The expansion of neurosurgery and neurology in Montreal and Toronto in the early 20th century was the stimulus for the development of neuropathology in Canada. Rooted in the disciplines of the neurosciences and laboratory medicine, neuropathology evolved into an independent discipline with the founding of the Canadian Association of Neuropathologists in 1960, and the recognition as a specialty by the Royal College of Physicians and Surgeons in Canada in 1965, fostering the development of several successful training programs. Nonetheless, a paucity of data remains concerning the background of training, scopes of practice, and career paths.

We conducted a survey of all physicians in Canada who have either practiced neuropathology or undergone relevant training.

In 2009, 53 physicians were engaged in the practice of neuropathology, either exclusively or a substantial proportion of their time. Most work in tertiary hospitals, but a few service non-academic medical centers. Three routes of training were identified: direct from medical school (often with relevant research training), indirect from another clinical neuroscience specialty, and following or in conjunction with certification in one of the other pathology specialties. Practice profiles differ slightly, and some of the neuropathologists entering from pathology have mixed anatomical pathology/neuropathology responsibilities. Many of those with prior exposure in the neurosciences are more productive with regard to research and publications.

Existing multiple options for neuropathology training have facilitated recruitment and allowed development of a mosaic of specialists able to fulfill the diversity of needs in Canadian medical and scientific communities.

Treatment options for Chiari I malformations include posterior fossa decompression (PFD) with additional techniques including laminectomy, intradural exploration, and duraplasty. Neuroimaging findings of cisterna magna volume, syringomyelia, and intraoperative ultrasonography may tailor surgical intervention.

We developed an algorithm classifying symptomatic Chiari I patients into three groups to define minimum operation. Without syringomyelia, the presence of cisterna magna defined Group A and the absence defined Group B. Patients with syrinx formed Group C. Mild structural pathology (Group A) or adequate space following PFD (Group B, normal intraoperative ultrasound (IOUS)) should be treated by PFD alone. Conversely, presence of syringomyelia (Group C) or inadequate space following PFD (Group B, abnormal IOUS) should additionally have duraplasty. We applied this algorithm to patients treated at a single institution over 16 years.

Twenty-four symptomatic Chiari I malformation patients were divided into three groups that did not differ by age, gender, or extent of tonsillar ectopia. All patients treated by this algorithm experienced clinical and radiographic improvement. This included eight Group B patients who underwent PFD only (n=6) or additional duraplasty (n=2) decided by IOUS.

Treatment of symptomatic Chiari I malformation may have inadequate outcome with conservative strategy or complications with aggressive strategy. This algorithm utilizes preoperative neuroimaging and intraoperative ultrasound to tailor intervention, with excellent clinical outcome and radiographic syrinx resolution on application to 24 patients. Further validation requires prospective multicenter evaluation with larger patient population.

Specialization is generally independently associated with improved outcomes for most types of surgery. This is the first study comparing the immediate success of outpatient lumbar microdiscectomy with respect to acute complication and conversion to inpatient rate. Long term pain relief is not examined in this study.

Two separate prospective databases (one belonging to a neurosurgeon and brain tumor specialist, not specializing in spine (NS) and one belonging to four spine surgeons (SS)) were retrospectively reviewed. All acute complications as well as admission data of patients scheduled for outpatient lumbar microdiscectomy were extracted.

In total, 269 patients were in the NS group and 137 patients were in the SS group. The NS group averaged 24 cases per year while the SS group averaged 50 cases per year. Chi-square tests revealed no difference in acute complication rate [NS(6.7%), SS(7.3%)] (p>0.5) and admission rate [NS(4.1%), SS(5.8%)] (p=0.4) while the SS group had a significantly higher proportion of patients undergoing repeat microdiscectomy [NS(4.1%), SS(37.2%)] (p<0.0001). Excluding revision operations, there was no statistically significant difference in acute complication [NS(5.4%), SS(1.2%)] (p=0.09) and conversion to inpatient [NS(4.3%), SS(4.6%)] (p>0.5) rate. The combined acute complication and conversion to inpatient rate was 6.9% and 4.7% respectively.

Based on this limited study, outpatient lumbar microdiscectomy can be apparently performed safely with similar immediate complication rates by both non-spine specialized neurosurgeons and spine surgeons, even though the trend favored the latter group for both outcome measures.

The ability to predict conversion to multiple sclerosis (MS) accurately when assessing a patient with a clinically isolated syndrome (CIS) is of paramount importance.Magnetic resonance imaging (MRI) is the best paraclinical tool currently available; however the significance of a history of an event suggestive of demyelination prior to CIS presentation has not been evaluated.

Aretrospective chart review of all optic neuritis cases presenting as CIS to a single neuro-ophthalmologist in London, Ontario between 1990 to 1998 was performed. Data were collected regarding demographics, past medical history, history of present illness, and family history. Conversion to MS was determined by the McDonald criteria after ten years of follow-up. Bayesian statistics and logistic regression were used to determine the best predictors of conversion to MS from CIS.

One hundred and sixteen optic neuritis subjects were included in the analysis. After ten years, 42.2% had converted to MS. The best predictor of future conversion remained at least one brain lesion, disseminated in space, on MRI (sensitivity 0.90, specificity 0.75). However, if the subject additionally had a history suggestive of a demyelinating event in the past that had not been confirmed clinically, the specificity increased to 0.96. These two traits taken together had an odds ratio of 27.8 for conversion to MS in the next ten years (p<0.001).

A history of an event suggestive of demyelination prior to presenting with optic neuritis as CIS increases the ability of the clinician to predict conversion to MS in the next ten years.

Patients with chronic migraine and medication overuse are significant consumers of health care resources.

To determine whether botulinum toxin type A prophylaxis reduces the cost of acute migraine medications in patients with chronic migraine and triptan overuse.

In this multicenter, open-label study, patients with chronic migraine (≥15 headache days/month) who were triptan overusers (triptan intake ≥10 days/month for ≥3 months) received botulinum toxin type A (95-130 U) at baseline and month three. Headache (HA) frequency and medication use were assessed with patient diaries, and headache-related disability by means of the MIDAS and Headache Impact Test-6 questionnaires.

Of 53 patients enrolled (mean age ± standard deviation, 46.5 years ± 8.4; 47 [88.7%] females), 48 (90.6%) completed the study at month six. Based on headache diaries, significant (P≤0.0002) decreases from baseline were observed for days per month with headache/migraine, days with any acute headache medication use, days with triptan use, and triptan doses taken per month. A significant (P<0.0001) increase from baseline in headache-free days per month was also observed. Prescription medication costs for acute headache medications decreased significantly, including significant reductions in triptan costs (mean reduction of -C$106.32 ± 122.87/month during botulinum toxin type A prophylaxis; P<0.0001). At baseline, 78% of patients had severe disability (MIDAS score) and 86.8% had severe impact due to headache (HIT-6 scores); at month six, this decreased to 60% and 68%, respectively.

Botulinum toxin type A prophylactic therapy markedly decreased costs related to acute headache medication use in patients with chronic migraine and triptan overuse.

The purpose of this study was to evaluate Canadian amyotrophic lateral sclerosis (ALS) centres with respect to: 1) the prevalence of Non-invasive positive pressure ventilation (NIPPV) and invasive mechanical ventilation via tracheostomy (TV) utilization, 2) the approach to NIPPV use, focusing upon the currently employed initiation criteria and 3) the barriers influencing NIPPV administration.

A descriptive survey research design aimed to obtain quantitative data and open-ended responses from an active physician at each of the 15 multidisciplinary Canadian ALS centres.

The principal findings of this study were: 1) NIPPV and TV are used in 18.3% and 1.5% of patients at Canadian ALS centres, respectively, 2) symptoms of respiratory insufficiency, namely orthopnea (clinical significance rated at 9.00/10 ± 1.48), dyspnea (8.27 ± 1.95) and morning headache (7.55 ± 1.21) are the most significant indicators for NIPPV initiation, 3) the primary barriers to NIPPV utilization are patient intolerance (70% of centres) and inaccessibility of respirologists and ventilation technologists (50% of centres).

Variability in NIPPV use has an impact upon the management of Canadian ALS patients. The establishment of more definitive NIPPV initiation criteria, emphasizing respiratory symptoms, and the attenuation of barriers to NIPPV use should be targeted so as to ensure optimal care for all ALS patients.

Diagnostic considerations for juvenile onset Parkinsonism (onset at <21 years of age) include juvenile Huntington disease, Wilson disease, dentatorubral-pallidoluysian atrophy (DRPLA), storage diseases, and mitochondrial cytopathies. Neuronal Intranuclear Inclusion Disease (NIID) must also be considered.

We present a case of juvenile onset NIID with a predominantly Parkinsonian presentation, followed later by corticospinal, cerebellar, and lower motor neuron symptoms.

Diagnosis of NIID can be made antemortem through rectal biopsy, however it was missed in this case. Rectal biopsy should be performed in all suspected cases, reviewed by an experienced neuropathologist and repeated if the suspicion for NIID is high. Pathologically, SUMO-1 immunohistochemistry appears to reliably label the neuronal inclusions and abnormal SUMOylation may play a part in the pathogenesis.

In Parkinson's disease (PD) cell loss in the substantia nigra is known to result in motor symptoms; however widespread pathological changes occur and may be associated with non-motor symptoms such as cognitive impairment. Diffusion tensor imaging is a quantitative imaging method sensitive to the micro-structure of white matter tracts.

To measure fractional anisotropy (FA) and mean diffusivity (MD) values in the corpus callosum and cingulum pathways, defined by diffusion tensor tractography, in patients with PD, PD with dementia (PDD) and controls and to determine if these measures correlate with Mini-Mental Status Examination (MMSE) scores in parkinsonian patients.

Patients with PD (17 Males [M], 12 Females [F]), mild PDD (5 M, 1F) and controls (8 M, 7F) underwent cognitive testing and MRI scans. The corpus callosum was divided into four regions and the cingulum into two regions bilaterally to define tracts using the program DTIstudio (Johns Hopkins University) using the fiber assignment by continuous tracking algorithm. Volumetric MRI scans were used to measure white and gray matter volumes.

Groups did not differ in age or education. There were no overall FA or MD differences between groups in either the corpus callosum or cingulum pathways. In PD subjects the MMSE score correlated with MD within the corpus callosum. These findings were independent of age, sex and total white matter volume.

The data suggest that the corpus callosum or its cortical connections are associated with cognitive impairment in PD patients.

In Canada, ancillary tests, such as selective four vessels angiography (S4VA), are sometimes necessary for brain death (BD) diagnosis when the clinical exam cannot be completed or confounding factors are present. Recent Canadian guidelines assert that brain death is supported by the absence of arterial blood flow at the surface of the brain and that venous return should not be considered. However, neuropathologic and angiographic studies have suggested that arteries might still be patent in BD patients. Current clinical practices in BD diagnosis following S4VA need to be better understood.

We conducted a retrospective study of all S4VA performed for the determination of BD in a level 1 NeuroTrauma centre from 2003 to 2007. The objective of the study was to describe the prevalence of intracranial arterial, capillary (parenchymogram) and venous opacification in our study population. All tests were reviewed independently by two neuroradiologists. Disagreements were resolved by consensus.

Thirty two patients were declared BD following S4VA during the study period. Nine of these patients (28%) presented some proximal opacification of intracranial arteries (95% CI 15-45%). As opposed, none had a cerebral capillary and deep venous drainage opacification (95% CI 0-10%).

The absence of cerebral deep venous drainage or parenchymogram might represent a better objective marker of cerebral circulatory arrest for brain death diagnosis when the use of S4VA is required. These findings open the path for further research in enhancing our interpretation of angiographic studies for brain death diagnosis.

To present a new semiological description of unruptured middle cerebral artery (MCA) aneurysms.

We present a series of three MCA aneurysms presenting with progressive or paroxystic somatosensory symptoms in combination with visceral, motor, language or autonomic symptoms.

A surgical approach was proposed for two aneurysms, and both patients experienced complete resolution of their symptoms. The third aneurysm was successfully excluded by endovascular coiling but the symptoms persisted.

To our knowledge this is the first description of unruptured aneurysms presenting with insular-related symptoms.

To examine predictive factors associated with onset of depression among individuals diagnosed with Parkinson's disease (PD).

Depression may precede or follow symptomatic parkinsonism in PD. It is frequently treatable but often overlooked.

The clinical series comprised 685 individuals who were diagnosed with PD and followed by one neurologist (RJU) from 1994 to 2007. The primary outcome was time to depression following the onset of PD. Diagnosis of depression was based on clinical assessment of depressive symptoms from patients (and spouse/family/caregiver) and antidepressant usage. A number of demographic, historical and clinical predictive factors were examined, including gender, age at symptomatic onset, disease duration, onset characteristics, clinical ratings, antiparkinsonian medications, cognitive status, depression history, and familial history of PD and other neurodegenerative disorders.

Seventy-two percent of patients developed depression within ten years of symptomatic PD onset, and the mean time to depression was 7.9 years (median: 5.7 years). Factors associated with depression included longer PD duration, greater impairment in activities of daily living, and positive family history of motor neuron disease (MND).

A high rate of individuals with PD develop depressive symptoms during the course of the disease. Based on first clinic visit characteristics, most factors examined were not helpful in identifying individuals with an increased risk of depression. However, disease duration, functional limitations and family history of MND should lead clinicians to an increased vigilance for identifying depression.

All-terrain vehicle (ATV) use is increasingly popular among people of all ages. Although ATV use is known to cause significant morbidity due to head and neck trauma, there is a lack of published data detailing ATV-related fatalities. We examined all ATV-related fatalities in Ontario from 1996 - 2005 to determine the epidemiology and risk factors as a guide for improved injury prevention strategies.

All ATV-related fatalities from 1996 - 2005 in Ontario were examined through Coroner's reports in the Office of the Chief Coroner of Ontario. Epidemiologic information and risk factors relating to the driver, environment, and vehicle were recorded.

There were 74 ATV-related fatalities from 1996 - 2005. There was only one fatality per year in 1996 and 1997 and a peak of 16 per year in 2004 and 2005. Head and neck injuries were the commonest causes of death. Males comprised 90.5% of the cases. The highest risk was from age 15 - 29, and 21% of fatalities occurred in children under 16. Northeastern Ontario had the highest fatality rate.

There was a major increase in the incidence of ATV-related fatalities in Ontario from 1996 - 2005 with the majority due to head trauma. Notable risk factors included alcohol use, riding at night, lack of helmet use, and excessive speed. We recommend the adoption of laws that focus on helmet requirements, a minimum driver age of 16, and certified training courses. Aggressive injury prevention efforts should be targeted toward males aged 15 - 29.

Primitive reflexes and parkinsonian signs are used by clinicians to differentiate among dementias. We reviewed our clinical sample to determine whether primitive reflexes were more prevalent in frontally-based dementias and whether parkinsonian signs were more common in dementia with Lewy bodies (DLB) than in other types of dementia.

We retrospectively reviewed charts from 204 patients with dementia who presented for consultation at Baycrest's Ross Memory Clinic between April, 2003, to December, 2007.

A greater proportion of subjects with DLB and dementia of the Alzheimer type with cardiovascular disease had primitive reflexes than subjects with frontotemporal dementia (FTD). Primitive reflexes were not positively predictive of FTD or vascular dementia (VaD). Dementia with Lewy bodies subjects were more likely to have parkinsonian signs than the other dementias, and bradykinesia and rigidity were positively predictive of FTD. The palmomental reflex was the most common primitive reflex in the sample, and cogwheeling was the most common parkinsonian sign. There was no significant difference between early- and late-stage groups in presence of primitive reflexes or parkinsonian signs.

Primitive reflexes appear not to be clinically discriminative of frontally-based dementias such as FTD and VaD.

To compare the reproducibility of semi-automated vessel analysis software to manual measurement of carotid artery stenosis on computed tomography angiography (CTA).

Two observers separately 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 observer performed this task twice on each carotid, the second analysis 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. Correlation coefficients were calculated for each group comparing the narrowest stenosis in mm, distal ICA in mm, and NASCET percent stenosis.

The semi-automated vessel analysis software provided excellent intraobserver correlation for narrowest stenosis in mm, distal ICA in mm, and NACSET percent stenosis (Pearson correlation coefficients of 0.985, 0.954, and 0.977 respectively). The semi-automated vessel analysis software provided excellent interobserver correlation (0.925, 0.881, and 0.892 respectively). The interobserver correlation for manual measurement was good (0.595, 0.625, and 0.555 respectively). There was a statistically significant difference in the interobserver correlation between the semi-automated vessel analysis software observers and the manual measurement observers (P < 0.001).

Semi-automated vessel analysis software is a highly reproducible method of quantifying carotid artery stenosis on CTA. In this study, semi-automated vessel analysis software determination of carotid stenosis was shown to be more reproducible than manual measurement.