In response to yesterday’s blog entry, SLC asked:

“In the current controversy over embryonic stem cell research, some of the advocates have pointed to ALS as one of the conditions that might be amenable to treatments derived from such research. Given Dr. Novella’s neurological specialty, could he comment on this issue?”

I’d be glad to.

ALS and other motor neuron diseases are ones for which stem cell therapy holds some hope and promise. Since these diseases result from the death of cells (motor neuron) the idea is that stem cells could be transplanted into the brains or spinal cords of patient and then coaxed into becoming neurons to replace the ones that are lost. It is further hoped that these new neurons will be healthy and not suffer the same fate as the neurons they are replacing.
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Yesterday I discussed the nature of disease, disorder, and syndrome – all as a prelude to the discussion of motor neuron disease, and the fact that it is not a disease. It is better understood as a clinical syndrome.

There are basically two types of motor neurons – the upper motor neurons which have their cell bodies in the motor cortex of the brain and send their axons down to the spinal cord to synapse on the lower motor neuron; and the lower motor neuron, which has its cell body in the anterior horn of the spinal cord and sends its axon out of the spinal cord to become part of a peripheral nerve and eventually synapse on a collection of muscle fibers. There are also interneurons that regulate motor function, but we won’t consider these for now.
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Famous British physicist, Stephen Hawking, recently announced that he plans to go to space. He will start by experiencing weightlessness on the “vomit comet” – a plane that can fly in a parabolic arc and provide about 25 seconds of free fall at a time. Stephen Hawking suffers from motor neuron disease – he lives his life in a wheelchair and at this point has barely any motor function. That he still dares to dream of going to space is a testament to his intellectual fortitude, courage, and will.

The story has prompted many questions to me (since this is one of my areas of expertise) about what the nature of his motor neuron disease is. Although I do not have access to Dr. Hawking’s personal medical information, I can discuss the issue in general – which turns out to be quite an interesting medical story.
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Below is an exchange between me and an e-mailer responding to this article I wrote about conspiracy theories. Although the specific topic is about the assassination of JFK, and in particular the head wound, you can see many of the elements common to all conspiracy thinking here. (To help, the e-mailer’s writing is in italics)

Laurie Philips

For a society supposedly formed to promote science and reason, I find your dismissal of the “back and to the left” evidence against Oswald in the JFK assasination astonishing! You seem to refute the idea by suggesting that the “jet” of brain matter coming out of the front would have more momentum than the bullet coming in the back… even though the matter recieves its momentum solely from the bullet! Therefore, the limiting case is no movement at all, where all the bullet’s momentum is transferred to the matter. It certainly could not gain more. What were you smoking when you came up with that one??? Please don’t spread such an obvious falsehood around, somebody might believe you.

There’s all kinds of other evidence, but it’s entirely irrelevant to prove that the official story is wrong. It doesn’t matter if you find a video of Oswald hanging out of the 6th story window with a rifle, firing away… Momentum is always conserved, there must have been a shot from in front of JFK therefore at least another shooter, therefore a conspiracy.
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Dr. Michael Gershon, a neuroscientist at Columbia University, has written a book entitled the Second Brain, about his specialty – the enteric nervous system (the nervous system of the gut). Dr. Gershon is an excellent scientist, and I applaud his attempt to popularize his work (something that more scientists should do), but I must say I think his characterization of the enteric nervous system as a second brain is unfortunate and confusing.

First some background. The vertebrate nervous system is divided anatomically into the central nervous system (CNS – the brain and spinal cord) and the peripheral nervous system (PNS – everything outside the brain and spinal cord, mainly nerves, but also including some neurons – nerve cell bodies). Functionally the peripheral nervous system can be divided into the sensory system, the motor system, and the autonomic system. The central nervous system has these components as well, but also has many more systems and functions. The autonomic nervous system can be functionally further broken down into the parasympathetic system, the sympathetic system, and the enteric system.
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I have received quite a few questions recently about a new migraine relief product called HeadOn – known for its annoying commercial that simply repeats the phrase “HeadOn, apply directly to forehead,” over and over.

Does HeadOn work? All that really need be said about this is that the product is homeopathic – that means it doesn’t work. The listed “ingredients” are diluted from between 6X and 30X (6X is a 1 in 10 dilution 6 times, or one part per million). That means you may have a stray molecule of potassium bichromate in there, but at 30X there likely will not be any golden seal. In my opinion, it should be considered fraudulent to list 30X of anything as an “ingredient”. (Their website vacuously informs us that by diluting the ingredients to such a degree, “their medicinal properties are released.”)
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Hope is a positive, and some might argue even necessary, human emotion, but false hope is its cruel and dark underbelly. No population is more in need of hope, and more vulnerable to false hope, than the desperately ill. Unfortunately we live in a world where false hope merchants prey upon the sick like sharks upon bleeding and thrashing fish.

There is just such a shark in China, one Dr. Hongyun Huang (not to be confused with disgraced Korean stem-cell researcher Hwang Woo Suk) who runs a clinic where he injects (or claims to inject) stem cells derived from olfactory sheath cells taken from aborted fetuses into the spines of those suffering from spinal cord injury or motor neuron disease. Dr. Huang claims (while simultaneous saying he promises nothing) that his treatment has resulted in miraculous cures of his patients. His clinic has lured the desperate from around the world, at a fee of $20,000 plus all associated expenses. He is abetted by a gushing and disgraceful press (such as in this Guardian article) who sensationalize his vision and courage, while cynically disparaging those skeptics in “western medicine.”
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A listener of my podcast, the Skeptics Guide to the Universe, sent in the following question:

“Okay, so chiropractic, acupuncture and reiki don’t work because the energy flows they are based on don’t exist. So here’s my question…how do we know they don’t exist? How do you test something like that?”
– Gregory Lynn

Mr. Lynn’s question is a reasonable one, and one I get quite frequently, but it contains a serious and common misconception – actually two. The first is that the burden of proof is on those who claim that a life force, or spiritual energy, does not exist. The second is that scientific methods can be used to test for the non-existence of something.
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On November 7th about a dozen United Airlines employees, including a few pilots, claim they saw an unusual disc-shaped object in the sky over O’hare International Airport, Chicago. The witnesses insist that what they saw was not a terrestrial aircraft of any kind, that it was anywhere from 6-24 feet in diameter, and that after hovering for a few minutes it vanished at impossible speed through the clouds.
Should we find it compelling that all of the witnesses insist that what they saw was real and that it was clearly visible? Should we lend credibility to the fact that some of the witnesses were airline pilots? How about the fact that the witnesses all seem sincere and were emotionally affected by the experience?

No. Because despite all this the witnesses suffer from a critical flaw that makes them unreliable as eye witnesses. They are human.
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A recent study suggests that about 60% of doctors in Scotland have prescribed either homeopathic (49%) or herbal (32%) remedies in the past year. Although only 5% of doctors were responsible for 50% of such prescribing, these are still alarming numbers.

Study author, Dr. James McLay, is quoted as saying, “The major problem with homeopathic preparations is the lack of scientific evidence that they are effective.”

While I agree that this is the major problem with herbal remedies, I would rank it #2 for homeopathic remedies. There is nothing far-fetched about herbal remedies. They are, put simply, drugs. They are just not very good drugs – they have not been purified, all their active ingredients have not been identified, and they are not properly studied. They are often prescribed with insufficient supporting evidence – sometimes based upon nothing but slick marketing.
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