DEMENTIA TYPE-HUNTINGTON'S CHOREA (HC)

SUMMARY

1. AD inheritance; heightened sensitivity of striatal dopamine receptors.

2. Triad of dementia, chorea, and psychiatric disturbances (including personality changes, depression & psychosis).

3. Memory is relatively spared, change in personality preceeds dementia.

4. Characteristic feature: motor impersistence.

5. Genetic counseling available, invariably fatal 15-20 years after onset (usually from aspiration pneumonia).

6. Tx: dopamine receptor blockers (Haloperidol, Phenothiazines); pre-synaptic dopamine depleters (Reserpine, Tetrabenazine).

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].