Mad Cow Lawyer & Attorney : Marler Clark : Mad Cow Blog

After reporting four cases of classic Creutzfeldt-Jakob Disease (CJD) last year, Tennessee health officials are saying the death of a Kingston woman in February and a Rockwood man earlier this month are also due to prion diseases.  We'd noted in our previous posting that one death and a critical  illness then --both in Roane County, Tennessee-- had generated some interesting local comments.  We wondered if there could be a "cluster' of rare disease.  Then we heard on our own.

 As 54 year old Brad Smith neared death on St. Patrick's Day, his brother Rod wrote us, saying:

The "man in Rockwood" is my 54 year old brother. He is now near death, a very difficult trial for his wife, daughters, and extended family. Initial symptoms began in August and the process has been downhill from there. Only after a specific request by his wife was he tested for CJD. A spinal tap came back with the positive diagnosis of CJD. Hearing about the other recent local death in Kingston was alarming, but now with your help learning of these other cases in Roane County it is definitely not sporadic cases of one in a million, but here we have four cases in a county of 52,000.

The next day on March 18th, Brad Smith died of  CJD.  Rod let us know with this report:

My brother passed this morning (3/18/09). He is mentioned in the above article as the man from Rockwood. His symptoms first showed in August. We found out two weeks ago through a spinal tap that it was CJD. Symptoms seemed to be in line with what CDC Atlanta shows as for classic CJD. But why so many cases in Roane County of a supposedly "sporadic" disease?

Health officials are blaming classic CDJ, not the variant CDJ that is related to Mad Cow Disease as the causes of the deaths of Mary Lee McGill, 69, and Brad Smith. We do not think they knew one another, but lived about a dozen miles apart.   This one deserves a careful look.   The people they've left behind deserve no less.

There's a story in the Knoxville News here.

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These days, it’s often the comments to the news, that makes the most interesting reading. For example take the public feedback that a story on the WBIR-TV website in Knoxville, TN.

The story about one death caused by Creutzfeldt-Jakob Disease in nearby Roane County, TN brings in 2 responses from individuals who say CJD was the cause of death for each of  their grandmothers in Roane, TN; one who says his father, a Roane resident, was just diagnosed with the disease, and another reader who says he’s heard of several other CJD deaths in the county.

The four who commented were responding to this:

The Tennessee Department of Health is investigating a case of Creutzfeldt-Jakob Disease, and saying in the meantime it is extremely unlikely that it is a human case of the disease known as 'mad cow.'

An early diagnosis of Creutzfeldt-Jakob has been made for one patient, one of 6-8 the state of Tennessee is likely to see this year.

The Centers for Disease Control identifies classic Creutzfeldt-Jakob as a degenerative disease of the nervous system, likely caused by normal prion proteins deforming into abnormal prion proteins.   {The bovine spongiform encephalopathy (BSE) disease, more commonly known as 'mad cow' disease, is also a prion disease.}

The state's four cases of Creutzfeldt-Jakob last year were all the classic disease.

One of those commenting said:

My grandmother passed away Feb 4, 2009 at home with CJD. She spent 3 weeks in ParkWest hospital before we recevied her diagnoses. And passed away ten days from the date she was diagnosed. She was 69 years old and from Roane County. Our family did have an autopsy done on her and we have recieved the first phase of it confirming she did have CJD. We have learned of a Man in Rockwood having it and a lady from Kingson in ParkWest with it now. This is a very rare disease and I do believe their has to be a link in Roane County causing it. This disease is horrible and devasting to the family. There is no treatment for it and it progesses very rapid. There is no stopping it or slowing it down once the patient gets it. My mother has contacted the CDC this morning.

And another:

Actually, my father in Roane County was diagnosed with Creutzfeldt-Jakob last week. Our family was contacted by a lady whose mother passed away earlier this year of the same disease, also in Roane County. Furthermore, a family friend visited our house today and told us of yet another diagnosis of a citizen of Roane County, who is currently hospitalized in Knox County. This is devastating both for the families involved and for our county, and considering the statistical rarity of the disease, it seems very strange that three people residing within the same geographical area were diagnosed if the disease is, in fact, acting sporadically. This disease is a monster, and further investigation to identify a possible common link would be extremely beneficial.

Roane County is on I-40 in eastern Tennessee.  Its farms and several small towns are home to about 52,000.  Go here for the WBIR report.

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 Yale University scientists have discovered a notable link between Alzheimer's proteins and Mad Cow disease, also known as Creutzfeldt-Jakob disease (CJD).

The prion protein, which is associated with the causes of CJD, is normally used in the brain to maintain brain health but can contribute to nerve damage should it get tangled up with amyloid-beta, another protein known as the chief suspect of causing Alzheimer's disease.

Stephen M Strittmatter, the senior author of the study and the Vincent Coates professor of neurology and director of cellular neuroscience, neurodegeneration and repair at Yale, said the discovery was somewhat of a "black box".

He continued: "We have known that amyloid-beta is bad for the brain but we have not known exactly how amyloid-beta does bad things to neurons.

"They start the cascades that make neurons sick."

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