Background Although apathy can be disabling in Huntington’s disease (HD) and seems to increase with disease progression, most patients, in contrast with their caregivers, do not complain about it.

Objective 1) To assess the presence and severity of apathy in patients with HD using a structured interview for patients and their caregivers.

2) To correlate the severity of apathy with disease duration, the presence of mood alterations and motor, cognitive and functional impairment.

Methods 30 subjects (16M/14F; 6 presymptomatic carriers, 24 patiens with early-intermediated HD) and 26 caregivers participated in the study after informed consent was obtained.

1) Demographic data from patients were recruited.

2) Apathy was assessed using the Lille apathy rating scale (LARS), which was administered to patients and their caregivers separately.

3) The Unified Huntington Disease Rating Scale (UHDRS) was used for motor, cognitive, functional and behavioural assessment.

4) Other assessments: Mattis Dementia Rating Scale, Hospital Anxiety and Depression Scale (HADS) and the SF-36 health survey.

Results LARS scores obtained from the patients differed from their caregivers. Abnormal scores were found in 4 presymptomatic patients. The scores obtained from their caregivers led to classify apathy as severe in 16 patients. We found that apathy correlated to disease duration and motor, cognitive and functional impairment, but not to depression.

Conclusions Apathy is frequent and severe in most HD patients and can be even present in presymptomatic cases. The severity of apathy increases with disease progression. The discordant scores between patients and their caregivers suggest the presence of low disease awareness in patients and reinforce the role of caregivers in the assessment of many clinical aspects of the disease.