Hereditary progressive chorea without dementia.
- 1 February 1988
- journal article
- case report
- Published by BMJ in Journal of Neurology, Neurosurgery & Psychiatry
- Vol. 51 (2) , 295-297
- https://doi.org/10.1136/jnnp.51.2.295
Abstract
A family with hereditary non-Huntington's chorea is presented. Transmission was autosomal dominant with variable penetrance. Chorea commenced in childhood and affected predominantly the head, face and upper limbs. Dysarthria appeared later, followed in two family members by elements of an axial dystonia. There was no intellectual impairment. Unlike previously described families, symptoms progressed steadily up to the eighth decade, causing considerable physical disability.Keywords
This publication has 7 references indexed in Scilit:
- Persistent rheumatic choreaNeurology, 1985
- Benign familial chorea: an association with intellectual impairment.Journal of Neurology, Neurosurgery & Psychiatry, 1984
- Benign (non-paroxysmal) familial chorea. Paediatric perspectives.Archives of Disease in Childhood, 1981
- Hereditary chorea without dementiaJournal of Neurology, Neurosurgery & Psychiatry, 1977
- Benign Familial Chorea With Onset in ChildhoodJAMA, 1973
- Benign, recessively inherited choreo-athetosis of early onset.Journal of Medical Genetics, 1969
- Hereditary Nonprogressive Chorea of Early OnsetNew England Journal of Medicine, 1967