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Abstract Background Headache disorders are the most common neurological disorders worldwide. Despite their widespread prevalence and importance, the topic of headache is inconsistently taught at both the undergraduate and postgraduate levels. The goal of this study is to establish a better picture of the current state of Headache Medicine (HM) training in Neurology postgraduate programs in Canada and describe the impact of the current pandemic on training in this domain. Methods Online surveys were sent to senior residents of adult Neurology programs in Canada. We also conducted telephone interviews with Neurology Program Directors. Descriptive statistics were analyzed, and thematic analysis was used to review free text. Results A total of 36 residents, and 3 Program Directors participated in the study. Most of the teaching in HM is done by headache specialists and general neurology faculty. Formal teaching is mainly given during academic half day. Most of the programs expose their residents to Onabotulinum toxin A injections and peripheral nerve blocks, but they don’t offer much formal teaching regarding these procedures. Residents consider HM teaching important and they would like to have more. They don’t feel comfortable performing interventional headache treatments, despite feeling this should be part of the skillset of a general neurologist. Conclusion Our study is the first to establish the current state of headache teaching in post-graduate neurology programs as perceived by trainees and program directors in Canada. The current educational offerings leave residents feeling poorly prepared to manage headaches, including procedural interventions. There is a need to diversify the source of teaching, so the educational burden doesn’t lie mostly upon Headache specialists who are already in short supply. Neurology Residency programs need to adapt their curriculum to face the current need in HM.

Abstract Background Vertigo remains a diagnostic challenge for primary care, emergency, and specialist physicians. Multidisciplinary clinics are increasingly being employed to diagnose and manage patients with dizziness. We describe, for the first time in Canada, the clinical characteristics of patients presenting with chronic and acute dizziness to both a multidisciplinary chronic dizziness clinic (MDC) and a rapid access dizziness (RAD) clinic at The Ottawa Hospital (TOH). Methods We performed a retrospective review of all patients presenting to the MDC and RAD clinics at TOH from July 2015 to August 2017. Results Overall, 211 patients (median age: 61 years old) presented to the RAD clinic and 292 patients (median age: 55 years old) presented to the MDC. In the RAD clinic, 63% of patients had peripheral dizziness, of which 55% had BPPV, and only one patient had functional dizziness. Interestingly, only 25% of RAD diagnoses were concordant with emergency department diagnoses; moreover, only 33% of RAD patients had HiNTS completed, while 44% had CT scans, of which only one scan had an abnormal finding. Prior to assessment, all patients in the MDC had an unclear cause of dizziness. 28% of patients had vestibular dizziness and 21% had functional dizziness, of which 43% had persistent postural perceptual dizziness. Moreover, 12% of patients with functional dizziness also suffered from comorbid severe anxiety and depression. Conclusions Dizziness is a heterogeneous disorder that necessitates multidisciplinary care, and clinics targeting both the acute and chronic setting can improve diagnostic accuracy, ensure appropriate diagnostic testing, and facilitate effective care plans for patients with dizziness.

Abstract Background Dizziness is a common presenting symptom in the emergency department (ED). The HINTS exam, a battery of bedside clinical tests, has been shown to have greater sensitivity than neuroimaging in ruling out stroke in patients presenting with acute vertigo. The present study sought to assess practice patterns in the assessment of patients in the ED with peripherally-originating vertigo with respect to utilization of HINTS and neuroimaging. Methods A retrospective cohort study was performed using data pertaining to 500 randomly selected ED visits at a tertiary care centre with a final diagnostic code related to peripherally-originating vertigo between January 1, 2010 - December 31, 2014. Results A total of 380 patients met inclusion criteria. Of patients presenting to the ED with dizziness and vertigo and a final diagnosis of non-central vertigo, 139 (36.6%) received neuroimaging in the form of CT, CT angiography, or MRI. Of patients who did not undergo neuroimaging, 17 (7.1%) had a bedside HINTS exam performed. Almost half (44%) of documented HINTS interpretations consisted of the ambiguous usage of “HINTS negative” as opposed to the terminology suggested in the literature (“HINTS central” or “HINTS peripheral”). Conclusions In this single-centre retrospective review, we have demonstrated that the HINTS exam is under-utilized in the ED as compared to neuroimaging in the assessment of patients with peripheral vertigo. This finding suggests that there is room for improvement in ED physicians’ application and interpretation of the HINTS exam.

Abstract Background ATP8A2 mutations have recently been described in several patients with severe, early-onset hypotonia and cognitive impairment. The aim of our study was to characterize the clinical phenotype of patients with ATP8A2 mutations. Methods An observational study was conducted at multiple diagnostic centres. Clinical data is presented from 9 unreported and 2 previously reported patients with ATP8A2 mutations. We compare their features with 3 additional patients that have been previously reported in the medical literature. Results Eleven patients with biallelic ATP8A2 mutations were identified, with a mean age of 9.4 years (range 2.5–28 years). All patients with ATP8A2 mutations (100%) demonstrated developmental delay, severe hypotonia and movement disorders, specifically chorea or choreoathetosis (100%), dystonia (27%) and facial dyskinesia (18%). Optic atrophy was observed in 78% of patients for whom funduscopic examination was performed. Symptom onset in all (100%) was noted before 6 months of age, with 70% having symptoms noted at birth. Feeding difficulties were common (91%) although most patients were able to tolerate pureed or thickened feeds, and 3 patients required gastrostomy tube insertion. MRI of the brain was normal in 50% of the patients. A smaller proportion was noted to have mild cortical atrophy (30%), delayed myelination (20%) and/or hypoplastic optic nerves (20%). Functional studies were performed on differentiated induced pluripotent cells from one child, which confirmed a decrease in ATP8A2 expression compared to control cells. Conclusions ATP8A2 gene mutations have emerged as the cause of a novel neurological phenotype characterized by global developmental delays, severe hypotonia and hyperkinetic movement disorders, the latter being an important distinguishing feature. Optic atrophy is common and may only become apparent in the first few years of life, necessitating repeat ophthalmologic evaluation in older children. Early recognition of the cardinal features of this condition will facilitate diagnosis of this complex neurologic disorder.