Abstract Details

Huntington’s disease is a progressive neuropsychiatric disorder that typically presents between 30-50 years of age, with most of our understanding of the disease being defined by this group. Despite increasing prevalence, few studies have looked at Huntington’s Disease patients with onset >59 years old.

To further expand our understanding of the clinic presentation and disease course of Late-onset Huntington’s disease (LoHD).

Retrospective chart review was performed on 38 patients identified to have LoHD, defined as best first symptom onset >59 years old. We collected information about first symptoms, age at symptom onset and diagnosis, CAG repeat length, family history, and disease course.

Of the 38 patients, 52.6% were female, average age at diagnosis was 70.47, 12 were deceased with average time from diagnosis to death of 7.8 years. Average CAG repeat length was 40.3 (range 36-43), with a negative correlation between age of onset and CAG repeat length (r= -0.28). First clinical symptom was motor in 55.3% with chorea, with or without gait disturbance, being most common. Four patients had presymptomatic testing, all with clinical diagnosis made within 2 years of symptom onset. Average presenting UHDRS was 32.2, with 52.6% being stage 1. Positive family history was present in 71%, with a sibling being the affected member 55.6% of the time. The Average time between symptom onset and diagnosis in those with a known family history versus those without was 2.69 and 4.81 years respectively (p = 0.0857).

Our findings suggest that, in addition to awareness of symptomatology, family history remains a vital clue to diagnoses. Our findings demonstrate a potential trend towards fewer years between symptom onset and diagnosis in those with a family history. A detailed family history may allow for earlier diagnosis, treatment, counseling, and opportunities for presymptomatic testing in family members of patients with Late-onset HD.