the use of targeted temperature management did not affect the timing of neuroprognostication, an increasing number of clinical findings suggestive of a poor prognosis affected the timing of when physicians were comfortable concluding patients had a poor prognosis. Variability existed in what factors clinicians’ thought were confounders. Physicians identified bilaterally absent pupillary light reflexes (85%), bilaterally absent corneal reflexes (80%), and status myoclonus (75%) as useful in determining poor prognosis. Computed tomography, magnetic resonance imaging, and spot electroencephalography were the most useful and accessible tests. Somatosensory evoked potentials were useful, but logistically challenging. Serum biomarkers were unavailable at most centers. Most (79%) physicians agreed ≥2 definitive findings on neurologic exam, electrophysiologic tests, neuroimaging, and/or biomarkers are required to determine a poor prognosis with a high degree of certainty. Distress during the process of neuroprognostication was reported by 70% of physicians and 51% request a second opinion from an external expert.

Significant variability exists in post-cardiac arrest neuroprognostication practices among Canadian physicians.

Optic nerve hypoplasia (ONH) and septo-optic-pituitary dysplasia (SOD) are common causes of congenital visual impairment. Our primary aim was to investigate the prevalence of abnormal neuroimaging features in patients with these disorders in Manitoba, Canada, and compare them with published reports.

A retrospective neuroimaging review was performed in patients resident in Manitoba with ONH/SOD.

There were 128 patients (M = 70) with ONH/SOD who had neuroimaging. Their mean age (SD) at the end of the study was 13.2 (7.5) years. Males were significantly more likely to have bilateral ONH and a small optic chiasm size, while females were more likely to have a left ONH and a small left optic chiasm size on neuroimaging (p = 0.049). ONH and small optic chiasm size were seen in most patients on neuroimaging. Absent septum pellucidum was noted in 40%, small pituitary gland size in 28%, neuronal migration disorders (NMD) in 20% (>1 type and bilateral in 13 cases), corpus callosum abnormalities were present in 9%, while olfactory bulbs-tracts and olfactory sulci were absent in 8.6% of cases. Unilateral ONH was not significantly associated with other structural brain abnormalities, while NMD were significantly associated with other midline brain abnormalities including a symmetrically small optic chiasm size.

The prevalence of structural neuroimaging abnormalities in our cohort with ONH/SOD was generally in the same range reported in other studies with corpus callosum abnormalities being relatively less common in our study. Bilateral NMD were relatively common among patients with NMD. The association between sex and ONH laterality requires further study.