News in brief: Brain iron overload point to therapeutic opportunities in MSA; People with MS miss out on NDIS support; RACP calls for urgent action on COVID-19 vaccination for people with disability

5 Oct 2021

Brain iron overload point to therapeutic opportunities in MSA

A study showing brain iron overload contributes to multiple system atrophy (MSA) has opened new pathways for therapeutic investigation.

The Australian-led study assessed age-related iron metabolism in different brain regions of transgenic and non-transgenic mice, and whether iron-lowering drugs could alleviate disease in the aged, transgenic group.

The transgenic (PLP-αsynuclein) mice — which over-expressed human wildtype α-synuclein in oligodendrocytes — accumulated iron and had perturbed iron-ferritin interactions in their substantia nigra (NS), putamen and cerebellum by 20 months old, the authors wrote in Neurobiology of Disease.

They also had reduced ceruloplasmin-bound copper in the substantia nigra and cerebellum, likely impairing ceruloplasmin’s ability to metabolise iron and triggering accumulation.

Deferiprone (1 mg/mL) and ceruloplasmin (5 mg/kg in saline) administered for two months in 12-month-old mice prevented motor performance decline and were associated with higher neuronal survival and reduced α-synuclein aggregate density in the SN, the authors reported.

“This is the first study to report brain iron accumulation in a mouse model of MSA,” they wrote.

Post-mortem studies have identified high levels of brain iron in MSA-affected patients, though iron’s role in disease progression and as a therapeutic target have yet to be determined.

“Our results indicate that elevated iron in MSA mice may result from ceruloplasmin dysfunction and provide evidence that targeting iron in MSA could be a viable therapeutic option,” the authors suggested.

Patients currently have no disease-modifying therapies and have an average prognosis of less than 10 years from diagnosis.

People with MS miss out on NDIS support

People with multiple sclerosis are not well supported by the National Disability Insurance Scheme (NDIS), new research suggests. A study involving 186 people with MS found that only 49% had an NDIS package approved, with the annual allowance ranging from $30, 318 for patients with mild disability, $38,361 for moderate disability and $115113 for severe disability.

Restricted mobility seemed to be the driving factor, and the package value correlated with EDSS steps, cognitive impairment and physical impact, but not psychological impact, according to the research led by Dr Jeannette Lechner-Scott, a neurologist at the University of Newcastle. The findings are published in Australian Health Review.

RACP calls for urgent action on COVID-19 vaccination for people with disability

The Royal Australasian College of Physicians (RACP) has serious concerns that people living with disability have not been prioritised for COVID-19 vaccinations, as states prepare to ease restriction in a matter of days.

The RACP has repeatedly raised concerns about the slow rollout of the COVID-19 vaccines to people with disability and the need to urgently prioritise this group. Disability care residents and staff were in the highest priority group for vaccination yet only 67% of residents and 59% disability screened workers are vaccinated.

People with disability are at increased risk of adverse outcomes if they are infected by COVID-19. A study in the UK reported that 58% of COVID-19 deaths were among people who had a disability, whilst another UK study found people with intellectual disability were eight times more likely to die of COVID-19 than the general population.

Dr Jacqueline Small, RACP President-elect and developmental paediatrician said: “The DRC report that people living with disability were deprioritised in the vaccine rollout is deeply concerning. We have had months to get this right. Now we are days away from states easing restrictions with less than half of NDIS participants aged 16 years and over fully vaccinated – that is really troubling.

“A coordinated connected response with inclusion of people with disability, local health districts, primary health networks and non-government organisations is needed urgently.”

report released by the national organisation Children and Young People with Disability Australia has shown that over 70% of respondents experienced difficulties in securing vaccinations.

The RACP said the federal government must publish data on the COVID-19 vaccine rollout for people with disability to enable a quick response at a local level where gaps in accessibility are identified. This must include regular, up-to-date disaggregated data on the vaccination numbers for all people with disability, as at present only NDIS participant data is available publicly. It must also include the rates of COVID-19 infection and death amongst all people with disability.

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