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CHAPTER 7 Another Parallel Case Also Missed By Those In Academia

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The other case was that of a very young girl who at age 16 began to have signs of progressive dementia. She was dead 18 months later. The local neurologist who had treated her had no idea what was going on. Her family took her about a year before her demise to Dr. Frank Martin, then a fairly new guy in town; he ventured as a possibility the diagnosis of ‘an early C.J.D.’ But her parents wanted to ascertain that possibility and tie all knots before she died, so they took her to ‘Mass Central’ in Bostin to see one of the real neurology gurus (a Dr. Fish?) in the country. Without pathology of her brain (not available till she would die) he was not sure. He mentioned in his notes that perhaps the girl was suffering an extremely young case of Juvenile Alzheimer’s or possibly even the rare C.J.D., but this had never been seen before age 20; so he could not tell for sure. Back home her parents stayed with Dr. Martin.

Dr. Martin took care of her, visiting her at the home of her parents monthly till she died. He was sure by then that this was C.J.D. and only C.J.D. even without a tissue examination!: Not only she had had the rapidly progressing dementia (and not ataxia like the previous case) but also brief, sporadic ‘muscle jumps’ that neurologists call myoclonic jerks – more typical of C.J.D. than of Alzheimer’s, mentioned by Dr. Fish.

When she finally died Dr. Martin was hoping -and ‘then’ so were her parents- that she would have an autopsy, so they finally would know for sure… Well, no!: Her parents, wanting to put everything behind, even without a definitive tissue diagnosis, changed their minds in the last minute and decided NOT to have the autopsy that initially they -and Dr. Martin- had counted on for sure!

It was early winter. The embalmed body was placed in reserve by the funeral home waiting for final burial in the spring when the ground would thaw… And just about then, Dr. Martin was trying to forget the case. He had given up the idea that a sure diagnosis would ever be known. But then…

Our unfortunate deceased young girl’s younger sister, out of the blue, had an unexpected seizure! All of a sudden her parents panicked: Something was going on in their family or household and THEN they needed a definitive diagnosis of the girl who had died a few months earlier. Dad called Dr. Martin to that effect. He again was willing to help. He took care of getting a court order to have her body ‘officially’ exhumed from the ground (even though strictly speaking she had not been buried but placed in a cooler). He contacted the funeral home and Dr. Collier, the pathologist. This guy was willing to come to the funeral home to carry out a limited autopsy there (of just her brain) and to have Dr. Martin assist him. And good towards the preservation of her tissues and the validity of an autopsy done so late, the remains were found in perfect condition. She looked as if she had just died the day before!

But well, contrary to expected, and to the parents’ and Dr. Martin’s dismay, after the autopsy was done and the brain tissue was examined carefully, the famous pathologist Dr. Collier was not sure! Damn! He presented the case as previously, to one of the neuropathology conferences. The many academicians in the audience had no idea (except Dr. Martin, but kept his mouth shut), and the various slides shown did not give anyone -or Dr. Collier- any precise diagnosis either!

But brain tissue samples of the girl’s had been sent to the NIH pathologists in Bethesda. These guys, as in the previous case, did their own examination of the tissue and of the slides sent. They were SURE that this was another case of C.J.D. as Dr. Martin alone had felt. They further inoculated tissue into a laboratory monkey who also came down (ha!) with C.J.D.! Case closed! Now the parents were sure and believed that Dr. Martin had been right all along. The guy in Bostin who also had thought of it as ‘one’ distant possibility (a ‘Dr. Fish’ or something) was informed.

Then came the time to prepare, write and publish the case of the young girl with C.J.D. -as the youngest case of C.J.D. in the world- in a prestigious medical journal. Both Dr. Martin and Dr. Collier wrote the paper. But being Dr. Martin Mediterranean-and English being his second language, his professional diction ‘not as good,’ Dr. Collier’s version prevailed. Dr. Martin’s paper had emphasized the interesting clinical history-so unusual in a young girl, and THAT was what made the paper important- while Dr. Collier’s version, as a pathologist, was mostly about the tissue findings (which he had missed anyway!)… Dr. Martin was not happy with the basic ‘laboratory tone’ of Dr. Collier’s version, this guy having had little to do with arriving at a clinical -or pathological!- diagnosis; but Dr. Martin gave up his version in favor of that of his senior ‘with better English’ and pretty color tissue photographs which he did not have.

But then another conflict aroused: On top of the wrong person’s version having prevailed to be sent to the New Britain Journal of Medicine, Dr. Collier had put himself as THE FIRST AUTHOR! (having placed Dr. Martin’s name and that of the guys at the NIH ‘following’). Usually the first author is the only one who counts in important scientific papers, and Dr. Martin felt that all the weight of the case and impetus to a final diagnosis had been only his (even if the words in the paper were Dr. Collier’s).

He protested. The doctors at the NIH were very familiar with such disputes of authors claiming but denied first authorship of many papers. They acted as mediators in the dispute and their opinion was in favor of Dr. Martin as first author – as opposed to Dr. Collier. They themselves would be named last. And that is how it was done; the paper was published in its first presentation by The New Britain Journal of Medicine. It gave Dr. Martin (real name changed again) no substantial glory since the nuances of his clinical story had been omitted, and it would later leave in the Faculty of the University against hima negative atmosphere that would haunt perversely to his demise…

Dr. Martin later inquired -as in the previous case where Dr. Bear had learned about the prior transmission by a corneal transplant- more details of the young girl’s preceding medical history. And as in the case of our young girl here with C.J.D., he found that approximately six months prior to the onset of her symptoms, that she had visited a local neurologist because of a blackout that he’d diagnosed as ‘just fainting.’ It turns out -interesting along the same lines of a possible human-to-human transmission, that he had done in the girl an EEG (=Electroencephalogram) using ‘needle,’ intradermal electrodes in her scalp! Even if ‘boiling’ of the needles had been carried out, it is known now that slow viruses -such as the one of C.J.D.- easily survive such – done then with alcohol or by ‘immersion in boiling water.’ Thus one can conjecture that our girl here also had ‘transmitted' C.J.D. by such needle electrodes! (that probably had been previously used in someone ‘with a fast neurological deterioration’ (perhaps diagnosed then as ‘another case of Alzheimer’s’).

These C.J.D. cases tell the reader first, interesting tales of rare medical conditions, and how the best specialists struggle to diagnose them; second, they tell us of a Dr. Frank Martin, foreign-born somewhere in Southwestern Europe who was at least as smart and sleuth diagnostician as the best of his US contemporary colleagues; thirdly, of a guy a bit outspoken who took risks as he ‘told’ his mind -to his own perdition eventually- some times clashing with his academic colleagues; these would eventually bear considerable weight -retaliatory- against him to bury him, as this author has mentioned in the previous case (chapter #6).

Who's Killing the Doctors? II

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