Chapter 16
Essex Falls, New York
Jack and Laurie were thrilled to return to Ted’s Diner, and walking in with Dr. Bob, as he was generally known around town, was like walking in with a movie star.
Just about everyone in the restaurant came up to say hello, including Ted himself.
It turned out the elderly man with the not-so-clean apron manning the grill and the fryer was none other than Ted.
Also, one of the older women servers in the matching black dresses turned out to be his wife, Charlotte.
“I’m impressed with the service you get here,” Laurie said after Charlotte had seated them at the only freestanding four-top table in the restaurant and taken their drink orders.
The table was situated in an alcove at the very front of the restaurant looking out at Main Street.
Apparently it was a table reserved by Ted for the town’s celebrities, like Dr. Bob.
“Ted and Charlotte’s fifth child had some medical issues,” Bob explained. “They ended up having to spend more time with me than they would have preferred.”
“Bob is being much too modest as usual,” Melanie said. “He managed their child through a very difficult and serious episode of leukemia, eventually achieving a total remission.”
“That’s truly wonderful, Bob,” Laurie said with sincere respect.
“Well, you do what you can,” Bob said offhandedly while blushing. Then: “What’s everybody going to have? My treat!”
They all ordered hamburgers with several portions of French fries for the table.
“Laurie, I want to thank you for walking us through the autopsy this morning,” Bob said the moment the waitress left.
He had to talk loudly to compete with the overall noise level in the old diner with its tile floor and embossed metal ceiling.
“I certainly could have benefitted from such a tutorial before I did my first autopsy. Unfortunately, I didn’t know what I didn’t know. ”
“Autopsies generally are not difficult,” Laurie said. “The important thing is to be thorough and consistent.”
“I get it,” Bob said.
“Let’s talk about neurodegenerative disease,” Jack suggested. “Or is it too noisy in here? Or is it too depressing over lunch? What does everybody think?”
“I don’t mind,” Bob said. “Do you mean Alzheimer’s disease in particular?”
“No, I’d like to discuss neurodegenerative disease in general, of which Alzheimer’s is a prime example.
I would have signed Stanley Kramer out as probable Alzheimer’s disease pending further testing, but you have said that today’s case looked like and clinically resembled your two previous cases, Barker and Hughes, and both of them tested negative.
In my book that means Stanley is probably going to test negative, too, wouldn’t you all agree? ”
Everyone nodded.
“So where does that leave us?” Jack questioned.
“I’d have to say ‘up-the-river-without-a-paddle.’ Then, looking directly at Bob he added: “Tell me again! In the New York Times article you cited involving the mysterious outbreak up in New Brunswick, Canada, what brain disease were they worried about?”
“Creutzfeldt-Jakob disease, at least initially,” Bob said.
“Whoa,” Jack said. “Meaning mad cow disease, which certainly caused one hell of a stir when it popped up in England some forty years ago. What was the ultimate outcome in New Brunswick?”
“Strangely enough, the New Brunswick cases ended up testing negative for mad cow disease, similar to our cases testing negative for Alzheimer’s.
Ultimately and luckily, the New Brunswick outbreak then just petered out and stopped over several months.
Unfortunately, not before quite a number of people sickened and died. ”
“It’s encouraging that it at least stopped,” Jack said. “So, there was no ultimate diagnosis made in that episode.”
“Certainly not when the article was written,” Bob said. “And I never heard any follow-up, which I’ve looked for, so I guess, no, a final diagnosis wasn’t ever made.”
“Too bad, because it seems we’re facing a remarkably similar situation,” Jack said. “The difference is that here it doesn’t appear like whatever is going on is stopping, at least not yet.”
“It’s definitely not stopping,” Melanie said.
“As I’ve mentioned, two of the patients scheduled for this afternoon have some of the same initial symptoms as the first dozen, a mix of muscle spasms, memory loss, gait problems, and personality changes, and both these new patients are particularly young.
One is still in his twenties, the other in her thirties.
I won’t be at all surprised if later on today we have two more significantly younger cases to add to our series. ”
“All the more reason we have to come up with a diagnosis,” Jack said. “People in their twenties don’t develop Alzheimer’s.”
“That’s not true,” Laurie interjected. “I remember reading that a nineteen-year-old boy in China was diagnosed with Alzheimer’s a year or two ago.”
“You are joking,” Jack said, staring at Laurie.
“I’m not,” Laurie said.
“Well, if it’s true, it’s certainly an outlier,” Jack said.
“Besides, according to NMS Labs, we’re not dealing here with Alzheimer’s, so it doesn’t matter.
But we are dealing with a neurodegenerative disease.
That’s perfectly clear considering the symptoms the patients are experiencing and especially with our having just had an opportunity to look at Stanley Kramer’s brain.
And what we could see grossly, which I admit was on the subtle side, will undoubtedly be more pronounced when we get to look at the histology. ”
“So, what’s the point you are trying to make?” Bob asked, staring at Jack, as were Laurie and Melanie.
“My point is that we have to go back to basics if we are going to have any luck solving this current outbreak of dementia and give up trying to put a convenient, familiar name, like Alzheimer’s or mad cow disease, on it.
My sixth sense is telling me it’s neither, considering its unusually rapid course. ”
“What do you mean ‘go back to basics’?” Laurie questioned.
Just as Jack opened his mouth to explain, the waitress appeared, and she was defying gravity.
Somehow she was carrying four hamburgers, two large French fries, and a generous container of Heinz ketchup.
Wasting no time and needing little help, she was able to distribute the hamburgers and set the ketchup and French fries onto the table.
After asking if there was anything else anyone wanted at the moment and getting noes for an answer, she took her leave.
“I’ll let us all enjoy our burgers,” Jack said before taking his first welcome bite and enjoying it thoroughly.
Just a few minutes later, after inhaling his hamburger, he pushed his empty plate away and leaned forward, forearms on the table so he didn’t have to speak so loudly in the noisy environment.
“While you eat, I’ll carry on where I left off.
I’m sure all of you are aware that it is generally believed that all neurodegenerative diseases, including those that are being seen here in Essex Falls, are caused by a rather peculiar protein culprit, called a prion. ”
Jack purposely paused and stared at each of his lunch companions in turn.
Everyone kept chewing, staring back at him, hoping one of the others would say something.
He knew that no one felt all that comfortable talking about prions, even himself for that matter.
Although the name had been devised a bit more than forty years previously by taking the first two letters of the word protein and combining them with the last four letters of the word infection, he knew it was still a controversial or even contentious subject, since its basic tenet—of an inanimate protein being infectious—was so revolutionary.
Prior to the advent of the concept of prions, it was an accepted tenet of biology that for something to be infectious it had to involve genetic material either in the form of DNA or RNA and reside in a virus, a bacterium, or a fungus.
“I’ve heard the term but I couldn’t tell you much about it,” Melanie admitted when Jack looked at her directly. “So, you think they are causing this outbreak we are seeing here in Essex Falls?”
“The current theory is that all neurodegenerative diseases are caused by infectious prions,” Jack said.
“All I know about prions is that they are proteins mostly associated with the brain with an unknown function,” Bob said.
“The problem with them is that they can go haywire and start interrupting brain function, which happens in Alzheimer’s disease.
They cause extensive nerve cell death and ultimately death of the individual. ”
“They are definitely proteins,” Laurie added. “Infectious proteins, which means that somehow they have the ability to cause their normal, noninfectious brethren to become infectious and form fibrils inside neurons and plaques outside, and it happens in an exponential fashion.”
“All true,” Jack said. “Unfortunately, I’m afraid we are a case of the blind leading the blind.
My sense is that none of us know much about prions, which is obviously an enormous handicap if we are trying to understand, diagnose, and hopefully stop an outbreak of what must be a prion disease.
Let’s think about what we do know. Laurie, you are first up.
Tell us more about how prions work their black magic. ”
“I wish our neuropathologist, Christine, was here,” Laurie said. “She could fill us in a lot better than I.”
“True, but she’s not here,” Jack said. “Maybe we can get her to come for a night or two, or in a worst-case scenario, we can organize a Zoom. In the meantime, let’s pool what we do know. You said that prions are infectious proteins. Tell the group what you mean.”