Kevin Busche

Acute Necrotizing Encephalopathy in Caucasian Children: Two Cases and Review of the Literature

Acute necrotizing encephalopathy is a fulminant neurologic disease seen predominantly in Japan and Taiwan. We present two cases diagnosed at a Canadian center within the same year in Caucasian children. Both were previously well, developed an acute viral illness with fever and vomiting, and progressed to brain death within 2 to 4 days. Neuroimaging and postmortem examination demonstrated the unique features of bilateral and severe necrosis of deep gray-and subcortical white-matter structures. The first case was associated with extensive, but transient, hepatic involvement, recent varicella and rotavirus infections, and detailed metabolic studies, including mitochondrial functional analysis, were normal. The second case tested positive for influenza A infection, whereas evidence of liver damage was lacking. Both children demonstrated early lymphopenia and myocardial necrosis, two features not previously associated with acute necrotizing encephalopathy. These cases are unique in their occurrence in non-Japanese children and are among the first published reports in Canada. (J Child Neurol 2005;20:527-532).

Long-Term Persistence with Injectable Therapy in Relapsing-Remitting Multiple Sclerosis: An 18-Year Observational Cohort Study

Disease modifying therapies (DMTs) reduce the frequency of relapses and accumulation of disability in multiple sclerosis (MS). Long-term persistence with treatment is important to optimize treatment benefit. This long-term, cohort study was conducted at the Calgary MS Clinic. All consenting adults with relapsing-remitting MS who started either glatiramer acetate (GA) or interferon-β 1a/1b (IFN-β) between January 1st, 1996 and July 1st, 2011 were included. Follow-up continued to February 1st, 2014. Time-to-discontinuation of the initial and subsequently-prescribed DMTs (switches) was analysed using Kaplan-Meier survival analyses. Group differences were compared using log-rank tests and multivariable Cox regression models. Analysis included 1471 participants; 906 were initially prescribed GA and 565 were initially prescribed IFN-β. Follow-up information was available for 87%; 29 (2%) were lost to follow-up and 160 (11%) moved from Southern Alberta while still using DMT. Median time-to-discontinuation of all injectable DMTs was 11.1 years. Participants with greater disability at treatment initiation, those who started treatment before age 30, and those who started between 2006 and 2011 were more likely to discontinue use of all injectable DMTs. Median time-to-discontinuation of the initial DMT was 8.6 years. Those initially prescribed GA remained on treatment longer. Of 610 participants who discontinued injectable DMT, 331 (54%) started an oral DMT, or a second-line DMT, or resumed injectable DMT after 90 days. Persistence with injectable DMTs was high in this long-term population-based study. Most participants who discontinued injectable DMT did not remain untreated. Further research is required to understand treatment outcomes and outcomes after stopping DMT.

Neurologic Disorders Associated with Weight lifting and Bodybuilding

Weight lifting and other forms of strength training are becoming more common because of an increased awareness of the need to maintain individual physical fitness. Emergency room data indicate that injuries caused by weight training have become more universal over time, likely because of increased participation rates. Neurologic injuries can result from weight lifting and related practices. Although predominantly peripheral nervous system injuries have been described, central nervous system disease may also occur. This article illustrates the types of neurologic disorders associated with weight lifting.

Making progress in the ethical treatment of medical trainees

There is an inherent conflict within clinician educators as we balance the roles of healthcare provider to patients in need of care with that of educator of learners in need of teaching. In this essay we use Beauchamp and Childress’ principles of biomedical ethics as a framework to compare the relationship that clinician educators have with their patients and their learners, and suggest that while we typically apply ethical principles when addressing the needs of our patients, these principles are frequently lacking in our interactions with learners. This dichotomy reflects a person-by-situation interaction that may be partly explained by the expectations of the regulatory bodies that define how clinicians should interact with patients and how educators should interact with learners. The result is that we may fall short in applying respect for autonomy, beneficence/nonmaleficence, and justice when addressing the needs of our learners. Fortunately there are ways in which we can incorporate these ethical principles into our interactions with learners while still adhering to accreditation standards and institutional policy. These include flipped classrooms and simulated learning experiences, incorporating aspects of instructional design that have been shown to improve learning outcomes, providing additional resources to learners with greater needs, and organizing training curricula around entrustable professional activities. Although the consistent application of ethical principles with all learners during all learning experiences is likely unachievable, we can, and should, move towards more ethical treatment of our learners.

Sphenoid sinus mucocele--rupture causing brainstem inflammation and stroke.

BACKGROUND Posterior rupture of a sphenoid sinus mucocele is a rare cause of brainstem injury. METHODS Case report. RESULTS A healthy young woman with a history of prior surgical excision of nasal polyps presented with a headache and evolving neurologic symptoms. The clinical presentation and imaging studies were consistent with a posterior rupture of a large sphenoid mucocele with subsequent inflammatory changes in the brainstem and evidence of brainstem stroke. Relatively rapid recovery coincided temporally with the resolution of local inflammation. Longer-term recovery continued over months, as expected following stroke. CONCLUSIONS Posterior rupture of a sphenoid sinus mucocele may cause brainstem injury by multiple mechanisms.

A patient with bilateral sciatic neuropathies.

position in a floor-level cupboard with a depressed level of consciousness after ingesting an overdose of amitriptyline and alcohol the night before. She was transferred to hospital and treated for tricyclic antidepressant overdose and was found to have rhabdomyolysis. Her creatine kinase peaked at 70 000 units/L. Within 24 hours after admission, the patient recovered her normal level of consciousness and complained of bilateral hip, groin and buttock pain exacerbated by movement. She also had weakness, paresthesias and numbness in both lower extremities, more pronounced on the right. Following initial consideration of other possible causes, Neurology was consulted. Her examination demonstrated normal cranial nerves and upper extremity function. The patient had pain with passive flexion of the hips. There was bilateral weakness of leg extension (2/5), hip abduction (3/5), knee flexion (3/5), ankle dorsiflexion (4-/5), plantar flexion (4-/5), foot inversion (4-/5), foot eversion (4-/5), toe dorsiflexion (4-/5), and toe plantar flexion (4-/5), with each slightly worse for the right leg. Deep tendon reflexes were absent at both ankles but present and symmetric elsewhere, including at the knees and adductor muscles. Plantar responses were flexor. Sensory exam revealed decreased pinprick sensation over the right foot and the lateral lower right leg, along with minimally decreased pin prick sensation over the left sole and medial aspect of the left foot. On day seven of admission, an enhanced Computed Tomography (CT) scan of the pelvis revealed moderately large areas of low attenuation in the gluteus muscles bilaterally, right greater than left (see Figure). The normal fat planes surrounding the sciatic nerve in the sciatic notch (figure, arrows) are obscured by low attenuation fluid or hematoma. The low attenuation is rounded and more prominent on the left than on the right. The low attenuation fluid or hematoma may reflect tracking from the gluteal muscle or direct nerve injury. The findings were consistent with areas of muscle injury, infarct or hematoma due to pressure necrosis and reflected the degree of pressure injury to the gluteal and buttock area. No compartment syndrome was felt to be present by either ICU specialists or surgical consultants. Surgical decompression was considered but not performed. An ultrasound of the lower extremities was negative for deep venous thrombosis. The patient was discharged 11 days after admission, using a wheelchair to mobilize. The patient returned for nerve conduction studies nine weeks after her initial presentation. At that time she was taking gabapentin and oxycodone for neuropathic pain over both distal legs. She required a cane to ambulate and wore a right ankle

How teachers can help learners build storage and retrieval strength

Abstract Aim: To be an effective teacher, content expertise is necessary but alone does not guarantee optimal learning outcomes for students. In this article, the authors discuss ways in which medical teachers can shape the learning of their students and enable them to become more efficient and effective learners. Methods: Using Bjork and Bjorks new theory of disuse as their framework, the authors discuss strategies to improve storage strength of to-be-learned information and strategies to improve retrieval strength of learned information. Results: Strategies to improve storage strength include optimizing cognitive load, providing causal explanations, and giving effective feedback. Strategies to improve retrieval strength include situated cognition and various types of retrieval practice. Conclusions: Adopting these teaching strategies should hopefully help teachers improve the learning outcomes of their students, but there is still a need for further research into the science of learning and the science of instruction, including comparative effectiveness of different teaching strategies and how best to translate findings from the psychology literature into medical education.

Weighing the Cost of Educational Inflation in Undergraduate Medical Education.

Despite the fact that the length of medical school training has remained stable for many years, the expectations of graduating medical students (and the schools that train them) continue to increase. In this Reflection, the authors discuss motives for educational inflation and suggest that these are likely innocent, well-intentioned, and subconscious—and include both a propensity to increase expectations of ourselves and others over time, and a reluctance to reduce training content and expectations. They then discuss potential risks of educational inflation, including reduced emphasis on core knowledge and clinical skills, and adverse effects on the emotional, psychological, and financial wellbeing of students. While acknowledging the need to change curricula to improve learning and clinical outcomes, the authors proffer that it is naïve to assume that we can inflate educational expectations at no additional cost. They suggest that before implementing and/or mandating change, we should consider of all the costs that medical schools and students might incur, including opportunity costs and the impact on the emotional and financial wellbeing of students. They propose a cost-effectiveness framework for medical education and advocate prioritization of interventions that improve learning outcomes with no additional costs or are cost-saving without adversely impacting learning outcomes. When there is an additional cost for improved learning outcomes or a decline in learning outcomes as a result of cost saving interventions, they suggest careful consideration and justification of this trade-off. And when there are neither improved learning outcomes nor cost savings they recommend resisting the urge to change.