LATE ONSET HUNTINGTON'S DISEASE AS A CAUSE OF DEMENTIA: WHERE SHOULD THE CLINICIAN'S INDEX OF SUSPICION LIE?
✍ Scribed by NICHOLAS HINDLEY; GAIL NORBURY; KIM JOBST; ELISABETH ROSSER; SUSAN HUSON; MARY-JANE PEARCE; ELIZABETH KING
- Publisher
- John Wiley and Sons
- Year
- 1996
- Tongue
- English
- Weight
- 378 KB
- Volume
- 11
- Category
- Article
- ISSN
- 0885-6230
No coin nor oath required. For personal study only.
✦ Synopsis
Our experience with two genetically confirmed cases of late onset Huntington's disease (HD) in a longitudinal dementia research study suggested that clinical misdiagnosis can easily occur. We therefore undertook genetic testing for H D in a further 84 elderly subjects, 81 of whom had come to postmortem; 75 subjects had dementia and nine were normal controls. A quarter of the demented group had demonstrated extrapyramidal symptoms in life but in none had H D formed part of the differential diagnosis. Although no genetically confirmed cases were found in this second group, the original cases serve as a reminder that late onset H D is a cause of dementia. Genetic confirmation should be sought when the condition forms part of the final differential diagnosis. Further studies conducted in the routine clinical setting are now required since it is in this environment that late onset HD is likely to be misdiagnosed in favour of other forms of dementia.