Sporadic Jakob-Creutzfeldt disease (sCJD) and dementia with Lewy bodies (DLB) have overlapping clinical symptoms that can lead to their misdiagnosis. We delineated the clinical overlap between sCJD and DLB, and assessed the value of magnetic resonance imaging (MRI) to differentiate between them.

Medical records, MRI, electroencephalogram (EEG) and cerebrospinal fluid (CSF) were reviewed from 56 sCJD and 30 DLB subjects.

46% of sCJD subjects met probable DLB criteria and 40% of DLB subjects met probable CJD criteria. A greater proportion of sCJD subjects had cerebellar signs (66% vs. 10%, p<0.001), myoclonus (64% vs. 30%, p=0.002), and visual symptoms (other than hallucinations) (61% vs. 7%, p<0.001), whereas more DLB subjects had hallucinations (70% vs. 39%, p=0.007) and fluctuations (57% vs. 23%, p=0.002). Cortical and/or basal ganglia MRI diffusion weighted imaging hyperintensities consistent with sCJD were seen in 96% of sCJD subjects but in none with DLB. Logistic regression in sCJD revealed that those meeting probable DLB criteria were more likely to have occipital lobe involvement on MRI (OR 1.4, p=0.058, model p=0.022). Parietal lobe involvement on MRI was a predictor of “Other Focal Cortical signs” (OR 1.9, p=0.021). EEG and CSF assessments lacked sensitivity for sCJD as 48% of sCJD patients had a negative EEG; 67% of the 36 sCJD patents with a CSF evaluation had a negative or inconclusive 14-3-3 result. Too few DLB patients had EEG or CSF to assess their utility.

Sporadic CJD and DLB have significant symptom overlap. MRI helps differentiate these diseases and is related to the signs/symptoms observed in sCJD.

Stress-induced central effects are regulated by brain neurotransmitters, glucocorticoids and oxidative processes. Therefore, we aimed to evaluate the simultaneous alterations in the monoamine and antioxidant systems in selected brain regions (frontal cortex, striatum and hippocampus) at 1 hour (h) and 24h following the exposure of restraint stress (RS), to understand their initial response and possible crosstalk.

RS (150 min immobilization) significantly increased the dopamine levels in the frontal cortex and decreased them in the striatum and hippocampus, with selective increase of dopamine metabolites both in the 1h and 24h RS groups compared to control values. The serotonin and its metabolite levels were significantly increased in both time intervals, while noradrenaline levels were decreased in the frontal cortex and striatum only. The activities of superoxide dismutase, glutathione peroxidase and the levels of lipid peroxidation were significantly increased with significant decrease of glutathione levels in the frontal cortex and striatum both in the 1h and 24h RS groups. There was no significant change in the catalase activity in any group. In the hippocampus, the glutathione levels were significantly decreased only in the 1h RS group.

Our study implies that the frontal cortex and striatum are more sensitive to oxidative burden which could be related to the parallel monoamine perturbations. This provides a rational look into the simultaneous compensatory central mechanisms operating during acute stress responses which are particular to precise brain regions and may have long lasting effects on various neuropathological alterations.

Review of human cysticercosis in Canada, to estimate the magnitude of the disease and to describe the pattern of disease expression in this country.

MEDLINE and manual search of case reports and case series of patients with cysticercosis diagnosed in Canada. Abstracted data included year of diagnosis, citizenship status, clinical manifestations, and form of cysticercosis.

A total of 21 articles reporting 60 patients were found. Forty (67%) of these patients were diagnosed in the past two decades. Most cases came from Ontario (n=43) and Quebec (n=14). Immigrants accounted for 96% of the 28 cases in whom citizenship information was available. Neurocysticercosis was observed in 55 patients, and isolated compromise of striated muscles in the remaining five. Seizures was the primary or sole manifestation of the disease in 72% of patients, and most of them had parenchymal brain cysticerci (either viable cysts or calcifications). Two of seven patients were positive for Taenia eggs. In no case were household contacts of the patients investigated for taeniasis.

An increasing number of patients with cysticercosis have been reported from Canada in the past two decades, suggesting that the prevalence of this parasitic disease may be on the rise. While most cases occur in immigrants, it is possible that at least some of these patients had acquired the disease in Canada.

To explore the use of magnesium (Mg), an endogenous ion and enzymatic co-factor used in a variety of medical applications, for the treatment of epileptic seizures resistant to traditional medical therapy.

For almost a century, Mg has been used as prophylaxis and treatment of seizures associated with eclampsia. Mg is a CNS depressant, with numerous functions intracellularly and extracellularly. However, because of the availability of well studied anticonvulsant drugs, Mg has not been tested widely in the treatment of epileptic seizures.

A retrospective chart review of 22 cases of drug resistant epilepsy, where a trial of empiric oral Mg supplementation (mainly in the form of Mg-oxide) was conducted.

Oral Mg supplementation was associated with a significant decrease in the number of seizure days per month, from 15.3 ± 13.2 (mean ± SD) to 10.2 ± 12.6 at first follow up (3-6 months, p=0.021), and to 7.8 ± 10.0 seizure days/month at second follow up (6-12 months, p=0.004). Thirty-six percent had a response rate of 75% or greater at second follow-up. Two patients reported seizure freedom. Most patients were well maintained on MgO 420mg twice a day, or in 2 cases, Mg Lactate, without significant adverse effects, the most frequent being diarrhea (4/22).

These results suggest that oral Mg supplementation may prove to be a worthwhile adjunctive medication in treating drug intractable epilepsy.

A prospective, double-blinded, placebo controlled study is warranted to evaluate the potential of Mg for the treatment of drug-resistant seizures.

Non-convulsive seizures have been reported to be common in neurocritical care patients. Many jurisdictions do not have sufficient resources to enable routine continuous electroencephalography (cEEG) and instead use primarily intermittent EEG, for which the diagnostic yield remains uncertain. Determining risk factors for epileptiform activity and seizures could help identify patients who might particularly benefit from EEG monitoring.

We performed a cohort study involving neurocritical care patients with admission Glascow Coma Scale (GCS) scores =≤ 12, who underwent ≥ 1 EEG. EEGs were reviewed for presence of interictal discharges, periodic epileptiform discharges (PEDs), and seizures. Multivariate analysis was used to identify predictors of these findings and to describe their prognostic implications.

393 patients met inclusion criteria. 34 underwent cEEG, usually because epileptiform activity was first detected on a routine EEG. The prevalence of PEDs or electrographic seizures was 13%, and was highest with anoxic encephalopathy and central nervous system infections. Other independent predictors for epileptiform activity included a history of convulsive seizure(s), increasing age, deeper coma, and female gender. Although patients with epileptiform activity had higher mortality, this association disappeared after adjustment for confounders.

Approximately 7-8 neurocritical care patients must undergo intermittent EEG monitoring in order to diagnose one with PEDs or seizures. The predictors we identified could potentially help guide use of resources. Repeated intermittent studies, or cEEG, should be considered in patients with multiple risk factors, or when interictal discharges are identified on an initial EEG. It remains unclear whether aggressive prevention and treatment of electrographic seizures improves neurologic outcomes.

Recent studies have suggested that carotid artery angioplasty and stenting (CAS) is a safe alternative to carotid endarterectomy (CEA) in average risk patients <70 years of age. We examined a consecutive series of patients who underwent CAS in order to determine the influence of patient age on outcome.

A retrospective, longitudinal cohort study of consecutive patients who underwent CAS at St. Michael's Hospital, Canada between January 2001 and November 2010 was performed. The outcome measures were 30-day stroke and 30-day composite death, stroke and acute myocardial infarction (MI). Patients were stratified based on age <70 and ≥70 years.

One hundred and fifty-nine patients underwent 165 CAS procedures. The 30-day risk of stroke was 3.8% while the composite outcome of death/stroke/MI was 8.2%. When stratified by age <70 and ≥70 years, the 30-day stroke rate was 0% versus 7.4% (p=0.03), and the composite outcome of death/stroke/MI was 2.6% versus 13.6% (p=0.02), respectively.

Patients <70 years of age undergoing CAS have a low rate of major complications, comparing favourably with historical CEA adverse event rates, and supporting the recent carotid stenosis literature that in the younger population CAS has a similar complication rate compared to CEA.

To compare North American Symptomatic Carotid Endarterectomy Trial (NASCET) stenosis values and NASCET grade categorization (mild, moderate, severe) of semi-automated vessel analysis software versus manual measurements on computed tomography angiography (CTA).

There were four observers. Two independently analyzed 81 carotid artery CTAs using semi-automated vessel analysis software according to a blinded protocol. The software measured the narrowest stenosis in millimeters (mm), distal internal carotid artery (ICA) in mm, and calculated percent stenosis based on NASCET criteria. One of these two observers performed this task twice on each carotid, the second analysis was delayed two months in order to mitigate recall bias. Two other observers manually measured the narrowest stenosis in mm, distal ICA in mm, and calculated NASCET percent stenosis in a blinded fashion. The calculated NASCET stenoses were categorized into mild, moderate, or severe. Chi square and analysis of variance (ANOVA) were used to test for statistical differences.

ANOVA did not find a statistically significant difference in the mean percent stenosis when comparing the two manual measurements, the two semi-automated measurements, and the repeat semi-automated. Chi square demonstrated that the distribution of grades of stenosis were statistically different (p<0.05) between the manual and semiautomated grades. Semi-automated vessel analysis tended to underestimate the degree of stenosis compared to manual measurement.

The mean percentage stenosis determined by semi-automated vessel analysis is not significantly different from manual measurement. However, when the data is categorized into mild, moderate and severe stenosis, there is a significant difference between semi-automated and manual measurements. The semi-automated software tends to underestimate the stenosis grade compared to manual measurement.

Conus medullaris syndrome (CMS) is a clinical neurologic syndrome caused by a conus medullaris lesion. CMS is a heterogeneous entity with various etiologies such as trauma or a space-occupying lesion. Multiple cases of CMS following spinal anesthesia have been reported, but CMS after radioisotope (RI) cisternography has not yet been reported.

We present four patients who developed CMS after RI cisternography.

All experienced neurological deficits such as paraparesis, sensory loss, and urinary incontinence three to four days after RI cisternography. Two showed abnormalities on lumbar magnetic resonance imaging, and three had complete symptom resolution within ten weeks.

The pathomechanism of the CMS is unclear, but we hypothesize that RI neurotoxicity might be responsible. It is possible that the use of low-dose 99mTc-DTPA or an alternative diagnostic tool such as magnetic resonance cisternography could help to prevent this complication.

Multiple Sclerosis is characterized by relapses separated by periods of relative quiescence. High dose intravenous corticosteroid pulses for three to five days is the current standard for the treatment of acute relapses, but recent evidence supports the use of equivalent doses of oral therapy as an alternative. The highest single dose preparation of oral prednisone is a 50mg tablet, requiring patients to take 25 tablets a day. Questions regarding compliance with this oral regimen have been raised.

To determine whether MS patients are complaint with 1250mg of oral prednisone daily for acute relapses.

Between November 2008 and December 2009, all patients diagnosed with an acute relapse in the London (Ontario) MS clinic were prospectively identified. If treatment with oral prednisone was initiated, subjects were given a survey to be mailed anonymously to the clinic.

Sixtyeight MS relapses were diagnosed and treated with corticosteroids in 66 patients of which 60 (58 subjects) were treated with 1250mg prednisone. Fifty-three (91.4%) surveys were returned. The reported compliance rate was high at 94.3% (50/53) with only one patient reporting being unable to take all the required pills due to intolerance. Most subjects (43, 86.0%) encountered at least one side effect, most commonly insomnia, mood changes and increased appetite. Two thirds of subjects (69.8%) indicated a preference for oral medication for future relapses.

High dose (1250mg) oral prednisone is an acceptable therapy to MS patients for the treatment of acute relapses with a high rate of compliance.

To examine how the introduction of amplitude-integrated electroencephalography (aEEG) to our neonatal intensive care unit (NICU) influenced clinical practice.

This was a retrospective study examining clinical practice three years before and three years after the introduction of aEEG monitors to our NICU. A time series analysis was performed to explore whether aEEG introduction was associated with changes in the rates of conventional EEGs performed, neurology consultations and neonates diagnosed with seizures.

Following aEEG introduction, the total number of conventional EEGs performed remained constant; however, there was significant shift in conventional EEG utilization towards neonates receiving fewer multiple EEGs and more single EEGs. There was no change in the rate of neurology consultations or the number of neonates diagnosed with seizures.

Introduction of aEEG monitors to our NICU has led to less reliance on conventional EEG as a tool for the serial evaluation of brain function. Since the number of neonates diagnosed with seizures did not increase, aEEG monitoring did not appear to uncover a significant subgroup of patients with subclinical seizures that would previously have gone undetected. Conventional EEG and aEEG are complementary tools for the assessment of newborn cerebral function.

Concussion is a prevalent brain injury in the community. While primary prevention strategies need to be enhanced, it is also important to diagnose and treat concussions expertly and expeditiously to prevent serious complications that may be life-threatening or long lasting. Therefore, physicians should be knowledgeable about the diagnosis and management of concussions. The present study assesses Ontario medical students’ and residents’ knowledge of concussion management.

A survey to assess the knowledge and awareness of the diagnosis and treatment of concussions was developed and administered to graduating medical students (n= 222) and neurology and neurosurgery residents (n = 80) at the University of Toronto.

Residents answered correctly significantly more of the questions regarding the diagnosis and management of concussions than the medical students (mean = 5.8 vs 4.1, t= 4.48, p<0.01). Gender, participation in sports, and personal concussion history were not predictive of the number of questions answered correctly. Several knowledge gaps were identified in the sample population as a whole. Approximately half of the medical students and residents did not recognize chronic traumatic encephalopathy (n = 36) or the second impact syndrome (n = 44) as possible consequences of repetitive concussions. Twenty-four percent of the medical students (n = 18) did not think that “every concussed individual should see a physician” as part of management.

A significant number of medical students and residents have incomplete knowledge about concussion diagnosis and management. This should be addressed by targeting this population during undergraduate medical education.

Child neurology training in Canada has changed considerably over time, with increasing requirements for standardized teaching of the fundamentals of child neurology and the CanMEDS competencies. We sought to determine the current status of child neurology training in Canada as well future directions for training.

A web-based survey was sent to program directors (PD's) of active pediatric neurology training programs. General questions about the programs were asked, as well as about success at the Royal College of Physicians and Surgeons of Canada (RCPSC) exam, breakdown of rotations, views on CanMEDS roles and questions on the future of pediatric neurology.

9/9 PD's completed the survey. 96.5% of all trainees successfully passed their RCPSC exam from 2001-2006. Breakdowns of the number and type of rotations for each year of training were provided. All CanMEDS roles were deemed to be important by PD's and programs have developed unique strategies to teach and assess these roles. 92.6% of trainees chose to go into academic practice, with the most popular subspecialty being epilepsy. All PD's favour joint training sessions particularly for neurogenetics and neuromuscular disease. Overall, PD's suggest recruitment for future child neurologists at the medical student level but are divided as to whether we are currently training too few or too many child neurologists.

This survey provides a view of the current state of pediatric neurology training in Canada and suggestions for further development of postgraduate training. In particular, attention should be given to joint educational programs as well as urgently assessing the manpower needs of child neurologists.