Neurology

An 81-year-old woman presented with subacute chorea as a paraneoplastic neurological syndrome

Rebecca Nothrop · Will Lee · Denise Lee · Amanda K Gilligan

Australian services for people with dementia are fragmented, challenging to navigate and hard to access The coronavirus disease 2019 (COVID‐19) pandemic has led to reflections around reforming Australia’s health care system.1 In view of future reforms, this article is intended to provoke policy and clinical discussion regarding what an effective, efficient model of service delivery meeting the needs of people with dementia and their families may look like. The opinion presented here belongs to the members of the National Health and Medical Research Council (NHMRC) National Institute for Dementia Research Special Interest Group in Rehabilitation and Dementia. For the purposes of this article, we define a model of service delivery as the systemic framework through which services are organised, accessed, funded and delivered. Services in Australia for people with dementia are inadequate Dementia is the leading cause of disability, the second leading cause of death in Australians aged over 65 years, and the leading cause of death in women in Australia. In 2020, it is estimated that Australia will spend $8.1 billion on health care and $3.8 billion on social services for people with dementia, with a further $6.1 billion in lost productivity and earnings.2 Australian services for people with dementia are often fragmented, challenging to navigate and hard to access.3 It can be difficult for people with dementia to obtain a diagnosis, there are limited health and social services for early dementia, including post‐diagnostic support, and existing services are often poorly coordinated.3,4 Services face workforce shortages and gaps in worker knowledge and skills related to dementia.5 People with dementia and their care partners have called for support and information after diagnosis; flexibly delivered services that support their quality of life, including meaningful activity; and inclusion in decision making.6 A philosophical and societal shift in thinking is required: from provision of care to enablement, where people living with dementia are empowered to continue to direct their own lives.7 We are not meeting the human rights of people with dementia to health care Australia does not currently meet the human rights of people with dementia to timely and accessible health services of appropriate quality or to participation in health care decisions.4,6 The right to quality health care is affected by the variable delivery of best‐practice dementia care by memory clinics,8 acute hospitals,9 primary care,10 and community and residential aged care,11 perhaps because the role of each of these is unclear. Australia’s systems and context Australia has a long‐standing commitment to a universal health system and to long term care for older people. The health and aged care systems were developed largely in isolation from one another and have failed to resolve conflicts around medical and social models of care for older people. Health care systems are slowly adapting to this era of chronic disease and population ageing,12 but person‐centredness and integration within and across acute, primary, community and residential aged care systems remain a challenge.11 Principles underpinning models of service delivery for Australians with dementia Members of our group reviewed principles underpinning services such as the Department of Health Aged Care Sector and the Council of Australian Governments National Disability Insurance Scheme. 13,14 We reached a consensus that the following principles should apply to models of service delivery for dementia that: has an overarching objective to maintain positive health and wellbeing of people with dementia, their care partners and families; recognises dementia as a disability, consistent with the World Health Organization Convention on the Rights of Persons with Disabilities, and promotes autonomy, social participation and rehabilitation; takes into account the cognitive disability of people with dementia in accessing support and being a partner (along with their families) in planning care through supported decision making; is delivered by a multidisciplinary workforce who have knowledge and skills around dementia; is accessible for all people with dementia and care partners; is ongoing, cost‐effective and economically sustainable; is needs‐based, not capped according to central budgets; is integrated for seamless experience for people with dementia and care partners, within and across primary, acute and subacute health care, aged care and social services; and is evidence‐based. Review of possible models of service delivery for dementia We identified models of service delivery for dementia and other chronic conditions based on input from our broad authorship group and searching the peer‐reviewed and grey literature. These models are described in the and considered in terms of fit with the principles above. We included care pathways even though these are not a model because they are often used to improve service access and integration. In addition, we map the models of service delivery to our health and aged care funding systems, illustrating the limited integration across systems (Box). Learnings from these models: The self‐directed approach places the needs of the person with dementia centrally but may require processes to ensure supported decision making. Information is also needed regarding the risks and benefits of self‐management versus budget holding or service provider management, integration with health care, and consideration of costs. Case management improves outcomes for the person with dementia and could be flexible and needs‐based if sufficient workforce and integration across systems could be achieved. However, it would require a significant investment of resources. Strengths of the primary care chronic disease management model include equity and familiarity of access, and care coordination by a trusted health professional or practice team. Weaknesses include the limited amount of treatment (ie, current cap of five subsidised allied health consultations per year), limited dementia management skills in some general practitioners and practice nurses, and often poor integration with aged care. Shared and stepped care models may be able to be adapted to combine the strengths of the primary care chronic disease and specialist approaches, but integration of aged care services would be essential. Stepped care may not be the best fit for people diagnosed with dementia in other settings (eg, hospitals or residential care facilities). A specialist team approach with a skilled workforce is well equipped to provide evidence‐based care, although this is unlikely to be made universally accessible (eg, in regional areas) and may be cost‐prohibitive. Navigator and care pathway approaches may increase access to services, but do not improve the type or amount of supports or treatment available. None of the models of service delivery that we identified in Australia or overseas appear to sufficiently meet the principles above. There is no clear recognition that dementia is both a social and a medical issue. Australia has moved strongly in the direction of recognising the rights of people with disabilities including social participation but there is limited appreciation of this need in respect to most models for dementia. Recognition of dementia as a disability is only apparent in the self‐directed care model. The models also do not sufficiently consider the needs of the person with dementia and care partners together. Barriers to all the current models are the poor dementia knowledge and the tendency to stigmatise people with dementia by many health and aged care professionals.15 Next step: investment in model development We need to combine desirable elements in the primary care chronic disease management, case management, and specialist multidisciplinary care models. Having a system with a point of entry through primary care could maximise accessibility. Having a dementia and aged care specialist (eg, dementia nurse or case manager) working with GPs would bring the required skills and knowledge. A close partnership with a specialist multidisciplinary team (in person or using telehealth) would assist with diagnosis, ongoing support and management of complex cases, with possibly the most complex cases being managed by the specialist team. There needs to be investment to develop a model that is accessible, integrated and effective in meeting the needs of people with dementia. Our service delivery model needs to be co‐designed with people with dementia, their care partners, health, aged care, and state and federal government stakeholders, including treasury departments. Public health, social equity and human rights principles should underpin model design. Research is needed to explore proposed models and their elements with current recipients, service planners and providers. Methodologies may include service mapping; gap, risk and unintended consequence analysis; and economic modelling. Potential models will then need to be tested in a coordinated series of pilots and rigorous health system trials building towards national implementation. History has shown that piecemeal demonstration pilots and practice improvement projects will not bring about large‐scale change. Australia’s last National Framework for Action on Dementia 2015–2019 has just lapsed.16 Our new framework should include the development of a model of service delivery that considers accessible pathways to diagnosis and effective and seamless ongoing support of health and wellbeing throughout the course of dementia. Box – Current service funding structures and service models for Australians with dementia GPs = general practitioners; NDIS = National Disability Insurance Scheme; NGOs = non‐government organisations; PHNs = primary health networks.

NHMRC National Institute for Dementia Research Special Interest Group in Rehabilitation and Dementia

It was mid-morning, halfway through ward round

Jillian Chau

Recent proposals to adopt tenecteplase as the recommended thrombolytic agent for stroke reperfusion will reduce its availability for patients with acute myocardial infarction

Mark Parsons · Leonid Churilov · Aletta E Schutte · Christopher Levi

Technology is revolutionising medicine, and telemedicine is having a measurable impact on stroke care

Richard I Lindley

Objectives: To evaluate the impact of the Victorian Stroke Telemedicine (VST) program during its first 12 months on the quality of care provided to patients presenting with suspected stroke to hospitals in regional Victoria. Design: Historical controlled cohort study comparing outcomes during a 12‐month control period with those for the initial 12 months of full implementation of the VST program at each hospital. Setting: 16 hospitals in regional Victoria that participated in the VST program between 1 January 2010 and 30 January 2016. Participants: Adult patients with suspected stroke presenting to the emergency departments of the participating hospitals. Main outcome measures: Indicators for key processes of care, including symptom onset‐to‐arrival, door‐to‐first medical review, and door‐to‐CT times; provision and timeliness of provision of thrombolysis to patients with ischaemic stroke. Results: 2887 patients with suspected stroke presented to participating emergency departments during the control period, 3178 during the intervention period; the patient characteristics were similar for both periods. A slightly larger proportion of patients with ischaemic stroke who arrived within 4.5 hours of symptom onset received thrombolysis during the intervention than during the control period (37% v 30%). Door‐to‐CT scan time (median, 25 min [IQR, 13–49 min] v 34 min [IQR, 18–76 min]) and door‐to‐needle time for stroke thrombolysis (73 min [IQR, 56–96 min] v 102 min [IQR, 77–128 min]) were shorter during the intervention. The proportions of patients who received thrombolysis and had a symptomatic intracerebral haemorrhage (4% v 16%) or died in hospital (6% v 20%) were smaller during the intervention period. Conclusions: Telemedicine has provided Victorian regional hospitals access to expert care for emergency department patients with suspected acute stroke. Eligible patients with ischaemic stroke are now receiving stroke thrombolysis more quickly and safely.

Chris F Bladin · Joosup Kim · Kathleen L Bagot · Michelle Vu · Natasha Moloczij · Sonia Denisenko · Chris Price · Nancy Pompeani · Lauren Arthurson · Casey Hair · Justin Rabl · Mick O'Shea · Patrick Groot · Leslie Bolitho · Bruce CV Campbell · Helen M Dewey · Geoffrey A Donnan · Dominique A Cadilhac

The potential of modern mass communication techniques for managing chronic disease should be explored further

Yang Si · Xiaoqiang Xiao · Cai Xia · Jiang Guo · Qiukui Hao · Qianning Mo · Yulong Niu · Hongbin Sun

Objective: To examine the prevalence of psychotropic medicine dispensing before and after older people enter residential care. Design: Retrospective national cohort study; analysis of Registry of Senior Australians (ROSA) data. Setting, participants: All concession card‐holding residents of government‐subsidised residential aged care facilities in Australia who entered residential care for at least three months between 1 April 2008 and 30 June 2015. Main outcome measures: Proportions of residents dispensed antipsychotic, benzodiazepine, or antidepressant medicines during the year preceding and the year after commencing residential care, by quarter. Results: Of 322 120 included aged care residents, 68 483 received at least one antipsychotic (21.3%; 95% CI, 21.1–21.4%), 98 315 at least one benzodiazepine (30.5%; 95% CI, 30.4–30.7%), and 122 224 residents at least one antidepressant (37.9%; 95% CI, 37.8–38.1%) during their first three months of residential care; 31 326 of those dispensed antipsychotics (45.7%), 38 529 of those dispensed benzodiazepines (39.2%), and 25 259 residents dispensed antidepressants (19.8%) had not received them in the year preceding their entry into care. During the first three months of residential care, the prevalence of antipsychotic (prevalence ratio [PR], 3.37; 95% CI, 3.31–3.43) and antidepressant dispensing (PR, 1.05; 95% CI, 1.04–1.07) were each higher for residents with than for those without dementia; benzodiazepine dispensing was similar for both groups (PR, 1.01; 95% CI, 0.99–1.02). Conclusions: Dispensing of psychotropic medicines to older Australians is high before they enter residential care but increases markedly soon after entry into care. Non‐pharmacological behavioural management strategies are important for limiting the prescribing of psychotropic medicines for older people in the community or in residential care.

Stephanie L Harrison · Janet K Sluggett · Catherine Lang · Craig Whitehead · Maria Crotty · Megan Corlis · Steven L Wesselingh · Maria C Inacio

Objectives: To assess the risks of stroke and cardiovascular mortality for Aboriginal and non‐Aboriginal Australians with atrial fibrillation. Design: Retrospective data linkage cohort study. Setting, participants: All people aged 20–84 years hospitalised with atrial fibrillation in Western Australia during 2000–2012. Main outcome measures: Stroke incidence rates and mortality after hospitalisation for atrial fibrillation, and 10‐year risks of stroke and of cardiovascular and all‐cause mortality. Results: Among 55 482 index admissions with atrial fibrillation, 7.7% of 20–59‐year‐old patients and 1.3% of 60–84‐year‐old patients were Aboriginal Australians. A larger proportion of Aboriginal patients aged 20–59 years had CHA2DS2‐VASc scores of 2 or more (59.8% v 21.8%). In 20–59‐year‐old Aboriginal patients, the incidence during follow‐up (maximum, 10 years; median, 7.1 years) of stroke (incidence rate ratio [IRR], 3.2; 95% CI, 2.5–4.1) and fatal stroke (IRR, 5.7; 95% CI, 3.9–8.9) were markedly higher than for non‐Aboriginal patients. Stroke incidence was higher for 60–84‐year‐old patients, but the difference between Aboriginal and non‐Aboriginal patients was smaller (IRR, 1.6; 95% CI, 1.3–2.0). Cardiovascular mortality during follow‐up was also higher for 20–59‐year‐old Aboriginal patients (IRR, 4.4; 95% CI, 4.3–5.9). The hazards of stroke (adjusted HR [aHR], 1.67; 95% CI, 1.22–2.28) and cardiovascular mortality (aHR, 1.47; 95% CI, 1.18–1.83) in younger Aboriginal patients remained significantly higher after multivariable adjustment; age/sex, principal diagnosis of atrial fibrillation, and CHA2DS2‐VASc score were the most influential factors. Conclusion: Stroke risk and cardiovascular mortality are markedly higher for Aboriginal than non‐Aboriginal patients with atrial fibrillation, particularly for patients under 60. Strategies for providing evidence‐based therapies and cardiovascular prevention to Aboriginal people with atrial fibrillation must be improved.

Lee Nedkoff · Erin A Kelty · Joseph Hung · Sandra C Thompson · Judith M Katzenellenbogen

To the Editor: Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and fatal neurodegenerative condition with no cure. Only two treatments with class I evidence exist — riluzole1 and edaravone2 — both with unclear mechanisms of action and modest survival benefits. In Australia, riluzole remains the only treatment approved by the Therapeutic Goods Administration. The Pharmaceutical Benefits Scheme limits initiation of riluzole to patients with at least 60% of predicted forced vital capacity, although facial weakness may make this an unreliable target. Initial and continuing treatment requires patients to be ambulant; or to have good upper limb function or to be able to swallow; and not to have respiratory failure. A recent retrospective study classified patients into different disease severity stages, ranging from 1 (one region involved) to 5 (death); patients with respiratory and nutritional failure were assigned to stage 4.3 The study identified that patients in stage 4 receiving 100 mg of riluzole daily did not progress to the next clinical stage (ie, death) as rapidly as those in milder stages. This suggests that the modest survival benefit experienced by patients taking riluzole comes about by extending the time spent at this stage. A quarter of patients present with bulbar or respiratory onset,4 making many ineligible for treatment, despite data suggesting they may benefit most.5 Mean survival in these forms of ALS is particularly short, meaning the modest survival benefit offered should be considered, as a majority of patients with advanced ALS do not wish to hasten death.6 A recent study of over 4000 trial participants confirmed benefit in both early and late stages,7 supporting use of riluzole throughout the disease. Few prospective studies on late‐stage treatments exist; patient choice in continuing treatment during advanced stages therefore remains paramount. Prospective studies are needed to establish whether the benefit of riluzole is weighted towards more advanced disease. However, recent studies, along with the recognition of the clinical spectrum of ALS, indicate that the current Pharmaceutical Benefits Scheme criteria are too stringent. As we move towards precision‐based medicine, different profiles of therapeutic response are likely. Regulators will be required to rapidly respond to emerging data to ensure the right patients can access the right medications.

Colin J Mahoney · Matthew C Kiernan

A 59-year-old woman attended a spasticity clinic with treatment-resistant atypical pressure ulcer in the right hand caused by focal spasticity secondary to upper motor neuron lesion

Anupam Datta Gupta · David H Wilson

A multifaceted approach will enable equitable stroke care for regional communities

Tayler Watson · Jeigh Tiu · Ben Clissold

Migraine affects over a billion people worldwide in any year and is the second most common cause of years lost due to disability

Michael Eller · Peter J Goadsby

To the Editor: Recreational misuse of nitrous oxide remains a significant public health problem,1 sustained in part by the ready availability online of gas‐containing canisters intended for use in the catering industry. Known as “nangs” or “whippits” and usually purchased in bulk, each canister contains 8 g of nitrous oxide. When inhaled, this gives a seconds‐long “high”, which is typically prolonged by using several “nangs” in a single session. Some individuals can consume hundreds each day. Prolonged exposure to nitrous oxide leads to the oxidisation of vitamin B12, rendering it unusable in key enzymatic reactions necessary for normal myelin synthesis.2 Over time, this leads to a potentially devastating neuropsychiatric syndrome that commonly presents with ataxia.3 Notably, the culprit shortage of vitamin B12 is a qualitative one and can be purely so, meaning that marked clinical deficits emerge in the presence of serum B12 levels that appear normal on standard laboratory assays. Furthermore, with continued exposure to nitrous oxide, these deficits will respond poorly to vitamin B12 supplementation. In a year‐long clinical audit at Royal Prince Alfred Hospital (2017–2018), seven nitrous oxide users, all aged between 20 and 30 years, presented with ataxia that ranged from mild to severe (Box 1). Most patients also had psychiatric symptoms. Nearly every patient estimated using 100 or more canisters of nitrous oxide per day in the months before being seen. Four patients also reported engaging in B12 supplementation (both oral and parenteral), aiming to circumvent the harmful sequelae of prolonged nitrous oxide misuse. Laboratory studies showed that all seven patients had accumulated homocysteine, as is usually seen when vitamin B12 is in short supply in the body.2 Individuals who reported taking supplements had serum B12 levels that were either normal or in excess of normal, implicating a qualitative deficiency of metabolically useful B12. Evidence of demyelination was seen on spinal cord imaging in six patients, including all those who used supplements, with the “inverted V” sign4 visible on T2‐weighted magnetic resonance imaging sequences (Box 2). Despite treatment according to best practice guidelines, all patients left hospital with persistent symptoms, and most were unable to walk or to attend to their bodily needs without the assistance of family members (modified Rankin score, 4). Sadly, one of the least affected individuals re‐presented to hospital with worsened symptoms because of continued nitrous oxide misuse. At every opportunity nitrous oxide users should be reminded of the futility of B12 supplementation, as one of many reasons why they should choose to avoid this profoundly destructive drug. Box 1 – Patients presenting with symptoms due to nitrous oxide misuse Age (years) Sex Canister use Duration of use B12 supplementation Ataxia severity* Psychiatric symptoms† Homocysteine level Serum B12 (active) MRI: “inverted V” sign‡ mRS: Day 1 mRS: discharge 20 Female 250/day 1 year No Severe Yes High Low (low) Yes 4 4 30 Male 60/day 1 year No Moderate Yes High Low (low) Yes 1 1 30 Male 100/day 6 months No Mild No High Low (normal) No 1 1 21 Male 200/day 1 year Yes Severe Yes High Normal (normal) Yes 4 4 23 Female 300/day 2 months Yes Severe Yes High Normal (high) Yes 4 4 23 Female 200/day 2 months Yes Severe Yes High High (high) Yes 4 4 28 Male 300/day 1 year Yes Mild No High Normal (normal) Yes 1 1 MRI = magnetic resonance imaging; mRS = modified Rankin score of neurological disability. * Ataxia: mild = visible gait disturbance; moderate = frequent falls; severe = inability to walk without assistance. † Psychiatric symptoms included mood disturbance, memory impairment and psychosis. ‡ MRI findings: “inverted V” sign on T2‐weighted MRI spinal cord imaging (Box 2). mRS: 0 = no symptoms; 1 = no significant disability despite symptoms; 2 = slight disability; 3 = moderate disability; 4 = moderately severe disability, unable to walk or attend to bodily needs without assistance; 5 = severe disability, bedridden; 6 = dead. Box 2 – T2‐weighted magnetic resonance imaging sequence showing “inverted V” sign, indicating the presence of dorsal column demyelination

Christopher Blair · Chris Tremonti · Leon Edwards · Paul S Haber · G Michael Halmagyi

Australian dementia research needs increased representation of people from culturally and linguistically diverse backgrounds

Lee‐Fay Low · Annica L Barcenilla‐Wong · Bianca Brijnath

The strengths of primary care should be harnessed to address complexities of the ageing population

C Dimity Pond · Catherine Regan

The PBS urgently needs to update anti-epileptic drug prescribing restrictions that put patients and prescribers at risk

Christian A Gericke · Terence J O'Brien

New therapies show promise, but effective treatment of non-motor, non-dopaminergic symptoms remains a major challenge

Michael W Hayes · Victor SC Fung · Thomas E Kimber · John D O'Sullivan

Being part of a network allows the clinician to think beyond the boundaries of their hospital

Peter J Hand

Reperfusion therapies for ischaemic stroke have transformed the prognosis for long term disability, but maximising patient benefit requires increased community recognition and faster treatment

Bruce CV Campbell

A 68- year- old man presented with hyperacute stroke one hour after symptom onset

Amy Ting · Abhay R Venkat · Yash Gawarikar · Ronak Patel

As a result of their widespread use, elucidating the influence of DMTs on fertility, pregnancy and breastfeeding is critical for assisting physicians and patients in weighing up the relative risks and benefits of continuing therapy. International pregnancy registries have a key role to play, and neurologists should be encouraged to contribute to these when possible. Furthermore, family planning counselling may be useful for patients with multiple sclerosis to help alleviate fears and concerns and to enable more informed decision making. A multidisciplinary approach, involving collaboration between neurologists, obstetricians, midwives, anaesthesiologists and fertility specialists (when required), is also recommended to help optimise outcomes for both the patient and the child. Decision making should be a shared experience between patient and physician, with a personalised approach developed to meet the unique needs of each individual patient.

Anneke Van Der Walt · Ai‐Lan Nguyen · Vilija Jokubaitis