To evauluate our novel ultrasound model for measurement of optic nerve sheath diameter (ONSD) and determine the intra- and inter-operator variability associated with this technique.

We conducted ten measurements of ONSD per model amongst eight different models with a single experienced operator to examine intra-operator variability. Similarly, we had seven different operators measure the OSND twice in eight different models, in order to determine inter-operator variability analyzed with a three level linear statistical model.

For intra-operator variability, the intra-cluster correlation coefficients for the experienced and novice operators were 0.643 and 0.453 respectively. This displayed improvement in intra-operator variability with experience. The inter-cluster correlation coefficient was 0 for the group of novice operators, indicating negligible difference amongst multiple operators in measuring any given model of ONSD. A strong, statistically significant, linear relationship between the actual model disc size and the ultrasound ONSD measures was identified, implying the reliability of the images produced by our novel model.

Utilizing a novel model for ONSD ultrasonography, we have determined the intraoperator reliability of ONSD measurement to be moderate, with no appreciable difference amongst multiple operators. Improvement in measurement reliability has been demonstrated between expert and novice operators with our model, indicating the potential benefit of simulation platforms for teaching the technique of ONSD ultrasound.

Whether an association exists between cerebral microbleeds (CMBs) and functional recovery after ischemic stroke is unclear. We aimed to evaluate the association between CMBs and functional outcome after acute ischemic stroke.

Consecutive patients with acute stroke were enrolled, and all patients were stratified into good and poor functional outcome groups at discharge and 6 months after ischemic stroke by using a modified Rankin Scale score. Cardiovascular risk factors, CMBs, and white matter hyperintensities were compared between these two outcome groups. Logistic regression analysis was used to estimate the risk of poor functional outcomes.

A total of 225 patients were enrolled, 121 of whom were classified as having a good functional outcome at discharge and 142 as having a good 6-month functional outcome. The presence of CMBs was associated with a poor functional outcome at discharge [CMBs (+) patients in poor vs. good functional group; 48.1% vs. 30.6%; p=0.007] and 6 months [53.0% vs. 30.3%; p=0.001]. After adjustment for confounding factors, only the presence of infratentorial CMBs was associated with a poor functional outcome at discharge and 6 months. The poor functional outcome group had more CMBs than the good outcome group at 6 months.

Infratentorial cerebral microbleeds were significantly associated with worse functional outcomes not only in the early phase of ischemic stroke but also in the chronic phase. These findings suggest that the presence of infratentorial CMBs can predict poor functional outcome after acute ischemic stroke.

The aim of the present study was to determine the optimum method to detect brainstem lesions in patients with Multiple Sclerosis (MS).

72 patients with the diagnosis of relapsing-remitting MS were prospectively included. Brainstem functional system score (BSFS) (part of the expanded disability status scale (EDSS) evaluating brainstem symptomatology) was calculated. Magnetic resonance imaging (MRI) was performed on 1.5T and T1, T2, PD and fluid-attenuated inversion recovery (FLAIR) sequences were analyzed for presence of brainstem lesions. Auditory evoked potentials (AEP) and ocular and cervical vestibular evoked myogenic potentials (oVEMP and cVEMP) were performed according to the standardized protocol.

From 72 patients, 18 (25%) had clinical involvement of the brainstem. MRI showed brainstem involvement in 29 (40%) patients. Of the neurophysiological tests, AEP showed pathological result in 16 (22%) patients, oVEMP in 36 (50%) patients, cVEMP in 18 (25%) patients, and VEMP (combination of oVEMP and cVEMP) in 45 (63%) patients. VEMP detected brainstem lesions in higher percentage than clinical examination, MRI and AEP, which was statistically significant (< 0.0001, 0.012 and < 0.0001, respectively).

Results of the present study have shown that VEMPs are the optimal method to detect brainstem lesions in multiple sclerosis and that they detect them significantly better than clinical examination, AEP or MRI.

Genetic and environmental factors are important determinants of disease distribution. Several disorders associated with ataxia are known to occur more commonly in certain ethnic groups; for example, the disequilibrium syndrome in the Hutterites. The aim of this study was to determine the ethnic and geographic distribution of pediatric patients with chronic ataxia in Manitoba, Canada.

We identified 184 patients less than 17 years-of-age with chronic ataxia during 1991-2008 from multiple sources. Their diagnosis, ethnicity and place of residence were determined following a chart review.

Most patients resided in Manitoba (N=177) and the majority in Winnipeg, the provincial capital. Thirty five Aboriginal, 29 Mennonite and 11 Hutterite patients resided in Manitoba. The latter two groups were significantly overrepresented in our cohort. Ataxia telangiectasia, mitochondrial disorders, and non-progressive ataxia of unknown etiology associated with pyramidal tracts signs and developmental delay were significantly more common in Mennonite patients. Four of five patients with neuronal migration disorders associated with chronic ataxia were Aboriginal. Few isolated disorders with chronic ataxia occurred in the 11 Hutterite patients including a Joubert syndrome related disorder.

Three disorders associated with chronic ataxia were more prevalent than expected in Mennonites in Manitoba. Few rare disorders were more prevalent in the Hutterite and Aboriginal population. Further research is needed to determine the risk factors underlying these variations in prevalence within different ethnic groups. The unique risk factor profiles of each ethnic group need to be considered in health promotion endeavors.

With over 44,000 individual farms, farm dwellers account for 11% of the population of Saskatchewan. There is limited data on brain and spine injuries acquired on farms. The objective of this study was to evaluate the epidemiology of head and spine injuries on Saskatchewan farms to assist the development of injury prevention initiatives.

Using the Canadian Centre for Agricultural Health and Safety's Saskatchewan Farm Injury Surveillance Database, farm-related head and spine injuries hospitalized > 24 hours were examined (1990-2007). We collected information regarding the type and mechanism of injury as well as the geographic location of both the injury and treatment.

The database captured 390 brain injuries and 228 spine injuries, including 16 spinal cord injuries. The majority of patients were male (73.3% of head injuries and 84.2% of spine injuries). The highest risk age groups were 50-59 years, with 24.1% of the spine injuries, and 40-49 years, with 19.2% of the head injuries. The most common causes of injury were falls and/or machinery-related. The average annual incidence of farm-related spine and head injury were 10.8 and 17.6 per 100,000 farm population, respectively. All patients included in this study were hospitalized for over 24 hours, with 44.7% of spine injuries spending over one week in hospital, and 20% of head injuries spending over three days in hospital.

Injury prevention initiatives should be targeted towards males aged 40-59 years residing in the southern areas of the province, with increased awareness towards the dangers of falls and operating tractors.

Peripheral neuropathy is a cardinal manifestation of the autosomal recessive spastic ataxia of Charlevoix- Saguenay (ARSACS), although its type of neuromuscular involvement has not been definitely established, and magnetic resonance imaging (MRI) plays an important role in the assessment of muscle and nerve diseases. The objective of this work has been to define the patterns of muscle weakness and of abnormal muscular MRI in ARSACS.

Five patients with a molecular diagnosis of ARSACS, aged 39 to 59 years, whose electrophysiological findings were consistent with an axonal neuropathy of distal distribution superimposed on a developmental defect of myelinization, underwent neurological and MRI lower-limb examinations. Conventional FSE T1-weighted and STIR sequences were performed, looking for fatty infiltration and oedema in the musculature of the thighs, legs and feet, together with their distribution along the longitudinal axis of the muscle bellies.

On clinical examination, paralysis was apparent in foot muscles; moderate weakness, in leg musculature; and normal strength, in thigh muscles. MRI demonstrated massive fat deposition in the foot muscles and medial gastrocnemii in every case, distal fat infiltration and oedema in every leg muscle group, and preservation of thigh muscles, albeit with diffuse minimal non-specific fat infiltration. An inverse correlation between strength and degree of fat infiltration in lower-limb muscles became apparent.

The preponderance of weakness and MRI abnormalities in distal muscle groups was concordant with the presence of a length-dependent axonopathy, as described in ARSACS.

Vinca alkaloids and platinum-containing chemotherapeutic drugs have the potential to cause chemotherapy-induced peripheral neuropathy (CIPN). This study determined the frequency of CIPN among children who were treated for acute lymphoblastic leukemia (ALL), lymphoma, brain tumour or Wilms tumour.

This retrospective cohort study reviewed 252 patients treated at the Children's hospital of Eastern Ontario from 2001-2011. Patients were considered to have CIPN if they developed clinical symptoms of CIPN such as limb paraesthesia, weakness and/or ataxia during chemotherapy and their treating neurologist or oncologist deemed that their symptoms were due to a peripheral cause. Patients were excluded if their treatment regime did not include chemotherapy.

The overall frequency of CIPN was 18.3% (46/252). Tumour-specific CIPN rates were: 18.9% (29/154) for ALL; 9.4% (3/32) for lymphoma; 17.9% (5/28) for Wilms tumour; and 23.7% (9/38) for brain tumour patients. Nerve conduction studies were completed for 17% of patients (all tumour types) and were abnormal in all but one patient. Among surviving CIPN patients (41/46), 93% showed no clinical deficits at their last examination, which was on average 56 months from time of diagnosis to last follow-up visit.

The frequency of CIPN was less than that previously reported in adults receiving chemotherapy. Children with CIPN have a favourable outcome with most showing clinical improvement during the maintenance phase of treatment or after chemotherapy completion.

Neurosurgery may involve significant blood loss and frequently requires allogeneic red blood cell (RBC) transfusion. Preoperative recombinant erythropoietin (EPO) may be used to improve erythroid status and recovery, and used either alone or in combination with preoperative autologous donation (PAD) it may reduce exposure to allogeneic RBC. We wished to study the use of EPO with and without PAD and the risk of RBC transfusion in neurosurgery.

Using a retrospective case-control design, 57 patients who received EPO preoperatively were matched 2:1 for age, sex, year of surgery, and International Classification of Diseases code most responsible for surgery (three were excluded because of stringent matching criteria, leaving 54 cases and 108 comparison subjects). Thirty-two cases participated in PAD. Medical and anesthetic records as well as laboratory investigations were reviewed and extracted.

Allogeneic RBC exposure was identical for EPO cases and comparison subjects (18.5%). Concomitant PAD and EPO did not reduce allogeneic RBC exposure (21.9%), and resulted in a greater number of RBC units transfused. Last recorded hemoglobin levels suggested that autologous RBCs were not more liberally used. Patients who engaged in PAD and EPO suffered from iatrogenic anemia. A significant proportion (58.6%) of the autologous RBCs was ultimately not used and discarded.

Further research is needed to determine the efficacy of EPO in neurological surgery. PAD does not appear to reduce the risk of allogeneic RBC transfusion, despite concomitant EPO. Indeed, PAD resulted in iatrogenic anemia and increased transfusion requirements. The cost-effectiveness of blood conservation efforts in neurosurgery deserves additional research.

To define the incidence of new contralateral intracranial lesions following decompressive hemicraniectomy for blunt traumatic brain injury, and explore the potential association with metabolic factors that contribute to coagulopathy.

We retrospectively reviewed the records and imaging of all patients treated with hemicraniectomy for blunt traumatic brain injury at our institution from May 2007 up to and including January 2012.

Twenty patients were identified during the time period to have undergone decompressive craniectomy for blunt head injury. The average age and Glasgow Coma Scale on presentation was 44.1 years (range: 19 – 72 years) and 6.5 (range: 3 – 14) respectively. All but one patient presented with an extra-axial hematoma as their surgical indication for craniectomy. Seven patients (35.0%) developed new contralateral lesions post-craniectomy. The average peri-operative pH, bicarbonate (HCO3) and hematocrit (HCT) levels for those with new contralateral lesions were lower than those without new lesions. Five of the seven patients (71.4%) with new lesions had abnormalities on their laboratory results that have been know to be attributable to coagulopathy, with four (57.1%) having two or more abnormal results. Eight of 13 (61.5%) patients without new lesion had laboratory abnormalites, with five (38.5%) having two or more abnormalities identified.

The incidence of new contralateral lesions post-craniectomy for blunt head injury is 35.0% in our experience. There is an association between the metabolic derangements linked to trauma related coagulopathy and the formation of new lesions.

Homozygous homeobox A1 (HOXA1) mutations cause a spectrum of abnormalities in humans including bilateral profound deafness. This study evaluates the possible role of HOXA1 mutations in familial, non-syndromic sensorineural deafness.

Forty-eight unrelated Middle Eastern families with either consanguinity or familial deafness were identified in a large deafness clinic, and the proband from each family was evaluated by chart review, audiogram, neuroimaging, and HOXA1 sequencing.

All 48 probands had normal neuro-ophthalmologic and general medical examinations except for refractive errors. All had congenital non-syndromic sensorineural hearing loss that was symmetric bilaterally and profound (>90 dBHL) in 33 individuals and varied from 40 to 90 dBHL in the remainder. Thirty-nine of these individuals had neuroimaging studies, all documenting normal internal carotid arteries and normal 6th, 7th, and 8th cranial nerves bilaterally. Of these, 27 had normal internal ear structures with the remaining 12 having mild to modest developmental abnormalities of the cochlea, semicircular canals, and/or vestibular aqueduct. No patient had homozygous HOXA1 mutations.

None of these patients with non-syndromic deafness had HOXA1 mutations. None had major inner ear anomalies, obvious cerebrovascular defects, or recognized congenital heart disease. HOXA1 is likely not a common cause of non-syndromic deafness in this Middle Eastern population.

Optimal treatment of glioblastoma (GBM) in the elderly remains unclear. The impact of age on treatment planning, toxicity, and efficacy at a Canadian Cancer Centre was retrospectively reviewed.

Glioblastoma patients treated consecutively between 2004 and 2008 were reviewed. Utilizing 70 years as the threshold for definition of an elderly patient, treatments and outcome were compared in younger and elderly populations.

Four hundred and twenty one patients were included in this analysis and median overall survival (OS) for the entire cohort was 9.8 months. 290 patients were aged <70 (median age 57, range 17–69) and 131 were aged ≥70 (median age 76, range 70–93). Patients ≥70 were more likely to receive best supportive care (BSC) and all patients >70 who were treated with radiotherapy received <60 Gy (P<0.001), except one. Patients aged >70 demonstrated inferior survival (one year OS 16% versus 54% for those <70, HR 3.46, P<0.001). In patients treated with BSC only, age had no impact on survival (median survival two months in both groups, HR 0.89, P=0.75). For those treated with higher doses of radiotherapy (>30 Gy to <60 Gy), one year survival was 19% versus 24% in patients aged >70 versus <70 (HR 1.47, P=0.02) respectively.

In this retrospective single institution series, elderly patients were more likely to be treated with BSC or palliative doses of radiotherapy. Randomized phase III study results are required for guidance in treatment of this population of patients.