New Treatments for Movement Disorders. Part 7

Just a couple of very fatuous concluding observations. There are a number of therapeutic options in Parkinson’s disease and the fact that there are so many of them that we are talking about means that we haven’t found a cure. No surprise. We haven’t found the gene, we haven’t found the cure, but we are making some headway, I think. So that was my Parkinson’s talk. I’d love to get going on the movement disorder stuff.

The question is, why is it that people with parkinsonism, many different types, seem to improve with ECT? An awful good question. My rejoinder question is, why is it that ECT, what is the pathophysiology of ECT, helping depression? I don’t know nor do I understand why but it certainly is true. In fact, I’ve used ECT for the treatment of very recalcitrant Parkinson’s disease, but the real thing is that we don’t see it anymore. The epidemic of encephalitis lethargica, what, 1920, thereabouts. The last reported case of post-encephalitic parkinsonism in the 50’s? Something like that. Levodopa-responsive but weird. There were weird things in that post-encephalitic parkinsonism like – read those early descriptions -oculogyric crises with arrhythmo- mania. Think about that one for a second. So their eyes are off to one side, they are gyrating to beat the band like it’s an acute dystonic reaction. It’s most bizarre and that’s what post-encephalitic parkinsonism is all about. Very strange manifestations, is what I’m getting at. The path on these things … again, what are some of the other weird features? Tics, these oculogyric crises that I mentioned, sleep reversal, startle myoclonus, early dementia, all these kinds of things. The path on these things have Lewy bodies that you would expect but you see gliosis in midbrain nuclei. Perhaps associated with all the weird kinds of eye movement abnormalities that you see in that disorder. So I would underline sort of midbrain path in that. Also written about by the Oliver Sachs, at great length.
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The parkinsonism, dementia, ALS complex of Guam, which also goes by the much more poetical name of Lydego-Bodeg (?). I think the Lydego refers to the parkinsonism and the Bodeg refers to the ALS features of this disorder. Some people present early with motor neuron features, some people present early with parkinsonian features, but they all look like hell later on in life. Now, there has been some talk about putative neurotoxins associated with this disorder. I stress the word putative. Because there was much enthusiasm about these but it didn’t bear fruit. Two of them. What they did, of course, is they were looking at a plant, the cycad nut because they use it for flour to bake their bread and all that. So they looked at the cycad nut and thought they had found the neurotoxin in the cycad nut. They thought it was BMAA. This beta n-methylamine-L alanine. Then they also thought about cycasin, this methanol beta glucoside. So you will see those names or those terms associated with the disorder, although there is some controversy surrounding that so I’m not sure that would be a fair question. In that disorder, the pathology resembles Alzheimer’s disease much more so than Parkinson’s disease. So you see gliosis, you see tangles, you see plaques widespread throughout those specimens. Hard to distinguish, in other words, from Alzheimer’s disease. It would be very unfair of them to show you a picture of that and say, “Could this be ALS complex of Guam?”

This is the approach; know the disease, know a little bit about it, move on. Please do not feel that you have to have encyclopedic knowledge of all these things because you’ve got too much. You’ve already had your kiddie neurology stuff so you know how much you have to do. And please go through the process, especially for you guys who are starting your preparation early, generate your lists. Become familiar with lists and associations. That’s the way to go. You don’t have to know everything there is to know about Crabbe’s. You don’t have to know everything there is to know about Niemann-Pick. I tried, brief mental hospitalization, fine now. Please just go with associations, in my opinion. My feeling is that generate lists and associations.

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