conference that was held early on Fridays in the Basic Sciences building of the Medical School. Each week the conference was started by the chief neuropathologist. He gave in a nut shell a brief outline of the case for the audience. The group consisted of neurology and neurosurgery attendings and residents and generally too of several medical students rotating through those two services. The shown case was generally of a recently deceased person.
The detailed clinical history was read, generally no more than two pages, by one of the residents. Then an attending discussed the different possibilities of what may have been, in his judgment, the most likely, correct diagnosis. The chief neuro-pathologist would then follow, presenting in a large screen the slides of the tissues studied in the autopsy, telling the audience what the final, correct diagnosis was.
The case in question was that of a 29 year old woman who had had an 18 month history of generalized progressive incoordination and loss of balance till her death, having been bed ridden for her last 6 months. A friend of Dr. Martin and his senior, Dr. Jack Bear, had taken care of her for some time till she’d died in the neurology ward, and another in his department, Dr. Carlo Porky, had also known her. This last guy, a child neurologist himself, had been consulted in the case -though he usually limited his practice to those under age 20- because of his expertise in neurodegenerative disorders, generally the turf of child neurologists. Both came to the podium, both presuming that she had had some sort of hereditary progressive encephalopathy, or perhaps some form of rapidly advancing M.S. (=Multiple Sclerosis) or P.M.L. (=Progressive Multifocal Leuko-encephalopathy).
No other clinical possibilities were offered by others in the audience as the Neuropathologist, Dr. Collier, asked others to venture their opinion before he gave the final dictum. But in the long pause, the young Dr. Frank Martin got up to say:
“Why can’t this be a case of C.J.D., Creutzfeldt Jacob Disease?”
Dr. Jack Bear quickly got up reacting to that comment by a guy so junior to him:
“Frank, if this turns out to be C.J.D., I’ll give you my Med School Diploma!”
Well, well, well. It DID turn out to be C.J.D.! It is a fatal, progressive encephalo-pathy, supposedly caused by a ‘slow virus.’ We hear that to this date Dr. Martin still keeps Dr. Bear’s Diploma! [just a copy]. On the other hand, Dr. Carlo Porky appeared annoyed with Dr. Martin having been the first to arrive at the correct diagnosis without the benefit of the pathology slides. From then on he was never in good terms with him.
We hear that the tissue of such patient was sent to the pathologists of the NIH (the National Institute of Health) in Bethesda, MD, who concurred with the diagnosis of C.J.D. They further inoculated such tissue into a monkey, who came down, the poor fellow, with C.J.D. several months later!
And upon learning of the diagnosis of C.J.D., Dr. Bear did more detective work into the background of the unfortunate patient and he found out that approximately 6 months prior to the onset of her incoordination she had had a corneal transplant AND that the donor of such cornea had been the cadaver of a person who had died of C.J.D.!
Needless to say, the case, as the first ever of a human-to-human transmission of C.J.D. (via the transplanted cornea) was published in the world-wide, prestigious New Britain Journal of Medicine (name changed). The listed authors were Dr. Bear, the Pathologist Dr. Collier and two neuropathologists of the NIH. There was no mention of the only physician -Dr. Frank Martin- who had been the one to arrive at the correct clinical diagnosis before the pathologist! Ah well…‘I was not meant for fame.’
Two other cases of C.J.D. were diagnosed in the subsequent years, both presented in similar conferences to the University Neurology and Neurosurgery staff. Onehad been missed completely by the main attending clinician AND by the neurologist who had discussed the case in the neuro conference, Dr. Carlo Porky. This guy had given a brilliant, exhaustive list of clinical possibilities but missed C.J.D. completely; only to be given again correctly by a raised hand in the audience, that of Dr. Frank Martin, before Dr. Collier gave the final word with his pathology slides.
You can imagine how really pissed off Dr. Porky was with Dr. Martin, who was not even part of the full time Faculty staff as HE was…
[We hear -the actual truth, not fictionalized for this novel- that at the point where the State officials (at the Office of Professional Conduct, OPC) were entertaining disbarring Dr. Martin, that Dr. Porky contributed decisively to his demise by voting him out of his Department Faculty Staff and Hospital privileges. Apparently -this writer hears- because Dr. Martin did not want to share night call with Dr. Porky! Thus his fateful sack was being sealed!]
CHAPTER 7
Another Parallel Case Also Missed By Those In Academia
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