9.21 Mad Cow Disease - An Insider Story

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21. Spongy Brain - Update 13 Septr 2021

In the 1920's Drs Creutzfeld and Jakob separately describe a sub-acute (months to year) brain disease. The patients develop what seemed a rapid Alzheimer's with weird eye symptom, loss of directional ability, bad handwriting, and died not long after. Most striking is a brain Swiss-cheese like, full of holes, and looking like sponge, so spongiform encephalopathy (SE). It is named Creutzfeld-Jakob Disease, CJD.

     As the 1930's saw no cause for CJD and it was rare, it is classed as minor dementia, filed and fairly forgot.

     Next, shift to the wild Fiore headhunter of New Guinea among whom in the 1950s, a scientist, D. Carleton Gajdusek, noted the rapidly fatal Kuru (Native for “the staggers”). The brains show the SE and the victims are women or children involved in preparing brains of dead warriors for ritual eating. This suggests a transmitted slow factor. So in 1966, Gajdusek injects Kuru brain substance into the brains of lab monkeys and after 2 years the monkeys come down with kuru. (And Gajdusek walks off with a Nobel Prize in 1976) Because tests for bacteria are negative, it is assumed due to a slow virus. What becomes more exciting is CJD caught from corneal transplants or from injections of growth hormone that contain pooled pituitary brain tissue or from metal rod brain implants, and, more frightening, because shades of AIDS, from blood transfusion. Somewhere, somehow, it seems, an infecting germ must exist.

     The slow virus idea runs to ruin. (But still mentioned as cause in 2021, page 224 right column, 12th ed. Kaplan & Sadock’s Synopsis of Psychiatry.) No researcher succeeds in viewing the virus on electron microscopy of brain tissue and anti-viral chemical procedures do not deactivate the factor.

     In 1967, a mathematician, John Stanley Griffith, suggests - Griffith JS (1967) Nature (London) 215, 1043-44 -  that the infecting agent may be a protein molecule capable of self-replication. In 1982, a young neurologist, Stanley Prusiner, makes a more research-based, detailed hypothesis that CJD, Kuru and like SE diseases are not due to a virus but are caused by a brain protein molecule. a PRION (proteinaceous infectious, and the "-on" to signify ‘particle’). The idea is a new concept because DNA dogma has it that only a nucleic acid (RNA/DNA) can reproduce itself. The hypothesis still has skeptics but now 38 years and one more (1997; JS Griffith died in 1972) Nobel Prize. One part of it now has proof from a newly discovered family of prion protein (PrP) that is common to all Spongiform Encephalopathy in human and other animal. The PrP protein is a variant of normally produced brain protein whose function is unknown. It gets overproduced because of its unique molecular structure and the brain cell has not evolved a way of getting rid of it so it piles up as folded molecules and that folding kills the neuron. And when enough neurons die, the SE illness becomes evident. Prion protein, as all proteins in living things, is produced on an RNA template, and the gene for PrP and its normal family of proteins has been identified on the short arm of chromosome 20. This explains the 15% of CJD that is purely genetic passing from affected parent to child as recessive gene condition and attacking brain with symptom at earlier age than acquired CJD.

     Now enter Mad Cow. The Spongiform Encephalopathy had been known in other animal – scrapie in sheep -and it also occurs in mink, and the 1980s saw the case of SE in a UK cow that went mad; ergo Mad Cow Disease (MCD). Also since the 1990s Chronic Wasting Disease (CWD) has been known in deer and elk in 10 western states centered about Fort Collins Colorado USA. (Eaters of deer and elk beware!) And it has even spread to stray cats! Also health food store purchasers of “velvet antler,” sold as nutritional supplement, and Asian users of “velvet Viagra,” a popular aphrodisiac should beware – because both are made of Midwest elk horn from the CJD area and may transmit a CJD variant to humans who eat one or the other.

The breakout of cow madness in the UK started with the introduction of cow feed that had sheep droppings. It is supposed that the scrapie prion passed through the intestinal tract blood barrier of the cow and crossed the species barrier to establish the new disease. Since the involved UK cows are used for beef-eating, the question arises: Could beef from cow transmit SE to human via steak or hamburger? Since a few British beef eaters have come down with CJD traced to eating mad cow, patients with spontaneous CJD are being asked about pre-illness eating habits. And it turns out that CJD is more common in hunters who cook and eat prey in field, and also it is common in populations that love to eat cow brain.

About CJD: It strikes in age 50s (but average age is getting younger). Transmission is through material contaminated with SE tissue or blood containing the PrP. It strikes the intellect by destruction of neurons in cerebrum and it disorders muscle coordination by its destruction of neurons in the cerebellum. The brain damage comes from the uncontrolled Prion Protein disordering the brain cell.

Symptoms are loss of intellect, loss of coordination with veering to one side, and eye signs. The CJD patient complains of brightness of neon light and inability to fix gaze on one point leading to shifty, unclear vision. An early symptom in one who was previously a heavy reader is loss of reading power. An unforgettable sign is myoclonus – a startle jerk. Here is one description by witness: “He tried to pick up a large glass of milk at supper. His arm flew up, and the milk went into a big arc and splashed on the floor, but he never let go of the glass.” Another: “… his left arm flew up, and he put it down and said nothing.”

I had chances to get data on several CJD deaths. All were outdoors men, hunters and eating squirrel including brain. One constantly drank un-pasteurized goat milk. Three of four had 20-year history of chronic fatigue syndrome. Two had double attack of herpes zoster shingles preceding CJD. All the men's wives tell of being first made aware something serious was going on by noticing husband was losing ability to drive straight. As disease progressed, each had to sign husband's checks because of the worsened handwriting. And in every case, CJD was misdiagnosed as neurosis and depression, then thought to be Alzheimer's and, finally, months before death, diagnosed as CJD by eye doctor because of typical eye symptom or sign.

    Spongy brain is one more reason not to eat meat.

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