Cortical basal ganglionic degeneration

Cortical basal ganglionic degeneration is a very weird disorder, very weird disorder. It is the only movement disorder in which it behooves you to do a sensory examination and a cortical sensory examination. It has been associated with these weird, sort of alien limb things, cortical sensory deficits. Very weird movements of the arm. So you ask them to do some kind of apraxia test and they will sort of do some kind of weird thing. “I’m trying, Doc, I’m trying.” Virtually no cortical sensation in this hand. Strictly unilateral. Often, if you have a movement disorders practice and you get a basal ganglionic with pain, run the other way. I’ll tell you a case. Here’s what they do. I can’t go to a movement disorders conference anymore because they are just too long. You get these movement disorders guys and they bring their video tapes in, like half an hour video tapes. And they say, “I want to present a case of cortical basal ganglionic degeneration with pain.” Groan. Why? Because the pain is this deafferentation pain. So terrible pain, cortical deafferentation pain. So the guy – completely neurologic, completely straight-faced, completely like it’s no problem whatsoever – says, “Well the pain was intractable to the usual interventions, including 25 mg of Elavil and a variety of analgesic preparations and several other antidepressant trials, and therefore we opted to amputate the hand.” Oh, that’s good. So there’s this hush from the crowd. Because we all know how these central pain things can be and you try everything on the face of the earth, and so they amputate the hand and now the pain continues. Now it’s here in the stump. So what do they do? They amputate there. It’s like a Monty Python thing. So it’s just amazing and they guy is still telling us, so what are you going to do? A hemi-body-ectomy? But it’s true. Cortical basal ganglionic is a strange disorder and it’s associated with temporal parietal perfusion deficits on Spectra-PET. It’s widespread involvement on that side, characteristic presence of the achromatic neural inclusions in a variety of places in the involved hemisphere. And it has pathology that overlaps with Pick’s. With Pick’s disease. That’s at the level of PATH now, not at the level of frontal atrophy-sparing superior temporal varix. Not at that level, at the level of what it looks like under a microscope. Cheap Canadian Pharmacy

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