Mysterious neuro illness splits health authorities

When Roger Ellis fell ill two years ago, his family rushed to the hospital, fearing he was having a heart attack. Doctors ruled that out, but days later, he had a seizure.

In the following weeks, the retired industrial mechanic, 64, who lived in the east Canadian town of Bathurst, New Brunswick, grew anxious and disoriented, and often repeated himself.

In his first three months in hospital, he lost 35kg and ate through a tube and used a wheelchair. “We nearly lost him a couple of times,” said his son, Steve.

Doctors ruled out epilepsy, a stroke, Creutzfeldt-Jakob disease (CJD), autoimmune encephalitis and cancer. Ellis spent more than a year in hospital before he was transferred to a care home, where he remains. His family believes he is in a cluster of people suffering from a mysterious progressive neurological illness.

The cases have stumped experts, prompting a row between officials who suggest the cases are unrelated, and scientists who argue that they may all have been triggered by environmental factors or contaminants.

Victims experience pains, spasms and behavioural changes, said Dr Alier Marrero, the neurologist who first identified the cluster. Many then showed signs of cognitive decline, muscle wasting, drooling, teeth chattering and frightening hallucinations.

So far, 48 cases have been publicly acknowledged, but officials have told the Guardian that the number of affected people now exceeds 100.

In a surprise turn of events, New Brunswick’s health minister, Dorothy Shephard, announced in late October that an epidemiological report had found no significant evidence that any known food, behaviour or environmental exposure could be responsible.

A controversial paper presented to the Canadian Association of Neuropathologists claimed that eight deaths attributed to the cluster were “misdiagnoses” of known diseases, such as Alzheimer’s and cancer.

The episode has also exposed the fractured relationship between the province and federal health authorities, who were asked not to assist in the investigation – and said they were prevented from testing tissue samples of deceased patients. Staff at Canada’s public health agency cast doubt on the province’s suggestion that there was no link between the cases.

Residents first learned of the mysterious cluster nearly a year ago, when a leaked memo from the province’s public health agency asked physicians to be on the lookout for symptoms similar to CJD – a rare, fatal brain disease caused by misformed proteins known as prions. But in late October, a paper presented by Dr Gerard Jansen of the Canadian Association of Neuropathologists shocked families by suggesting that eight of the victims had died from unrelated brain diseases.

“We’ve asked unequivocally for that study to be pulled and for an apology to be issued,” said Kat Lanteigne, head of the advocacy group BloodWatch.

Experts believe BMAA – a neurotoxin found in blue-green algae blooms – could help to explain the varied symptoms. In one study, high levels of BMAA were found in lobster. Harvesting lobster is one of New Brunswick’s biggest economic drivers – promoting speculation that efforts to rule out the existence of a cluster could be motivated by political decision-making.

Families and scientists have grown troubled by the New Brunswick government’s apparent efforts to distance itself from Marrero, the neurologist who identified the cluster.

Shephard told reporters there had been “issues” in the reporting process that allowed the situation to escalate and omitted to mention that Marrero had worked with federal scientists, as well as neurologists in other provinces, when first identifying the cluster.

But for experts in brain disease, the age range of patients, the volume of cases and the geographic location of those suffering suggest more thorough investigation is needed.

A second investigation, led by a committee of neurologists from across the province – but probably not including Marrero – is expected to release a second report early next year after reviewing the 48 patients, nearly all of whom were treated by Marrero.

“They really seem to be discrediting him without using his name,” said Ellis. “Of all people, he has been the one that has been the most communicative during these last few months.”

There is speculation that efforts to rule out the existence of a cluster are driven by political decisionmaking