Impaired face-like object recognition in premanifest Huntington's disease.

Progressive striatal atrophy has long been considered the pathological hallmark of Huntington's disease (HD), but is it now recognized that malfunction and degeneration of posterior-cortical territories are also prominent characteristics of the disease. The limited knowledge about the functional impact of these posterior-cortical changes could be partially attributed to the lack of sensitive measures to capture them. We hypothesized that early malfunction of specific territories of the ventral visual pathway in premanifest HD would lead to difficulties in the recognition of complex stimuli and to differences in their neurophysiological correlates. To test this idea, we used an object, face and face-like object recognition task to be conducted during an electroencephalographic recording. Compared to healthy-matched controls, premanifest participants showed a significantly increased number of recognition errors in the face-like object condition. Moreover, premanifest participants showed a dramatic decrease in the N170 component elicited for the face-like objects. This N170 decrease was significantly associated with the number of recognition errors and with severity of apathy and global cognitive performance. The lack of differences in other clinical and cognitive measures supports a selective deficit in recognition of face-like objects and their neurophysiological correlates in premanifest HD. These deficits occurred in participants up to 15 years before the estimated time to disease onset and correlated strongly with cognitive and behavioral measures known to be sensitive to HD progression. This finding highlights the existence of selective visuoperceptive deficits years before motor-based onset of HD and emphasizes the need to develop sensitive measures to capture early visual system changes in this population. Assessing the integrity of the visual cortex and its related functions in HD could help to identify early markers of disease progression.