DEMENTIA TYPE-CJD

SUMMARY

1. Creutzfeldt-Jakob disease (CJD) is a very rare infectious prion diseases, fatal in < 1 year in > 90%.

2. Types: sporadic (sCJD, 95%), familial (~ 5%), iatrogenic (iCJD), and variant (vCJD). Usually, sCJD presents at ages 55-65.

3. vCJD is believed (but not definitively proven) to be caused by the prion that causes "mad cow disease" - it appears that the prion jumped species and now infects humans.

4. iCJD is caused by receipt of infected human tissues, hormones (growth hormone, gonadotropins, dural grafts, corneal or liver transplants), or exposure to contaminated surgical instruments.

5. Familial CJD has genetic associations.

FEATURES
6. CJD develops as a rapidly progressive dementia (weeks) with characteristic startle myoclonus (response to loud noises/startle).

7. The early stages of the neurologic disease are characterized by changes in behavior, emotional response, and intellectual function, often followed by ataxia and visual distortions, psychosis.

Reference(s)

Wilkinson, I., Furmedge, D. and Sinharay, R. (2017). Oxford handbook of clinical medicine. Oxford: Oxford University Press. Get it on Amazon.
Feather, A., Randall, D. and Waterhouse, M. (2020). Kumar And Clark’s Clinical Medicine. 10th ed. S.L.: Elsevier Health Sciences. Get it on Amazon.
Hannaman, R. A., Bullock, L., Hatchell, C. A., & Yoffe, M. (2016). Internal medicine review core curriculum, 2017-2018. CO Springs, CO: MedStudy.
Therapeutic Guidelines. Melbourne: Therapeutic Guidelines Limited. https://www.tg.org.au [Accessed 2021].