MDS 2011

Apathy in Huntington’s disease from an  Argentinean series of cases.

Authors: Bottini Bonfanti A, Persi G; Parisi V; Gatto EM.

Background:

Huntington’s disease (HD) is a progressive neurodegenerative disease characterized by involuntary movements, cognitive impairment, and neuropsychiatric symptoms including depressed mood, anxiety, irritability and apathy. Prevalence of apathy in HD has been estimated from 34% to 76%. Apathy has been linked with frontal dysfunction and cognitive decline and cause a significant burden for HD patients (HDp) and caregivers.

Objective: To assess the prevalence of apathy in a series of Argentinean HDp and it relationship with depression, executive function impairment and nCAG lenght.

Patients and methods:

Twelve HD outpatients of the Institute of Neuroscience Buenos Aires compose the sample. Written informed consent was obtained from all participants prior to study procedures. All but three individuals have a genetically confirmed diagnosis of HD. Demographic and epidemiological data were analyzed. An extensive battery of test was performed including: INECO Frontal Screening (IFS), Mini Mental State Examination, Apathy Scale (AS) and the Beck Depression Inventory.

Results: The sample included 8 women (66.67%) and 4 men; mean age 48.58 12.23ys; mean age at onset 40±8.89 ys. Mean nCAG repeats in expanded allele were 43.11±2.31. Apathy was present in 2 individuals (16.67%) whereas only 1 patient showed depression (Beck =18) (8.33%). Apathy was associated with depression in one case. All patients but one (91.67%) has an IFS impairment that reflects a frontal cortex involvement.  We failed to demonstrate a correlation between apathy and IFS impairment as well as a correlation between apathy and nCAG lengths.

Conclusions: Although, this is a small sample, our results agree with previous reported in the literature. Frontal impairment is very frequent in HDp; apathy is more prevalent than depression and both disorders appears separable and independently. Due to the major effect of apathy on daily functioning and quality of life it seems a new interesting therapeutic target to explore.