Neuronal intranuclear inclusion disease in New Zealand: A novel discovery.

Neuronal intranuclear inclusion disease, caused by a GGC repeat expansion in the 5'-untranslated region of NOTCH2NLC, is a rare neurodegenerative condition with highly variable clinical manifestations. In recent years, the number of reported cases have increased dramatically in East Asia. We report the first four genetically confirmed cases of neuronal intranuclear inclusion disease in New Zealand, all having Polynesian ancestry (three New Zealand Maori and one Cook Island Maori). Phenotypically, they resemble cases reported from recent large East Asian cohorts.

The Socioeconomic Impact of Diseases of Working Equids in Low and Middle-Income Countries: A Critical Review.

Working equids provide a crucial contribution to the livelihoods and food security of communities in low- and middle-income countries (LMICs). Nevertheless, they are a neglected category within animal health policies and interventions of governmental and non-governmental institutions. This critical review aims to assess the socioeconomic impact of diseases of working equids in LMICs. By highlighting the implications of diseases on working equid welfare, human wellbeing and livelihoods, this review seeks to sensitise policymakers within governments and international organisations to develop policies and interventions aimed at protecting the health of working equids and, consequently, the health and livelihoods of their dependent communities. Twenty relevant publications were identified through the search of five databases (CAB Abstracts, Web of Science Core Collection, BIOSIS, EMBASE and Scopus), backward citation searching and screening of indexes of proceedings and Special Issues retrieved from the database search. The review findings show that diseases of working equids have detrimental socioeconomic effects. However, this subject is under-researched and restricted to few diseases and geographical settings. Considering the complexity of the issue, this review demonstrates that the 'One Health' approach represents an opportunity to clarify the link between equid health, human wellbeing and livelihoods, facilitating the translation of research into policy.

Data analysis of zoonoses notifications in Aboriginal and Torres Strait Islander populations in Australia 1996-2021: implications for One Health.

Introduction: Zoonoses are a health concern for Aboriginal and Torres Strait Islander peoples in Australia that face elevated risk of disease related to the environment and animals. Internationally, One Health is encouraged to effectively manage zoonoses by taking integrated approaches involving animal, human, and environmental health sectors to improve health outcomes. However, Australia's health systems manage zoonotic diseases in animals and people separately which does not support a One Health approach. For the effective management of zoonoses, a strong evidence base and database regarding the epidemiology of zoonotic pathogens is needed. However, we currently lack this evidence limiting our understanding of the impact of zoonoses on Aboriginal and Torres Strait Islander populations. Methods: As a first step towards building the evidence base, we undertook a descriptive analysis of Aboriginal and Torres Strait Islander zoonotic notifications in Australia from 1996 to 2021. We presented notifications as annual notification rates per 100,000 population, and percentages of notifications by state, remoteness, sex, and age group. Results: Salmonellosis and campylobacteriosis were the most notified zoonoses with the highest annual notification rates of 99.75 and 87.46 per 100,000 population, respectively. The north of Australia (Queensland, Northern Territory and Western Australia), remote and outer regional areas, and young children (0-4 years of age) had the highest percentages of notifications. Discussion: To our knowledge, these findings are the first national presentation of the epidemiology of zoonoses within Aboriginal and Torres Strait Islander populations. A greater understanding of transmission, prevalence and impact of zoonoses on Aboriginal and Torres Strait Islander peoples (including animal and environmental health factors) is required to inform their effective management through a One Health approach.

One Health and Australian Aboriginal and Torres Strait Islander Communities: A One Health Pilot Study.

Many Aboriginal and Torres Strait Islander communities face barriers in accessing animal healthcare and are exposed to disproportionate environmental health exposures leading to increased risk of disease. A One Health approach has been promoted to address public health risks and improve human, animal, and environmental health outcomes in communities. We undertook a pilot One Health study in Aboriginal and Torres Strait Islander communities in Queensland collecting animal, human, and environmental health data from 82 households. We performed a descriptive analysis and assessed the association between human and environmental health exposures and animal health outcomes. Most households were not crowded (82.9%) but did report a high level of environmental health concerns (86.6%). The majority of households owned cats and dogs (81.7%), with most animals assessed as healthy. There was no association between human and environmental health exposures and animal health outcomes. As most households experienced concerns regarding housing conditions, environmental health programs should prioritise improving household factors. There was also strong support for animal healthcare (including access to medicines and veterinarians, education programs and population management), indicating that a One Health approach is desired by communities.

Alexander disease: The story behind an eponym.

In 1949, William Stewart Alexander (1919-2013), a young pathologist from New Zealand working in London, reported the neuropathological findings in a 15-month-old boy who had developed normally until the age of seven months, but thereafter had progressive enlargement of his head and severe developmental delay. The most striking neuropathological abnormality was the presence of numerous Rosenthal fibers in the brain. The distribution of these fibers suggested to Alexander that the primary pathological change involved astrocytes. In the next 15 years, five similar patients were reported, and in 1964 Friede recognized these cases reflected a single disease process and coined the eponym "Alexander's disease" to describe the disorder. In the 1960s, electron microscopy confirmed that Rosenthal fibers were localized to astrocytes. In 2001, it was shown that Alexander disease is caused by mutations in the gene encoding glial fibrillary acidic protein, the major intermediate filament protein in astrocytes. Although the clinical, imaging, and pathological manifestations of Alexander disease are now well known, few people are familiar with Alexander's career. Although he did not make a further contribution to the literature on Alexander disease, his observations and accurate interpretation of the neuropathology have justified the continued use of the eponym "Alexander disease."

Seroprevalence of canine distemper virus (CDV) in the free-roaming dog (Canis familiaris) population surrounding Chitwan National Park, Nepal.

Canine distemper virus (CDV) is a global multi-host pathogen that is capable of causing considerable mortality in a range of species and is important in the field of conservation medicine. Nepal's Chitwan National Park is a protected area providing habitat for 32% of the country's mammal species including endangered carnivores such as the Bengal tiger (Panthera tigris tigris) that are susceptible to CDV. The presence of free-roaming dogs around protected areas could represent a source of infectious disease for transmission to local wildlife. A cross-sectional demographic and canine distemper virus seroprevalence study of 100 free-roaming dogs from the Chitwan National Park buffer zone and surrounding area was conducted in November 2019. The overall seroprevalence indicating past exposure to canine distemper virus was 80.0% (95% CI: 70.8-87.3). Of the host variables assessed, sex and age were positively associated with seroprevalence at the univariable level, with male dogs demonstrating lower seroprevalence than females (OR = 0.32, 95% CI: 0.11-0.91) and adult dogs demonstrating higher seroprevalence than juveniles (OR = 13.94, 95% CI: 1.37-142.29). The effect of sex was no longer significant at the multivariable level, but the direction of the effect remained the same. The effect of age remained significant after multivariable analysis (OR = 9.00, 95% CI: 1.03-192.75). No spatial associations were demonstrated in relation to the buffer zone area or boundary of Chitwan National Park. Free-roaming dog neutering and vaccination programmes can provide a useful baseline for future CDV studies in the region, and a proxy to monitor disease threats to susceptible wildlife.

Encephalitis in adults in the Auckland and Northland regions of New Zealand, 2009 to 2018.

We conducted a retrospective study to determine the incidence and frequency of different subtypes of encephalitis in patients aged 15 and older in the Auckland and Northland regions of New Zealand between 2009 and 2018. Residents in Auckland and Northland presenting with encephalitis between 2009 and 2018 were identified from three overlapping databases: positive cerebrospinal fluid (CSF) viral polymerase chain reaction (PCR) tests, CSF neuronal antibody requests, and CSF neuronal antibody tests sent overseas. A diagnosis of autoimmune encephalitis required fulfilment of diagnostic criteria published by Graus and colleagues (2016). One hundred and thirty-six (69, 50.7% female) patients met study inclusion criteria. The median age was 59 (range 15-92). The annual incidence was 1.10 cases per 100,000 person-years. Of these 136 patients, 56 (41.2%) had an infectious aetiology, with varicella zoster (26, 46.4%) and herpes simplex (23, 41.1%) being the most common agents. Autoimmune encephalitis was diagnosed in 32 patients (23.5%). LGI-1 antibody was the most commonly identified neuronal autoantibody (10 patients, 13.2%). Forty-eight patients (35.3%) had encephalitis of unknown cause. In-hospital mortality for infectious encephalitis was 12.5%, autoimmune encephalitis 6.3%, and encephalitis of unknown cause 10.4%. Compared to a previous analysis of encephalitis in adults in Auckland, the incidence of encephalitis and autoimmune encephalitis had increased. The proportion of patients with an unknown cause for encephalitis had decreased.

Healthcare Management of Human African Trypanosomiasis Cases in the Eastern, Muchinga and Lusaka Provinces of Zambia.

Human African trypanosomiasis (HAT) is a neglected tropical disease that has not received much attention in Zambia and most of the countries in which it occurs. In this study, we assessed the adequacy of the healthcare delivery system in diagnosis and management of rHAT cases, the environmental factors associated with transmission, the population at risk and the geographical location of rHAT cases. Structured questionnaires, focus group discussions and key informant interviews were conducted among the affected communities and health workers. The study identified 64 cases of rHAT, of which 26 were identified through active surveillance and 38 through passive surveillance. We identified a significant association between knowledge of the vector for rHAT and knowledge of rHAT transmission (p < 0.028). In all four districts, late or poor diagnosis occurred due to a lack of qualified laboratory technicians and diagnostic equipment. This study reveals that the current Zambian healthcare system is not able to adequately handle rHAT cases. Targeted policies to improve staff training in rHAT disease detection and management are needed to ensure that sustainable elimination of this public health problem is achieved in line with global targets.

A randomized clinical trial to compare ketamine-butorphanol-azaperone-medetomidine and detomidine-etorphine-acepromazine for anesthesia of captive Przewalski horses (Equus przewalskii).

OBJECTIVE: To compare ketamine-butorphanol-azaperone-medetomidine (KBAM) to detomidine-etorphine-acepromazine (DEA) for field anesthesia in captive Przewalski horses (Equus przewalskii). ANIMALS: 10 adult Przewalski horses. PROCEDURES: A prospective randomized crossover trial was conducted. Each horse was immobilized once with KBAM (200 mg ketamine, 109.2 mg butorphanol, 36.4 mg azaperone, and 43.6 mg medetomidine) and once with DEA (40 mg detomidine premedication, followed 20 minutes later by 3.9 to 4.4 mg etorphine and 16 to 18 mg acepromazine). Both protocols were administered by IM remote dart injection with a washout period of 6 months between treatments. Selected cardiorespiratory variables and quality of anesthesia were recorded. Antagonists were administered IM (KBAM, 215 mg atipamezole and 50 mg naltrexone; DEA, 4 mg RX821002 and 100 mg naltrexone). RESULTS: All horses were anesthetized and recovered uneventfully. Inductions (DEA, 6.8 min; KBAM, 11.6 min; P = 0.04) and recoveries (DEA, 3.2 min; KBAM, 19.6 min; P < 0.01) were faster with DEA compared with KBAM. Quality scores for induction and recovery did not differ between protocols, but maintenance quality was poorer for DEA (P < 0.01). Clinical concerns during DEA immobilizations included apnea, severe hypoxemia (arterial partial pressure of oxygen < 60 mm Hg), muscle rigidity, and tremors. Horses treated with KBAM were moderately hypoxemic, but arterial partial pressures of oxygen were higher compared with DEA (P < 0.01). CLINICAL RELEVANCE: Captive Przewalski horses are effectively immobilized with KBAM, and this protocol results in superior muscle relaxation and less marked hypoxemia during the maintenance phase, but slower inductions and recoveries, compared with DEA.

Ranging patterns and factors associated with movement in free-roaming domestic dogs in urban Malawi.

Rabies is a neglected zoonotic disease that causes around 59,000 deaths per year globally. In Africa, rabies virus is mostly maintained in populations of free-roaming domestic dogs (FRDD) that are predominantly owned. Characterizing the roaming behavior of FRDD can provide relevant information to understand disease spread and inform prevention and control interventions. To estimate the home range (HR) of FRDD and identify predictors of HR size, we studied 168 dogs in seven different areas of Blantyre city, Malawi, tracking them with GPS collars for 1-4 days. The median core HR (HR50) of FRDD in Blantyre city was 0.2 ha (range: 0.08-3.95), while the median extended HR (HR95) was 2.14 ha (range: 0.52-23.19). Multivariable linear regression models were built to identify predictors of HR size. Males presented larger HR95 than females. Dogs living in houses with a higher number of adults had smaller HR95, while those living in houses with higher number of children had larger HR95. Animals that received products of animal origin in their diets had larger HR95, and only in the case of females, animals living in low-income areas had larger HR50 and HR95. In contrast, whether male dogs were castrated or not was not found to be associated with HR size. The results of this study may help inform rabies control and prevention interventions in Blantyre city, such as designing risk-based surveillance activities or rabies vaccination campaigns targeting certain FRDD subpopulations. Our findings can also be used in rabies awareness campaigns, particularly to illustrate the close relationship between children and their dogs.

Zoonoses and the Aboriginal and Torres Strait Islander population: A One Health scoping review.

With limited access to animal health services, and high disease burdens among domesticated animals, Aboriginal and Torres Strait Islander communities in Australia face higher risk of disease including zoonoses. However, we lack understanding of the contribution of often preventable zoonoses to the health of these communities, which would enable us to enhance public health strategies and improve health outcomes. We conducted a scoping review to identify the current state of evidence on zoonoses in the Aboriginal and Torres Strait Islander population. We examined the size, scope and characteristics of the evidence base and analysed the zoonoses detected in the studies within a One Health framework. We identified 18 studies that detected 22 zoonotic pathogens in animals, people, and the environment, with most studies detecting pathogens in a single One Health sector and no studies investigating pathogens in all three sectors. Findings indicate that despite the strong conceptual foundations of One Health throughout the evidence base, evidence is lacking in application of this concept. There is a need to undertake further research that prioritises Aboriginal and Torres Strait Islander leadership, considers the contribution of human, animal and environmental health factors, and investigates the prevalence and impact of zoonoses in communities through a One Health approach.

Autoinflammatory syndromes in neurology: when our first line of defence misbehaves.

Autoinflammatory syndromes result from a defective innate immune system. They are characterised by unexplained fever and systemic inflammation involving the skin, muscle, joints, serosa and eyes, along with elevated acute phase reactants. Autoinflammatory syndromes are increasingly recognised as a cause of neurological disease with a diverse range of manifestations. Corticosteroids, colchicine and targeted therapies are effective if started early, and hence the importance of recognising these syndromes. Here, we review the neurological features of specific autoinflammatory syndromes and our approach (as adult neurologists) to their diagnosis.

One Health in Indigenous Communities: A Critical Review of the Evidence.

Indigenous populations around the world face disproportionately high rates of disease related to the environment and animals. One Health is a concept that has been used effectively to understand and address these health risks. One Health refers to the relationships and interdependencies between animal, human, and environmental health and is an emerging research field that aligns with indigenous views of health. To understand the applicability of One Health in indigenous communities, a critical review was undertaken to investigate evidence of One Health research in indigenous communities internationally, assess the strength of evidence, and understand what gaps are present. This review included the appraisal of twenty-four studies based in five regions: Canada, Africa, Australia, South America, and Central America. The review found that there is a need for studies of high strength, with rigorous methods, local leadership, and active involvement of indigenous viewpoints, to be undertaken in indigenous communities internationally that focus on One Health. It highlights the need to further consider indigenous viewpoints in research to reduce limitations, increase effectiveness of findings, consider appropriateness of recommendations, and benefit communities.

Altered mental status in "Guillain-Barré syndrome" -a noteworthy clinical clue.

Guillain-Barré syndrome (GBS) is widely regarded as a "pure" peripheral nervous system disorder. However, this simplistic interpretation belies the fact that central nervous system involvement, often manifesting as derangements in mental status can occur as a complication of the "pure" form of the disorder, as part of GBS variants, as well as in a number of mimic disorders. Despite being common in clinical practice, there is no guidance in the literature as to how to approach such scenarios. Herein, we detail our approach to these cases.

Visual snow: Not so benign.

Visual snow is the perception of flickering dots throughout the entire visual field and occurs with other symptoms of dysfunctional central sensory processing. We describe a patient who presented with visual snow, illusory visual motion, photopsia, and reduced night vision. He subsequently developed progressive cognitive impairment, myoclonus and ataxia. A diagnosis of sporadic Creutzfeldt-Jakob Disease was confirmed on post-mortem examination more than 49 months after symptom onset. The visual snow syndrome is typically benign, but our patient illustrates that occasionally it is the first manifestation of a serious brain disease. Careful application of the diagnostic criteria for the visual snow syndrome is important, particularly with the use of neuroimaging to exclude pathology in the occipital cortex.