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International audience; Disinhibition, mainly caused by damage in frontotemporal brain regions, is one of the major causes of caregiver distress in neurodegenerative dementias. Behavioural inhibition deficits are usually described as a loss of social conduct and impulsivity, whereas cognitive inhibition deficits refer to impairments in the suppression of prepotent verbal responses and resistance to distractor interference. In this review, we aim to discuss inhibition deficits in neurodegenerative dementias through behavioural, cognitive, neuroanatomical and neurophysiological exploration. We also discuss impulsivity and compulsivity behaviours as related to disinhibition. We will therefore describe different tests available to assess both behavioural and cognitive disinhibition and summarise different manifestations of disinhibition across several neurodegenerative diseases (behavioural variant of frontotemporal dementia, Alzheimer's disease, Parkinson's disease, progressive supranuclear palsy, Huntington's disease). Finally, we will present the latest findings about structural, metabolic, functional, neurophysiological and also neuropathological correlates of inhibition impairments. We will briefly conclude by mentioning some of the latest pharmacological and non pharmacological treatment options available for disinhibition. Within this framework, we aim to highlight i) the current interests and limits of tests and questionnaires available to assess behavioural and cognitive inhibition in clinical practice and in clinical research; ii) the interpretation of impulsivity and compulsivity within the spectrum of inhibition deficits; and iii) the brain regions and networks involved in such behaviours.

Some patients with organic amnesia show confabulation, the production of statements and actions unintentionally incongruous to the subject's history, present and future situation. It has been shown that confabulators tend to report as unique and specific personal memories, events or actions that belong to their habits and routines (Habits Confabulations). We consider that habits and routines can be characterized by multiplicity, as opposed to uniqueness. This paper examines this phenomenon whereby confabulators mistake multiplicity, i.e., repeated events, for uniqueness, i.e., events that occurred in a unique and specific temporo-spatial context. In order to measure the ability to discriminate unique from repeated events we used four runs of a recognition memory task, in which some items were seen only once at study, whereas others were seen four times. Confabulators, but not non-confabulating amnesiacs (NCA), considered repeated items as unique, thus mistaking multiplicity for uniqueness. This phenomenon has been observed clinically but our study is the first to demonstrate it experimentally. We suggest that a crucial mechanism involved in the production of confabulations is thus the confusion between unique and repeated events.

Introduction Signs of left unilateral neglect often occur after damage to the right hemisphere and entail a left–right imbalance in stimulus processing. Typically, neglect patients deviate rightward when bisecting lines. An underestimation of the left portion of the line and/or a right overestimation could explain this effect. Methods Here we dissected their respective contribution by asking participants to compare a vertical segment to a horizontal segment, either on the left or on the right. We also tested whether neglect patients exhibited the symmetry law, whereby normal participants underestimate symmetrically bisected lines as compared to asymmetrically bisected lines. Results Controls and patients underestimated symmetric figures. Depending on the degree of left–right horizontal competition, neglect patients underestimated left-sided stimuli or overestimated right-sided stimuli. Conclusions We concluded that two independent deficits contribute to neglect signs: a deficit in attentional orienting to the left, which can be worsened by left hemianopia, perhaps depending on impaired functioning of right-hemisphere attentional networks, and a tendency for attention to be captured by right-sided stimuli, possibly resulting from the activity of an isolated left hemisphere. Finally, the symmetry law was preserved in neglect patients, and thus appears to be driven by pre-attentive mechanisms.