An MRI scan of the skull of a 43-year-old man thought to be the first New Zealand case of iCAA, a rare and incurable brain disease. Image / NZ Medical Journal
An MRI scan of the skull of a 43-year-old man thought to be the first New Zealand case of iCAA, a rare and incurable brain disease. Image / NZ Medical Journal
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A New Zealand man who received a brain membrane graft from a dead body when he was a baby has been permanently hospitalised with an incurable brain disease.
In what is believed to be the first case of its kind in Aotearoa, the 43-year-old hasbeen diagnosed with a very rare condition known as iatrogenic cerebral amyloid angiopathy, or iCAA.
The disease can happen after the use of contaminated neurological materials taken from dead bodies, also known as cadavers.
The case is highlighted in a report in the NZ Medical Journal published today.
The report states that “iCAA occurs due to seeding of pathological (amyloid-beta) proteins into brain tissue from human-derived grafts and has been described as a distinct clinical type of CAA”.
An MRI scan of the skull of a 43-year-old man thought to be the first New Zealand case of iCAA, a rare and incurable brain disease. Image / NZ Medical Journal
CAA – cerebral amyloid angiopathy – is a progressive cerebrovascular disorder that can cause bleeding in the brain and a progressive cognitive decline. It is strongly associated with Alzheimer’s disease and old age.
The authors of the report – Chuen Siang Low, Sarah Buchanan, Nicholas Cutfield, Sophie Parker and Robin Fox – are all doctors at the Neurology Department at Dunedin Hospital.
The man, who had until recently been living independently and working full time, now requires hospital-level care. His family has been counselled about the risk of future intracranial bleeding.
Born in the early 1980s, he was found to have a scalp defect that resulted in the exposure of the underlying dura mater – the outermost protective membrane covering a person’s brain and spinal cord.
Doctors treated the issue with lyophilised (freeze-dried) cadaveric dura mater – covering the exposed area by grafting a piece of membrane harvested from a dead body.
Cadaveric dura mater was once commercially available and used in neurosurgery for dural repair on people around the world.
“Cadaveric lyophilised dural grafts are one of the commonest reported causes of iCAA and were used in neurological procedures in New Zealand in the 1980s,” the authors wrote.
“Usage ceased when they were recognised as a cause of iatrogenic (caused by a medical procedure) Creutzfeldt-Jakob disease – the best known example of seeding of abnormal proteins causing a neurodegenerative condition.”
The authors noted that the condition has recently gained public attention due to media coverage of cases found overseas.
Last year, UK woman Natalie Bralee-Brett, 53, spoke of her despair after being diagnosed with the disease. Heartbreakingly, her younger brother has also been diagnosed with the disease.
They are thought to be the only siblings identified on the international iCAA register, which has 52 confirmed cases, according to Sky News.
In 1997 the World Health Organisation advised against the use of cadaveric dura mater grafts after it was found they had been giving some people Creutzfeldt-Jakob Disease (CJD).
Around that time, CJD was also being spread by the consumption of cattle infected with Bovine Spongiform Encephalopathy (BSE) or “mad cow disease”.
‘Possible’ there are more cases in NZ
The New Zealand man, who has a history of focal epilepsy, sought medical attention due to an increase in seizure frequency, cognitive decline and behavioural changes that included paranoid thoughts.
Doctors involved in the case said his neurological history, involving the dural graft from a cadaver as a baby, led them to consider iCAA.
“It is unknown how many patients in New Zealand received cadaveric dural grafts, as no registry was kept,” the report said.
“To our knowledge, this is the first reported case of iCAA in New Zealand.
“However, the long delay from exposure to symptoms means it is possible more cases of iCAA will be seen in New Zealand.”
The authors concluded that the case highlighted the importance for doctors of considering iCAA in younger patients with suggested imaging and a history of dural graft use.
“Due to the long delay from exposure to symptoms, the exposure may not be immediately evident without looking into old case notes.”
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