Glioblastoma is the most common and malignant primary brain tumour in adults. Maximum feasible surgical resection, radiotherapy and temozolomide chemotherapy at initial diagnosis have improved prognosis but rapid recurrence is typical and survival remains brief. There is an urgent need for effective new treatments and approval of the antiangiogenic agent bevacizumab for recurrent glioblastoma by Health Canada in 2009 has been the most notable recent therapeutic advance for this disease. This review with illustrative case studies highlights how bevacizumab has been incorporated into the treatment of glioblastoma in Canada and describes the ongoing controversies surrounding its clinical application.

Background: A definitive diagnosis of multiple sclerosis (MS), as distinct from a clinically isolated syndrome, requires one of two conditions: a second clinical attack or particular magnetic resonance imaging (MRI) findings as defined by the McDonald criteria. MRI is also important after a diagnosis is made as a means of monitoring subclinical disease activity. While a standardized protocol for diagnostic and follow-up MRI has been developed by the Consortium of Multiple Sclerosis Centres, acceptance and implementation in Canada have been suboptimal. Methods: To improve diagnosis, monitoring, and management of a clinically isolated syndrome and MS, a Canadian expert panel created consensus recommendations about the appropriate application of the 2010 McDonald criteria in routine practice, strategies to improve adherence to the standardized Consortium of Multiple Sclerosis Centres MRI protocol, and methods for ensuring effective communication among health care practitioners, in particular referring physicians, neurologists, and radiologists. Results: This article presents eight consensus statements developed by the expert panel, along with the rationale underlying the recommendations and commentaries on how to prioritize resource use within the Canadian healthcare system. Conclusions: The expert panel calls on neurologists and radiologists in Canada to incorporate the McDonald criteria, the Consortium of Multiple Sclerosis Centres MRI protocol, and other guidance given in this consensus presentation into their practices. By improving communication and general awareness of best practices for MRI use in MS diagnosis and monitoring, we can improve patient care across Canada by providing timely diagnosis, informed management decisions, and better continuity of care.

Purpose: To compare patient effective dose resulting from two alternative imaging protocols for pre-coiling assessment of intracranial aneurysms: a series of 2D Digital Subtraction Angiography (DSA) projections, and a 3D rotational angiography (RA) acquisition. Methods: In a retrospective analysis, we investigated 44 patients who underwent endovascular coiling in our institution. Images were acquired on a biplane Image Intensifier system not equipped with dose-area product (DAP) meter. Conventional 2D DSA images were simulated with an anthropomorphic skull phantom. Entrance skin dose was measured with a 60 cc ion chamber, and the PCXMC Monte Carlo based software was used to calculate patient effective dose. For the RA protocol, a 16 cm computed tomography (CT) dosimetry phantom and a 100 mm pencil ion chamber were employed to measure the CT dose index. Patient effective dose was calculated with the ImPACT calculator. An unpaired two-tailed t-test was used to determine the significance of differences between patient doses in each group. Results: Sixteen patients underwent the 2D DSA protocol with multiple projections; their mean number of cine runs was 5.1; the mean effective dose was 2.11 millisievert (mSv) (range 1.69–3.43 mSv). Twenty eight patients were assessed using the 3D RA protocol with the effective dose of 1.29 mSv. The difference between the means of two dose distributions was statistically significant (p=0.00028). Conclusion: Our study demonstrated that the patient effective dose was significantly lower from the 3D RA protocol than that from the 2D DSA protocol used in the planning of coiling of intracranial aneurysm.

Background: Essential tremor (ET) is the most common tremor disorder in adults. In addition to upper limbs, the tremor in ET may also involve head, jaw, voice, tongue, and trunk. Though head tremor (HT) is commonly present in patients with ET, large comparative studies of ET patients with HT (HT+) and without HT (HT−) are few. Methods: To determine whether ET with HT is a distinct clinical subtype by comparing ET patients with and without HT, a chart review of 234 consecutive patients with ET attending the neurology clinics of the National Institute of Mental Health and Neurosciences, India, was done. A movement disorder specialist confirmed the diagnosis of ET in all patients using the National Institutes of Health collaborative genetic criteria. Results: HT was present in 44.4% of the patients. Comparison between HT+ and HT− showed that the HT+ group patients: (1) were older, (2) had later onset of tremor, (3) had unimodal distribution of age at onset with a single peak in the fifth decade, (4) had more frequent voice tremor, and (5) were more likely to have mild cervical dystonia. HT was part of presenting symptoms in nearly two thirds of the ET patients and in the rest it was detected during clinical examination. Conclusions: Several demographic and clinical variables suggest that ET patients with HT have a distinct clinical phenotype.

Background: Myotonic dystrophy (DM1) is an autosomal dominant, progressive, and multisystem condition that impacts affected individuals physically, socially, and emotionally. Understanding individuals’ perceptions of their disease is critical to ensuring appropriate information, education, and counseling. Methods: We conducted a content analysis of findings from a larger study that used a novel, qualitative research approach called photovoice to explore nine patients’ experiences of living with DM1. Participants took pictures that illustrated barriers or facilitators to living with DM1; their photographs then formed the basis of semistructured interviews. Transcripts were analyzed and, among themes, we identified one titled “DM1 truths and misinformation” that described participants’ disease knowledge. Analysis revealed four categories within this broader theme: “the physical and emotional cost of DM1,” “managing my DM1,” “genetics and me” and “patients as advocates and educators.” Results: Findings showed that DM1 participants had good core knowledge with respect to their disease and its implications. However, each participant held as fact fragments of misinformation that shaped decision-making and pointed to a clear need for strategies to mitigate variable interpretation of health information. Conclusions: We conclude that there is a need for increased education and awareness about symptoms, genetic information and treatment strategies for patients, their family members, and health care providers.

Background: Recent evidence suggests that an extreme shift may occur in sphingosine metabolism in neuroinflammatory contexts. Sphingosine 1-phosphate (S1P)-metabolizing enzymes (SMEs) regulate the level of S1P. We recently found that FTY720, a S1P analogue, and SEW2871, a selective S1P receptor 1 (S1P1) agonist, provide protection against neural damage and memory deficit in amyloid beta (Aβ)-injected animals. This study aimed to evaluate the effects of these two analogues on the expression of SMEs as well as their anti-inflammatory roles. Methods: Rats were treated with intracerebral lipopolysaccharide (LPS) or Aβ. Memory impairment was assessed by Morris water maze and the effects of drugs on SMEs as well as inflammatory markers, TNF- α and COX-II, were determined by immunoblotting. Results: Aβ and LPS differentially altered the expression profile of SMEs. In Aβ-injected animals, FTY720 and SEW2871 treatments exerted anti-inflammatory effects and restored the expression profile of SMEs, in parallel to our previous findings. In LPS animals however, in spite of anti-inflammatory effects of the two analogues, only FTY720 restored the levels of SMEs and prevented memory deficit. Conclusion: The observed ameliorating effects of FTY720 and SEW7821 can be partly attributed to the interruption of the vicious cycle of abnormal S1P metabolism and neuro-inflammation. The close imitation of the FTY720 effects by SW2871 in Aβ-induced neuro-inflammation may highlight the attractive role of S1P1 as a potential target to restore S1P metabolism and inhibit inflammatory processes.