Background: Status dystonicus (SD) is a life-threatening form of dystonia with limited treatments available. We sought to better understand the processes, outcomes, and complications of deep brain stimulation (DBS) for pediatric SD through a systematic review alongside an institutional case series. Methods: Data regarding treatment, stimulation parameters, dystonia severity and outcomes was collected for the case series (n=7) and systematic review (n=70, conducted in accordance with PRISMA guidelines). This was analysed descriptively (rates, outcome measures). For the case series we created probabilistic voxel-wise maps for improvement in dystonia based on brain region stimulated. Results: All patients in our case series and > 95% of patients in the systematic review had resolution of SD with DBS, typically within 2-4 weeks. Most patients in the review (84%) and all patients in the case series had DBS implanted to the globus pallidus internus. In terms of dystonia severity scores, there was a mean improvement of 25% (case series) or 49% (systematic review). Reported mortality was 4% in the systematic review. Conclusions: DBS for pediatric SD is feasible and safe. It allows for increased survival as well as quality of life - however risks still exist. More work is needed to determine timing, eligibility, and stimulation parameters.

We investigated the association between clinical rotation at a specialized headache center and headache knowledge of resident trainees. Using standardized pre- and post-questionnaires, change in self-reported knowledge of headache disorders and management in 31 participants undertaking clinical rotations were evaluated. There was a statistically significant improvement in self-reported measures of headache disorder knowledge post-rotation [mean score (SD), 3.19(0.543), p < 0.001] and significant improvement in overall knowledge measured using case-based questionnaires pre- vs. post-rotation [7.1(1.4) vs. 7.9(1.5), p = 0.003]. Rotation at a specialized headache center improved trainees’ self-reported knowledge and test-based scores, suggesting that such rotation should be included in postgraduate curriculum.

Background: Dystonia is common in children with acquired and inherited neurological disorders. Status dystonicus (SD) is the most severe form of dystonia that can lead to life-threatening complications if not treated promptly. We identified a local provider knowledge gap in the acute management of dystonia, leading to uncertainty and delays in care. To our knowledge, no in-hospital clinical pathway exists for the ward-based management of acute dystonia. We hypothesized that a stepwise clinical pathway would standardize and improve comfort in managing hyperacute dystonia. Methods: We formed a multidisciplinary working group and developed a pathway based on literature review and expert consensus. Aims of the pathway included: reducing delays in recognition and treatment of acute dystonia, limiting variation in management, and decreasing progression to SD. A survey was administered to providers assessing knowledge and comfort post-implementation. Results: There has been high usability with 58% (18/31) of providers surveyed having used the pathway at least once. Provider comfort has improved, with 89% (25/28) of respondents reporting increased comfort managing SD due to the clarity of the pathway and stepwise directions. Conclusions: The pathway fills a gap in the in-hospital management of dystonia and has led to increased provider comfort.

Background: Although palliative care (PC) is recommended for patients with amyotrophic lateral sclerosis (ALS), many patients receive PC very late or not at all. Our study goals included 1) determing the feasibility of early PC 2) describing patient/caregiver satisfaction with early PC and 3) measuring the impact of early PC on quality of life (QOL) and mood. Methods: Patients followed at the multidisciplinary ALS clinic in Ottawa, Canada and their caregivers were eligible for the study irrespective of duration or severity of disease. All participants completed questionnaires tracking QOL and mood and all were offered a palliative care consultation. Participants completed a satisfaction survey post-PC consultation. Results: 32 patients and 20 caregivers received a PC consultation, conducted virtually. All of them found the consult beneficial and none of the patients reported preferring the consultation later in their illness. The PC consultations were most highly rated by patients with high levels of anxiety and worse bulbar function, and by caregivers of patients with low function. There was no statistically significant change in mood or QOL compared to the 7 participants who declined PC consultation. Conclusions: PC consultations are feasible and beneficial at all stages of illness. Patients with anxiety and bulbar dysfunction may benefit most.