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Introduction: Pure progressive amnesia, a form of progressive focal cortical atrophy is thought to represent the early stages of a rare form of Alzheimer's disease (AD). This syndrome is characterized by the insidious and slowly progressive breakdown of memory, in the absence of a significant impairment in other cognitive domains or in the realm of behavior. The aims of the present study were to contribute to the characterization of this poorly documented type of amnesia, to compare it with other forms of amnestic syndromes resulting from lesions to the medial temporal lobes and to discuss its potential pathophysiological basis., Patients and Method: We carried out three single case studies in patients presenting with pure progressive amnesia. They all underwent a neuropsychological evaluation that included an extensive assessment of spatial and recognition memory, along with brain magnetic resonance imaging and a cerebral blood flow study., Results: All three patients had a severe deficit in the storage of context-free information, along with a severe visual recognition memory impairment, as previously reported in a case study on a patient with pure progressive amnesia (Cognitive Neuropsychology 23 (2006) 1230-1247). Yet, spatial memory remained well preserved, and patients maintained totally independent in everyday life. In addition, a significant atrophy of the medial temporal structures was found., Discussion: This specific pattern of impairment differs from other types of amnestic syndromes after medial temporal damage and raises the question of lesional topography, as well as possible compensatory phenomena. We suggest that pure progressive amnesia results from selective damage to the ventral subhippocampal route into the hippocampal formation leading to impaired context-free memory. Spatial memory may remain intact because the dorsal parahippocampal route into the hippocampus remains functional. Pure progressive amnesia may contribute to a better understanding of the neural systems involved in declarative memory and provide a better understanding into the nature of the memory impairment that characterizes the initial stages of AD.

Introduction: The term "autobiographical memory" (AuM) refers to contextually bound experiences that occurred in a specific time, place, and affective setting. AuM is a component of memory commonly impaired in amnestic disorders. Alteration occurs rarely in isolation but rather in the setting of a larger memory impairment. Isolated AuM deficit is a controversial clinical entity, however, recently reported in the context of temporal lobe epilepsy. This study aims at characterizing this poorly documented clinical syndrome and at discussing its potential pathophysiological basis., Patients and Methods: We studied a group of three subjects with a history of pharmacosensitive epilepsy and severe AuM complaints. They all were submitted to a neuropsychological evaluation that included an extensive episodic memory assessment, along with wake/sleep electroencephalogram (EEG) and brain magnetic resonance imaging (MRI)., Results: We observed the following findings: preserved autonomy and intact global cognitive functioning; normal performance to standardized episodic memory assessment, contrasting with decreased performance to specific AuM evaluation; frontal and/or temporal epileptic activity on EEG; and normal structural brain MRI., Conclusion: We reported on a group of patients exhibiting selective impairment of some components of personal memory, associated with interictal frontal and/or temporal abnormalities on EEG. To account for these findings, we hypothesise that interictal epileptic-related activity is impeding long-term consolidation or storage of autobiographical material.

The perirhinal cortex is a structure that lies within the medial temporal lobe. In the present paper, we review current knowledge of the anatomical boundaries and functional correlates of this structure. In the past decade, numerous animal studies have attempted to understand the contribution of the perirhinal cortex to memory. Taken together, they suggest that the perirhinal cortex is crucially involved in recognition memory. This function appears to be independent from those assumed to be subserved by the hippocampus. In humans, data are scarce but tend to corroborate results found in the animal literature. The perirhinal cortex appears to support context-free (non-episodic) knowledge, such as general knowledge about the world and "item-specific" memories. Models of declarative memory that take into account the specific contribution of the perirhinal cortex are discussed, along with their potential application to early cortical neurodegenerative disorders.

Progressive focal cortical atrophies are degenerative conditions characterised by the insidious onset and gradual exacerbation of an impairment in a single cognitive domain related to circumscribed cerebral atrophy. Several focal cortical syndromes with deficits in the realm of cognition are reviewed: progressive impairment of language (primary progressive aphasia), speech (progressive anarthria), semantic memory (semantic dementia), episodic memory (pure progressive amnesia), vision (progressive perceptual or visuo-spatial deficits) and gesture (progressive apraxia). These conditions are histologically heterogeneous and can be associated with focal non-specific neuronal loss and gliosis with some spongiform changes (non-specific lesions), pathological features of Pick's disease (inclusion bodies and swollen neurones) or Alzheimer's disease (AD) (senile plaques and neurofibrillary tangles). A relationship between neuropsychological profiles and lesional types emerges from this review of the literature. Non-fluent primary progressive aphasia, semantic dementia and progressive anarthria are usually associated with non-specific lesions and Pick-type pathology. Progressive disorders of episodic memory and progressive visuo-spatial deficits are more often related to AD. If adequate clinical characterisation can determine the underlying disorder, it appears even more important to establish the neuropsychological profile in patients with cortical degenerative disease. Progressive deficits of only one domain of cognition may well be due to preferential involvement of anatomically and functionally defined neural systems and could therefore be considered as "system atrophies". There remains no doubt that these syndromes are particularly well suited models for studies on the relationship between cerebral functions and their neural substrate.