CJD is a disease caused by a misfolding protein, called a prion. (PREE-on) Prion diseases are a group of rare and fatal brain diseases which occur in both humans and animals. In humans, it is known as CJD. Cows get BSE, which stands for bovine spongiform encephalopathy. Deer and elk contract CWD, or chronic wasting disease. There is no cure or clinical diagnosis for CJD. There is no cure for any of the diseases in the prion family.

Wednesday, February 21, 2007

What are those jerky muscle movements called?

Today's lesson in CJD is about jerky muscle movements, or myoclonus. (Pronounce that my-oh-clone-us). This was also one of my mother's CJD symptoms and I remember exactly the moment I first saw it. Prior to the major onset of her symptoms, she was very tired and would be napping by 2 or 3 p.m. each day. (She worked from home and did get up at 4:30 a.m. when my dad got ready to go to work, but the early wake up time had never bothered her before then).

My mom was sleeping in my dad's recliner and I could see her left leg moving. It was twitching and it kind of had a little bit of a bounce to it but the movements were very tiny. I thought my mother was dreaming she was running or something to that effect. What I saw was myoclonus.


Do you know how your body gets that last little jump right before falling asleep? That has scared the hell out of me for the last two years because it always makes me afraid I am having the beginnings of CJD myself -- a totally natural fear since my mom's CJD was familial.

But guess what? That's perfectly normal and everybody does it. That's according the NINDS, which has some pretty helpful information about myoclonus.

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