Background: Motor neurone disease (MND) is a neurodegenerative disease that leads to significant morbidity and mortality. The Global Burden of Disease (GBD) project estimated the MND all-age global prevalence is 4.5 (95% confidence interval (CI)=4.1-5.0) per 100,000 persons and the all-age incidence of 0.78 (95% CI = 0.71-0.86) per 100,000 person-years. Whether the prevalence and incidence of MND within South Australia using registry data is in keeping with these global estimates has not been studied.
Objective: To describe the prevalence and annual incidence rates (IRs) of MND in South Australia between 2017 and 2019.
Methods: A retrospective cohort study calculating the point prevalence of MND on 31 December 2017, 2018 and 2019 utilizing data from the Australasian Motor Neurone Disease Registry (AMNDR). The annual IRs between 2017 and 2019 were calculated using population data reported in the 2016 Australian census and age-standardized rates utilized the world population from the United Nations Population Division's World Population Prospects.
Results: A total of 256 MND patients were identified, of whom 114 were alive on 31 December 2019. Based on the 2016 population of 1,676,653 persons, the estimated prevalence was 6.79 per 100,000 persons. The crude IR for the all-age South Australian population was 3.34 per 100,000 person-years (95% CI, 2.85-3.88). The estimated annual IRs based on those ≥20 yo were 4.98 (3.84-6.35), 3.68 (2.71-4.88), and 4.21 (3.17-5.49) per 100,000 person-years for 2017, 2018, and 2019, respectively.
Conclusion: The crude prevalence and incidence of MND in South Australia were considerably higher than global estimates. This may reflect higher rates of the disease or higher rates of case ascertainment compared to the GBD project.
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http://dx.doi.org/10.1080/21678421.2022.2108326 | DOI Listing |
Seizure
January 2025
Neurology department, Royal Brisbane and Women's Hospital, Brisbane, Australia.
Objectives: There have been conflicting reports about the frequency of neural autoantibodies in epilepsy cohorts, which is confounded by the lack of clear distinction of epilepsy from acute symptomatic seizures due to encephalitis. The aim of this study was to determine the frequency of neural autoantibodies in a well characterised population of refractory focal epilepsy of known and unknown cause.
Methods: Cases were recruited from epilepsy outpatient clinics at the Princess Alexandra, Mater, Royal Brisbane and Women's and Cairns Base Hospitals from 2021 - 2023.
JMIR Res Protoc
January 2025
Quality Use of Medicines and Pharmacy Research Centre, UniSA Clinical and Health Sciences, University of South Australia, Adelaide, Australia.
Background: Adverse medicine events (AMEs) are unintended effects that occur following administration of medicines. Up to 70% of AMEs are not reported to, and hence remain undetected by, health care professionals and only 6% of AMEs are reported to regulators. Increased reporting by consumers, health care professionals, and pharmaceutical companies to medicine regulatory authorities is needed to increase the safety of medicines.
View Article and Find Full Text PDFAngew Chem Int Ed Engl
January 2025
Shandong University, Chemistry and Chemical Engineering, CHINA.
In this contribution, we designed a new xanthate RAFT agent by introducing (5,6,7,8-tetrahydro-2-naphthalenyl)oxy (TNO) as the Z group, namely 2-[(((5,6,7,8-Tetrahydro-2-naphthalenyl)oxycarbonothioyl)thio)ethyl propanoate] (TNXEP). Due to the presence of the TNO group, TNXEP enabled highly controlled and ultrafast photoiniferter RAFT polymerization under violet (λ = 405 nm) and blue (λ = 450 nm) light. This approach was effectively extended to aqueous media for polymerization-induced self-assembly (PISA), facilitating the synthesis of polymeric nanoparticles.
View Article and Find Full Text PDFHepatol Commun
February 2025
Centenary Institute of Cancer Medicine and Cell Biology, the University of Sydney, Sydney, New South Wales, Australia.
Sci Transl Med
January 2025
Department of Pediatrics, Washington University in St. Louis, School of Medicine, St. Louis, MO 63110, USA.
Children with neurodegenerative disease often have debilitating gastrointestinal symptoms. We hypothesized that this may be due at least in part to underappreciated degeneration of neurons in the enteric nervous system (ENS), the master regulator of bowel function. To test this hypothesis, we evaluated mouse models of neuronal ceroid lipofuscinosis type 1 and 2 (CLN1 and CLN2 disease, respectively), neurodegenerative lysosomal storage disorders caused by deficiencies in palmitoyl protein thioesterase-1 and tripeptidyl peptidase-1, respectively.
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