I. Appollonio et al., WHICH DIAGNOSTIC PROCEDURES IN THE ELDERLY - THE CASE OF LATE-ONSET HUNTINGTONS-DISEASE, Journal of geriatric psychiatry and neurology, 10(1), 1997, pp. 39-46
The typical adult-onset form of Huntington's disease (HD) is a clinica
l condition in which the latest advances of genetic research can be us
efully applied during the course of the diagnostic process; not so cle
ar are the guidelines for the much less frequent late-onset variant. W
e have recently seen three patients in their late sixties who had been
misdiagnosed for up to 10 years due to the apparently isolated, mild,
and slowly progressive nature of their hyperkinetic movements or cogn
itive disorders. Only after the results of DNA sequencing on a blood s
ample became available could the appropriate diagnosis of late-onset H
D be reached. By contrast, neuroimaging studies lacked sufficient sens
itivity and specificity. Appropriate neurogeriatric assessment in thes
e cases should follow specific guidelines and should always include se
lected high-technology procedures.