Alzheimer’s disease (AD) is the most common cause of dementia in older adults. Acceptance of the cholinergic hypothesis led to a search for medications which could enhance central cholinergic activity in this condition. There are now three cholinesterase inhibitors available for the treatment of AD in Canada.

To review the currently available cholinesterase inhibitors approved for the treatment of AD in Canada and to provide guidance on who and how to treat with these agents.

Donepezil, rivastigmine, and galantamine are approved for the treatment of AD in Canada. In clinical trails, patients with mild to moderate AD treated with these agents experienced modest improvements in cognition, function, behaviour, and/or global clinical state. The magnitude of benefits seen with each agent appeared to be similar. While to date, there is no convincing evidence that one is more efficacious or effective, they do differ in their pharmacokinetics, additional mechanisms of action, and side effect profiles. Therefore, the selection of agent will be based on considerations such as side effect profiles, ease of administration, personal familiarity/experience, and beliefs about the importance of the noted differences in their pharmacokinetics and additional mechanisms of action.

We believe that these agents should be offered to all individuals with a mild to moderate dementia where Alzheimer’s pathology is felt to be a contributing factor. We view all three available cholinesterase inhibitors as first-line drugs.

Patients with Parkinsonism have a progressive disorder requiring substantial expertise to manage effectively.

Over a six-year period we evaluated physician utilization and related costs for a large, unselected cohort of 15,304 Parkinsonian patients from the general population, comparing them to 30,608 age- and sex-matched controls within a universal health care system in Ontario, Canada.

On average, 45% of Parkinsonian patients saw neurologists annually. The cumulative rate of at least one neurological consultation was only 59.5% over the six years. Patients aged <65 had a much greater likelihood of consulting a neurologist (73.3%) compared to those ≥65 (37.2%). Most Parkinsonian patients (97.2%), regardless of age, saw family physicians/general practitioners each year; 50.4% saw internal medicine consultants.

Parkinsonian patients had increased likelihood of utilizing neurologists, primary care physicians and internists compared to controls; related costs of physicians’ services were higher. Further research is necessary to evaluate differences in outcomes and costs between neurologists and other physician service providers.

This report summarizes an invitational symposium on epilepsy and Canadian laws governing motor vehicle driving held in Québec City in November 1998.

Invited neurological experts from Canada, the USA, and Europe; and representatives of provincial and territorial licensing bodies, the Canadian Council of Motor Transport Administrators, the Canadian Medical Protective Association, and the Canadian Medical Association participated. An edited version of transcribed audiotapes was prepared. Specific issues discussed were whether or not a physician should be required to report a patient with epilepsy to the licensing authority (mandatory reporting), the nature and quantification of the risks posed by epileptic drivers, and what would be a reasonable law regulating driving by people with epilepsy in Canada.

The consensus among medical experts was that mandatory reporting should be abolished in Canada and that a 6-12 month seizure-free period was appropriate before most patients could return to driving private cars. Experts also believed that these standards should be uniform across Canada. There was strong disagreement with the recommendation of the Canadian Medical Association that all such drivers be reported to provincial licensing authorities even in provinces without mandatory reporting rules.

Physicians should be familiar with and follow the rules regarding epilepsy and driving in the provinces where they practice. Nevertheless, current evidence is against mandatory physician reporting of drivers with epilepsy and the neurologists recommended that this be abolished throughout Canada. Shorter seizure-free intervals should also be considered before resuming driving of private cars.

The management of optic chiasmatic gliomas is controversial, partly related to failure to separate out those tumors involving the optic chiasm only (chiasmatic tumors) from those also involving the hypothalamus (chiasmatic/hypothalamic tumors). The purpose of this study was: (i) to analyze the outcomes of chiasmatic and chiasmatic/hypothalamic tumors separately; and (ii) to determine the appropriateness of recommending radical surgical resection for the chiasmatic/hypothalamic tumors.

A retrospective chart review of all newly diagnosed tumors involving the optic chiasm from 1982-1996 at British Columbia’s Children’s Hospital was performed.

There were 32 patients less than 16 years of age, 14 with chiasmatic and 18 with chiasmatic/hypothalamic astrocytomas, with an average duration of follow-up of 5.8 years and 6.3 years, respectively. Ten of the patients with chiasmatic tumors and none with chiasmatic/hypothalamic tumors had neurofibromatosis I. Thirteen of the 14 chiasmatic tumors were managed with observation only, and none had progression requiring active intervention. For the chiasmatic/hypothalamic tumors, eight patients had subtotal resections (>95% resection), six had partial resections (50-95%), three had limited resections (<50%), and one had no surgery. There were fewer complications associated with the limited resections, especially with respect to hypothalamic dysfunction. There was no correlation between the extent of resection (subtotal, partial, or limited) and the time to tumor progression (average 18 months).

In conclusion, chiasmatic and chiasmatic/hypothalamic tumors are different entities, which should be separated out for the purposes of any study. For the chiasmatic/hypothalamic tumors, there was more morbidity and no prolongation of time to progression when radical resections were compared to more limited resections. Therefore, if surgery is performed, it may be appropriate to do a surgical procedure that strives only to provide a tissue diagnosis and to decompress the optic apparatus and/or ventricular system.

The place of health-related quality of life (HRQoL) instruments in clinical research trials and clinical practice as compared to more traditional clinical outcome measures such as headache intensity and frequency is unclear.

To review the current status of HRQoL measurement in migraine.

A literature search was done for HRQoL and migraine. Selected articles dealing with migraine and commonly used HRQoL instruments and HRQoL measures used in recent clinical trials were reviewed.

Several general and migraine specific HRQoLinstruments can detect changes over time in response to at least major changes in migraine therapy. Both also show a correlation with clinical headache features. However, their sensitivity to detect clinically significant changes over time is not clear.

The SF-36, a general HRQoLmeasure and several migraine-specific HRQoL instruments are useful endpoints for migraine clinical trials. Their role in clinical practice is yet to be established.

A systematic review of the evidence pertaining to methylprednisolone infusion following acute spinal cord injury was conducted in order to address the persistent confusion about the utility of this treatment.

A committee of neurosurgical and orthopedic spine specialists, emergency physicians and physiatrists engaged in active clinical practice conducted an electronic database search for articles about acute spinal cord injuries and steroids, from January 1, 1966 to April 2001, that was supplemented by a manual search of reference lists, requests for unpublished additional information, translations of foreign language references and study protocols from the author of a Cochrane systematic review and Pharmacia Inc. The evidence was graded and recommendations were developed by consensus.

One hundred and fifty-seven citations that specifically addressed spinal cord injuries and methylprednisolone were retrieved and 64 reviewed. Recommendations were based on one Cochrane systematic review, six Level I clinical studies and seven Level II clinical studies that addressed changes in neurological function and complications following methylprednisolone therapy.

There is insufficient evidence to support the use of high-dose methylprednisolone within eight hours following an acute closed spinal cord injury as a treatment standard or as a guideline for treatment. Methylprednisolone, prescribed as a bolus intravenous infusion of 30 mg per kilogram of body weight over fifteen minutes within eight hours of closed spinal cord injury, followed 45 minutes later by an infusion of 5.4 mg per kilogram of bodyweight per hour for 23 hours, is only a treatment option for which there is weak clinical evidence (Level I- to II-1). There is insufficient evidence to support extending methylprednisolone infusion beyond 23 hours if chosen as a treatment option.

Proof from randomized controlled trials that carotid endarterectomy (CEA) is efficacious in stroke prevention has resulted in a large resurgence of its use in recent years. We wished to determine if patients in our region were being selected and treated with complication rates consistent with the randomized trials.

We have completed four audits of CEAs performed in our region since 1994, each followed by feed-back of results to the participating surgeons. Operations for > 70% symptomatic stenosis were considered appropriate, those for 50%-69% symptomatic and > 60% asymptomatic stenosis were considered uncertain and all others, including those in medically or neurologically unstable patients, were designated inappropriate. In part 4, the referral source and nature of the patients was also determined.

Part 1 (April 1994 - September 1995) found that of 291 CEAs performed 33% were appropriate, 48% were uncertain and 18% were inappropriate, and 40% of patients who underwent CEA were asymptomatic. In part 2 (September 1996 - September 1997) appropriate indications significantly improved to 49% of 184 CEAs (P=0.005), uncertain indications remained nearly the same at 47%, inappropriate indications fell to 4% (P=0.00002), and asymptomatic patients remained at 40%. The results of part 3 (October 1997 - October 1998) remained nearly the same as part 2 (249 CEAs, 47% appropriate, 51% uncertain, 2% inappropriate, 45% asymptomatic). Part 4 (October 1999 - October 2000) results were significantly better than part 3, appropriate indications increasing from 47% to 58% of 222 CEAs (P=0.02), and an elimination of inappropriate operations (P=0.03). Stroke and death complications declined over the study period from an overall rate of 5.2% in part 1 to 2.3% in part 4. In part 4 the majority of patients (69%) were referred to surgeons directly from general practitioners, including 58 (73%) of the 80 asymptomatic patients who underwent CEA.

Regular auditing and feedback of results and information to surgeons has resulted in significant and continued improvements in the surgical performance of CEAin our region. Since the majority of patients are referred directly to surgeons by general practitioners, it is important that this group of physicians be familiar with current CEA guidelines.

La fréquence de la dépression au cours de la maladie de Parkinson (MP) conduit à s’interroger sur les difficultés du diagnostic et sur les liens entre la dépression et l’histoire naturelle de la maladie.

Une série consécutive de 135 sujets atteints d’une MPidiopathique a été examinée sur le plan psychiatrique (DSM-III-R, échelle de dépression de Goldberg), neurologique (en distinguant les signes “axiaux” des autres signes parkinsoniens), neuropsychologique ( et en particulier tests frontaux).

Plus de la moitié des sujets ont une dépression qui apparaît plus fréquente dans les formes akinétiques et les formes fluctuantes de la maladie. Les sujets déprimés n’ont pas de déficit cognitif plus marqué mais leurs scores aux tests frontaux sont plus élevés. En outre les signes axiaux de la maladie (instabilité posturale, rigidité axiale) sont plus marqués chez les parkinsoniens déprimés, suggérant un lien entre la dépression et les lésions non dopaminergiques de la maladie. Alors que le ralentissement, les troubles de l’appétit et du sommeil, la fatigue peuvent être observés même chez des parkinsoniens non dépressifs, la séparation de la population parkinsonienne en plusieurs groupes montre que certains symptômes ne sont jamais rencontrés chez les parkinsoniens non dépressifs: il en est ainsi apparu que “l’impression que la vie ne vaut pas la peine d’être vécue”, “la perte d’espoir pour l’avenir”, “l’impression de ne pas être à la hauteur, de ne rien faire de bien”, “la baisse d’énergie”, “la tristesse matinale” caractérisent la dépression parkinsonienne. La dépression parkinsonienne revêt deux grandes formes cliniques. La première est la plus riche en manifestations somatiques: troubles du sommeil, fatigue matinale; elle correspond aux dépressions les plus sévères avec une absence d’espoir pour l’avenir , une perte de confiance en soi. La seconde est pauvre en manifestations somatiques alors que l’apathie y est très fréquente ainsi que le ralentissement.

Cette étude permet ainsi de préciser les symptômes de la dépression parkinsonienne qui doit être mieux reconnue pour être plus souvent traitée. Les liens entre la dépression et les signes axiaux de la maladie expliquent sans doute pourquoi la L-dopa et les agonistes dopaminergiques améliorent les signes moteurs de la dépression sans agir le plus souvent sur les manifestations dépressives.

In a formal needs assessment, conducted prior to the Canadian Headache Society's recent national continuing education workshop, participants expressed particular enthusiasm for enhancing their own communication skills or their teaching of those skills.

Responding to both interests, this paper offers a practical conceptual framework for thinking systematically about how to improve physician-patient communication to a professional level of competence.

The three-part, evidence-based framework first defines communication in medicine in terms of five underlying assumptions about communication and the learning of communication skills. It then discusses three categories of communication skills (content, process, and perceptual skills) and six goals that physicians and patients work to achieve through their communication with each other. The second part of the framework explores “first principles” of effective communication and includes a brief look at the historical context that has significantly influenced our thinking about, and practice of communication in health care. Part three of the framework describes one approach for delineating and organizing the specific skills that research supports for communicating effectively with patients - the Calgary Cambridge Guide.

It is clear from the literature that better physician communication skills improve patient satisfaction and clinical outcomes and that good communication skills can be taught and learned.

It is important that physicians learn the principles of good physician-patient communication and apply them in clinical practice. Medical education programs at all levels should include teaching of physician-patient communication.

Primary central nervous system lymphoma (PCL) is more frequently encountered by neurosurgeons given the increasing incidence among both nonimmunocompromised and immunocompromised patients. The most frequent surgery is stereotactic biopsy. Historically, radiation therapy has been the standard treatment modality for this disease and median survival was in the 15-month range. More recently, multi-modality therapy combining radiation therapy with chemotherapy (systemic, intrathecal, and/or intra-arterial) have resulted in longer survivals. We reviewed survival data for our series of patients treated for PCL over the last decade.

Thirty-four patients with histologically confirmed PCL were treated at our center. Multivariate Cox regression analysis was performed to determine which factor(s) (age, gender, HIV status, Karnofsky Performance Scale, chemotherapy, single modality therapy, histology, location, number of lesions, surgical resection) had a significant impact on survival.

The overall median survival was 19 months. Patients receiving multi-modality therapy (n=17) (chemotherapy and radiation) had a median survival of 34 months compared to four months for patients receiving single modality therapy (n=17 including seven HIV positive patients). Multi-modality therapy was the only significant factor affecting survival in this multivariate analysis (p<0.0001).

Chemotherapy plus radiotherapy significantly enhances survival over patients treated with single modality therapy alone. Quality of life issues should be addressed on a case by case basis as additional treatment modalities are initiated.

Fusiform cerebral aneurysms are dilatations of the entire circumference of a segment of cerebral artery, usually considered due to atherosclerosis in adults. They are relatively thick-walled and elongated, causing neural compression or ischemia when discovered. We have noted a subset of fusiform cerebral aneurysms that vary from this common description.

Out of a series of 472 intracranial aneurysms treated over 11 years, 11 patients between the ages 16 and 67 years (mean age 37) were identified who had discrete fusiform aneurysms unassociated with generalized cerebral atherosclerosis, connective tissue disorder or inflammation. Three presented with hemorrhage, six with neural compression by the aneurysm and two were discovered incidentally.

Nine aneurysms were located in the posterior circulation, the other two in the intracranial carotid artery. Their mean length and width were 16.3 and 11 mm, respectively. Three aneurysms contained thrombus. The eight aneurysms that were exposed surgically were partly or substantially thin-walled with normal appearing parent arteries. Eight were treated with proximal occlusion and three were circumferentially “wrapped”. Parent artery occlusion caused one death and one mild disability and the remaining patients made good recoveries (follow-up 0.5 - 10 years).

There is a subset of cerebral aneurysms with discrete fusiform morphology, apparently unrelated to cerebral atherosclerosis or systemic connective tissue disease, thin-walled in part or whole, more common in the vertebrobasilar system, and possessing a risk of rupture. Treatments currently available include proximal occlusion or aneurysm “wrapping”, different approaches than neck-clipping or endovascular coiling of side-wall saccular cerebral aneurysms that leave the parent artery intact.

Outcomes must be measured as a first step toward improving performance. We sought to measure the national and provincial outcomes from carotid endarterectomy (CE) and explain provincial differences.

We analyzed a large Canada-wide administrative hospital discharge database of all patients, except those in Québec, receiving CE in 1994-1997 and used logistic regression for risk adjustment to measure adverse outcomes nationally and by province. Our main outcome measures were in-hospital stroke and/or death.

A total of 14,268 patients underwent CE in the years 1994-1997. The overall death rate was 1.3% and the combined stroke and/or death rate was 4.1%. There was a trend towards improvement over the four years. The provinces of Saskatchewan and Newfoundland had significantly higher adverse event rates for the risk-adjusted combined outcome measure.

The outcome of CE in Canada is good and showed improvement over four years. However, significant differences in provincial outcomes were found. This suggests that regionalization across provincial boundaries may be needed to promote higher surgeon and hospital case volumes and thus improve outcomes.

To determine the practice patterns for methylprednisolone administration for patients with acute spinal cord injury (SCI) within the spinal surgery community across Canada, and the reasons behind these patterns.

Canadian neurological and orthopedic spine surgeons were surveyed at their respective annual meetings with a questionnaire asking seven questions with respect to their practice standards.

Sixty surgeons completed the survey representing approximately two-thirds of surgeons treating acute SCI within Canada. The NASCIS III dosing regimen is the most commonly prescribed steroid protocol. However, one-quarter of surgeons do not administer steroids at all. Of those who administer methylprednisolone, most do so because of peer pressure or out of fear of litigation.

The vast majority of spine surgeons in Canada either do not prescribe methylprednisolone for acute SCI, or do so for what might be considered the wrong reasons. These results demonstrate the need for an evidence-based practice guideline. The Candian Spine Society and the Canadian Neurosurgical Society fully endorse the recommendations of the steroid task force (see preceding paper).

Though various textbooks describe clinical manoeuvres that help detect subtle motor deficits, their sensitivity, specificity and predictive values have not been determined. We investigated the sensitivity, specificity and predictive values of various manoeuvres in order to determine the most sensitive and reliable test or combination thereof.

Straight arm raising (Barré), pronator drift, Mingazzini’s manoeuvre, finger tap, forearm roll, segmental strength and deep tendon reflexes were tested in 170 patients with (86) and without (84) a proven lesion in the motor areas confirmed by computed tomography.

Segmental motor strength had good specificity (97.5%) but poor sensitivity (38.9%) and negative predictive value (NPV) (58.7%). The forearm roll had a similar profile. Finger tap had a sensitivity of 73.3% and a specificity of 87.5%. Barré and pronator testing had a sensitivity and specificity of 92.2% and 90.0% respectively. Hyperreflexia had a sensitivity of 68.9% and a specificity of 87.5%. An abnormality of pronator, reflexes or finger tap had a sensitivity of 97%, and when these three tests were positive, specificity was 97%. When all six tests were positive, the positive predictive value was 100%, when all six tests were negative the NPV was 100%.

The detailed segmental examination has very good specificity for detecting motor deficits, but the sensitivity and NPV are unacceptably low. Pronator drift with finger tap and reflexes is the most reliable and time-effective combination of tests for the detection of subtle motor lesions, and could replace the segmental motor examination as a screening for motor lesions.

Few data are available about the diagnostic yield of the lactate stress test (LST) in a large group of patients with mitochondriopathy (MCP).

Serum lactate was determined once before, three times during, and once after a 15-minute, constant 30W workload on a bicycle in 62 controls, aged 17 to 84 years, 155 patients with MCP, aged 17 to 87 years, and 31 patients with neurological disorders other than MCP.

Lactate's upper reference limits at rest, 5, 10, 15 minutes after starting, and 15 minutes after finishing the exercise were 2.0, 2.1, 2.1, 2.1 and 1.8 mmol/l respectively. The test was regarded abnormal if more than two of the five lactate values exceeded the cut-off levels. Among the 103 patients with abnormal LST, 64 (62 %) had normal resting lactate. The sensitivity of the test was 67% and the specificity 94%.

The LST proved to have a high sensitivity and specificity in the detection of patients with MCP, being thus a simple but powerful tool to assess the impaired oxidative metabolism in MCP patients.