Highlights
- •Clinical characteristics of late-onset of Huntington's Disease not well defined.
- •Late-onset patients present more frequently with gait and balance problems.
- •Overall motor and cognitive performance were worse, disease motor progression was slower.
- •Repeat size was significantly lower in the late-onset compared to common-onset.
- •Fewer late-onset patients had a positive family history compared to common-onset.
Abstract
Background
The frequency of late-onset Huntington's disease (>59 years) is assumed to be low
and the clinical course milder. However, previous literature on late-onset disease
is scarce and inconclusive.
Objective
Our aim is to study clinical characteristics of late-onset compared to common-onset
HD patients in a large cohort of HD patients from the Registry database.
Methods
Participants with late- and common-onset (30–50 years)were compared for first clinical
symptoms, disease progression, CAG repeat size and family history. Participants with
a missing CAG repeat size, a repeat size of ≤35 or a UHDRS motor score of ≤5 were
excluded.
Results
Of 6007 eligible participants, 687 had late-onset (11.4%) and 3216 (53.5%) common-onset
HD. Late-onset (n = 577) had significantly more gait and balance problems as first
symptom compared to common-onset (n = 2408) (P < .001). Overall motor and cognitive
performance (P < .001) were worse, however only disease motor progression was slower
(coefficient, −0.58; SE 0.16; P < .001) compared to the common-onset group. Repeat
size was significantly lower in the late-onset (n = 40.8; SD 1.6) compared to common-onset
(n = 44.4; SD 2.8) (P < .001). Fewer late-onset patients (n = 451) had a positive
family history compared to common-onset (n = 2940) (P < .001).
Conclusions
Late-onset patients present more frequently with gait and balance problems as first
symptom, and disease progression is not milder compared to common-onset HD patients
apart from motor progression. The family history is likely to be negative, which might
make diagnosing HD more difficult in this population. However, the balance and gait
problems might be helpful in diagnosing HD in elderly patients.
Keywords
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Article info
Publication history
Published online: November 29, 2018
Accepted:
November 6,
2018
Received in revised form:
September 10,
2018
Received:
June 18,
2018
Identification
Copyright
© 2018 Elsevier Ltd. All rights reserved.
