Highlights • Cannabidiol (CBD) was used in convulsive status epilepticus and was not effective. • Portrayal of benefits of CBD in main stream and social media was often unrealistic. • Ethics ...committee was useful in negotiating a treatment pathway involving CBD.
Description A 51-year-old woman with no significant medical history was found collapsed at work having sustained a head strike. The patient was able to be discharged home after a short period of ...rehabilitation with persisting restricted eye movements but no significant functional impairment. Duret haemorrhage, also known as secondary brainstem haemorrhage, occurs as a result of transtentorial herniation of the brain in the context of acute trauma (eg, subdural haematoma).1 2 It is hypothesised to occur as a result of the downward pressure shearing small mobile blood vessels within the brainstem from the relatively immobile basilar artery.2 Surgical decompression can also result in haemorrhage from reperfusion injury.2 Due to their location, there is an association with poor prognosis of death or severe neurological disability, which often results in earlier withdrawal of life-sustaining therapies.2 Our case highlights the caution required when assessing prognosis in patients with Duret haemorrhage.
Genomic technologies such as next-generation sequencing (NGS) are revolutionizing molecular diagnostics and clinical medicine. However, these approaches have proven inefficient at identifying ...pathogenic repeat expansions. Here, we apply a collection of bioinformatics tools that can be utilized to identify either known or novel expanded repeat sequences in NGS data. We performed genetic studies of a cohort of 35 individuals from 22 families with a clinical diagnosis of cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS). Analysis of whole-genome sequence (WGS) data with five independent algorithms identified a recessively inherited intronic repeat expansion (AAGGG)exp in the gene encoding Replication Factor C1 (RFC1). This motif, not reported in the reference sequence, localized to an Alu element and replaced the reference (AAAAG)11 short tandem repeat. Genetic analyses confirmed the pathogenic expansion in 18 of 22 CANVAS-affected families and identified a core ancestral haplotype, estimated to have arisen in Europe more than twenty-five thousand years ago. WGS of the four RFC1-negative CANVAS-affected families identified plausible variants in three, with genomic re-diagnosis of SCA3, spastic ataxia of the Charlevoix-Saguenay type, and SCA45. This study identified the genetic basis of CANVAS and demonstrated that these improved bioinformatics tools increase the diagnostic utility of WGS to determine the genetic basis of a heterogeneous group of clinically overlapping neurogenetic disorders.
Decompressive hemicraniectomy (DHC) has been shown to reduce mortality in malignant middle cerebral artery (MCA) infarction. Our primary objective was to compare 1‐year mortality between patients ...receiving DHC for malignant MCA infarction at our institution based on hospital of origin. We retrospectively reviewed the medical records of all patients treated for malignant MCA infarction with DHC at our institution over a 3‐year period. One‐year mortality rates and time to surgery were comparable regardless of whether the patient first attended the tertiary referral centre or a peripheral centre.
Background
Telestroke uses videoconferencing technology to allow off‐site experts to provide stroke thrombolysis decision support to less experienced front line clinicians.
Aim
To assess the impact ...of a new telestroke service on thrombolysis rates and door‐to‐needle times in participating provincial hospitals and service resources to aid transition to a sustainable telestroke service.
Methods
This is a sequential comparison of ‘pre’ (December 2015 to May 2016) and ‘post’ (June 2016 to December 2016) implementation outcomes. The main outcomes were thrombolysis rate and door‐to‐needle time. All patient data were captured prospectively in a central database. Data captured and analysed also included technical problems, consumer and clinician feedback, and additional service resources required.
Results
Over the study period, 164 telestroke assessments were completed, including the ‘hub’ hospital. Among the participating provincial hospitals, 21 of 343 patients (6.1%) were thrombolysed in the 6‐months prior to June 2016 and 50 of 318 patients (15.7%) during the 6‐month following implementation of telestroke; odds ratio 2.86 (95% confidence interval 1.68–4.89); P = 0.0001. Overall, mean (standard deviation) regional hospital door‐to‐needle time reduced from 79.6 (31.4) to 62.7 (23.3) min (P = 0.015). Videoconferencing failure occurred in 4.8% of cases. Consumer and clinician feedback was positive. The main resource challenge was doubling of out‐of‐hours neurologist workload.
Conclusion
Telestroke was associated with a significant increase in thrombolysis rate and reduction in door‐to‐needle time in provincial hospitals indicating improved patient care. Quantification of the extra neurologist workload allowed for a seamless transition to ‘business as usual’ using a novel annual subscription funding and service model.
This study investigated the demographic and disease characteristics of motor neurone disease (MND), as well as specific aspects of healthcare provision for MND patients in the greater Wellington ...region.
Data was collected from clinical records of all known patients with a diagnosis of Motor Neurone Disease MND in the Capital and Coast and Hutt Valley district health board catchment zones over a 12-month period. Survival data was collected at 2-year follow-up.
The study population consisted of 40 patients. The mean age at diagnosis was 66.2 years (SD of 13.2). Thirty patients (75%) were New Zealand European. Predominantly limb signs were present in 12 (30%), and 11 (27.5%) had bulbar signs, while 14 (35%) had a mixed pattern and a further 3 (7.5%) had respiratory muscle weakness. At 2-year follow-up, the median survival time following symptom onset in the 32 deceased patients was 29 months (range 6-126 months).
The demographics and disease characteristics of MND in this cohort of patients from the Wellington region is similar to those found in overseas studies. The majority of patients received care in accordance with established guidelines, although certain aspects of healthcare delivery could be further improved.
Abstract Distinguishing between generalized tonic-clonic seizures (GTCSs) and convulsive psychogenic nonepileptic seizures (PNESs) can be difficult at the bedside, and this distinction has important ...implications for patient care. This study used a fully blinded method to examine postictal breathing parameters to identify features distinguishing between generalized tonic-clonic seizures (GTCSs) and convulsive psychogenic nonepileptic seizures (PNESs). Three blinded readers examined edited video recordings of the postictal phase of 72 convulsive seizure episodes recorded from 56 patients. There were 59 GTCS episodes and 13 PNES episodes. Postictal breathing after a PNES episode was more rapid than after a GTCS episode and, thereafter, normalized more rapidly. Postictal breathing after a GTCS episode was more likely to be characterized by stertorous respirations. Postictal breathing after a PNES episode was very unlikely to be characterized by stertorous respirations. Postictal respiratory pattern can assist in discriminating between GTCS and convulsive PNES.
We present a qualitative evaluation of a clinical orientation program for medical registrars within the Wellington region in New Zealand, designed and implemented by current advanced registrars. This ...program was intended to improve the transition from house officer to medical registrar. The program was qualitatively evaluated using focus groups comprising participants, presenters and senior nursing staff. Purposive samples were drawn from each of these groups. The most significant finding was the perception of enhanced professional collegiality among medical staff. There were benefits to participants and presenters with improved communication between medical registrars. We believe there are individual, institutional and patient care benefits with a region-specific, clinical orientation for new medical registrars.
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