Semantic amnesia without dementia: documentation of a case

We described the case of a patient affected by a progressive semantic memory disorder associated with prevalent temporal lobe atrophy. This deficit seems to be pure in the sense that it has not been found to overlap with other cognitive deficits (intellectual, linguistic, perceptual, visuo-spatial etc.) for a long time. Furthermore, despite his impaired semantic knowledge, the autobiographical memory of the patient was largely intact. This case therefore represents a form of semantic amnesia without dementia, and supports the hypothesis that there is a partial distinction between semantic and episodic memory.

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Sommario Descriviamo il caso di un paziente con atrofia bilaterale (prevalentemente temporale) affetto da un deficit progressivo semantico. Non si evidenziarono (almeno per lungo tempo) altri deficit cognitivi e la memoria autobiografica/episodica, in contrasto a quella semantica, risultò sufficientemente conservata. Il caso rappresenta, perciò, una forma di amnesia semantica in assenza di deterioramento cognitivo e sostiene l'ipotesi della distinzione tra memoria semantica ed episodica.

     

Anterograde and retrograde amnesia in a person with bilateral fornix lesions following removal of a colloid cyst

AD, a 45-year-old man, presented with a severe and global anterograde amnesia following surgery for removal of a colloid cyst. Structural neuroimaging confirmed bilateral lesions to the fornix and a small lesion in the basal forebrain. Testing for remote episodic memory of autobiographical events, and for remote semantic memory of personal and public events, and of famous people, revealed that AD had a severe retrograde amnesia for autobiographical episodes that covered his entire lifetime, and a time-limited retrograde amnesia for semantic memory. Because the fornix and basal forebrain lesions disrupted major afferent and efferent pathways of the hippocampus, it was concluded that the integrity of the hippocampus and its projections are needed to retain and/or recover autobiographical memories no matter how old they are. By contrast, hippocampal contribution to semantic memory is time-limited. These findings were interpreted as consistent with Multiple Trace Theory, which holds that the hippocampal system is essential for recovering contextually rich memories no matter how old they are, but is not needed for recovering semantic memories.     

Charting the acquisition of semantic knowledge in a case of developmental amnesia

We report the acquisition and recall of novel facts by Jon, a young adult with early onset developmental amnesia whose episodic memory is gravely impaired due to selective bilateral hippocampal damage. Jon succeeded in learning some novel facts but compared with a control group his intertrial retention was impaired during acquisition and, except for the most frequently repeated facts, he was also less accurate in correctly sourcing these facts to the experiment. The results further support the hypothesis that despite a severely compromised episodic memory and hippocampal system, there is nevertheless the capacity to accrue semantic knowledge available to recall.     

Visual memory loss and autobiographical amnesia: a case study

Amnesia typically results from trauma to the medial temporal regions that coordinate activation among the disparate areas of cortex that represent the information that make up autobiographical memories. We proposed that amnesia should also result from damage to these regions, particularly regions that subserve long-term visual memory [Rubin, D. C., & Greenberg, D. L. (1998). Visual memory-deficit amnesia: A distinct amnesic presentation and etiology. Proceedings of the National Academy of Sciences of the USA, 95, 5413-5416]. We previously found 11 such cases in the literature, and all 11 had amnesia. We now present a detailed investigation of one of these patients. M.S. suffers from long-term visual memory loss along with some semantic deficits; he also manifests a severe retrograde amnesia and moderate anterograde amnesia. The presentation of his amnesia differs from that of the typical medial-temporal or lateral-temporal amnesic; we suggest that his visual deficits may be contributing to his autobiographical amnesia.     

Deficits in past remembering extend to future imagining in a case of developmental amnesia

Patient and neuroimaging studies report that the ability to remember past personal experiences and the ability to envision future personal experiences are interconnected. Loss of episodic memory is typically accompanied by loss of future imagining, and engaging in either activity recruits common brain areas. The relationship between episodic memory and future imagining is also suggested by their co-emergence in ontogenetic development. However, it is unknown whether a failure of one ability to emerge in early development precludes the development of the other ability. To investigate this possibility, we tested H.C., a young woman with amnesia of developmental origin associated with bilateral hippocampal loss, and demographically matched controls on an adapted version of the Autobiographical Interview using Galton-Crovitz cueing. In response to cue words, participants described both past personal events and imagined future personal events that occurred, or could occur, in near and distant time periods. Results indicated a parallel pattern of impairment for both past and future event generation in H.C., such that her narratives of both types of events were similarly deficient. These results indicate that mental time travel can be compromised in hippocampal amnesia, whether acquired in early or later life, possibly as a result of a deficit in reassembling and binding together details of stored information from earlier episodes.     

Avoidance conditioning in amnesia: a single case experimental study

We present a single case study of avoidance conditioning in a seriously amnesic patient with bilateral ischemic lesions of the brain. A four-path maze was used for conditioning. The procedure consisted in an aversive stimulus that the patient received every time he traced one of the four maze-paths connected to an electric shock generator with a stylus. Using a single case experimental design A-B-A, we found that the patient learned to avoid the path connected to the electric shock, although he never showed awareness of this. Our study confirms once again that conditioning is one of the residual learning capabilities in amnesic patients. Different neural systems are probably involved in conditioning processes.

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Sommario Gli autori descrivono uno studio su caso singolo di evitamento condizionato in un grave amnesico affetto da lesioni ischemiche cerebrali bilaterali. Per il condizionamento venne usato un labirinto a quattro vie ed il paziente riceveva uno stimolo avversativo ogni volta che con uno stilo percorreva una delle vie collegata a un generatore di schock elettrici. Attraverso un disegno sperimentale A-B-A è stato possibile dimostrare come il paziente imparò ad evitare la via connessa allo shock elettrico pur senza averne nessuna consapevolezza. L'esperimento rappresenta una ulteriore conferma che nei soggetti amnesici il condizionamento costituisce una delle capacità di apprendimento residue. è ipotizzabile che differenti sistemi neurali siano interessati nei processi di condizionamento.

     

Influence of patients' emotional state on the recovery processes after a transient global amnesia

Introduction

Currently, there is no consensus on the delay necessary to a complete recovery after a transient global amnesia (TGA). However, it seems that slight episodic memory disorders extend beyond 24 h. Although this impairment is probably a consequence of the TGA attack, other factors such as patients’ emotional state can intervene in the slow recovery process.

Methods

In a first experiment, we studied the dynamic of recovery processes after a TGA. Thus, we assessed the anterograde and retrograde components of episodic memory in 19 patients one day, one month and one year after the attack. In a second experiment, we examined the impact of patients’ emotional state on memory disorders, in using an original neuropsychological protocol (using material with emotional features) and an assessment of anxiety and depressive mood. This protocol was carried out in 19 other patients examined four months and one year after TGA.

Results

In the first experiment, we highlighted mild memory disorders affecting the anterograde component of episodic memory one day after the episode. In the second experiment, we showed these mild memory disorders could be detected several months after TGA. Moreover, patients who had the more depressive tendencies recognized the fewer items and those who displayed the highest level of anxiety supplied the fewer specific remote memories.

Conclusions

Our results showed that patients displayed very mild memory disorders several months after the episode of TGA, not affecting the daily routine. This impairment was influenced by patients’ emotional state, which could suggest that a high level of anxiety or depression can slow down the recovery. However, we cannot be sure that the deleterious effect of patients’ emotional state on their cognitive performances is specific to TGA. Other investigations are necessary to unravel this issue.     

Recollection and familiarity in dense hippocampal amnesia: a case study

In the amnesia literature, disagreement exists over whether anterograde amnesia involves recollective-based recognition processes and/or familiarity-based ones depending on whether the anatomical damage is restricted to the hippocampus or also involves adjacent areas, particularly the entorhinal and perirhinal cortices. So far, few patients with well documented anatomical lesions and detailed assessment of recollective and recognition performance have been described. We report a comprehensive neuroanatomical assessment and detailed investigation of the anterograde memory functions of a previously described severe amnesic patient (VC). The results of four previously published neuroradiological investigations (resting PET, qualitative MRIs, volumetric MRI and functional MRI) together with the results of two new investigations (voxel-based morphometry and magnetic resonance spectroscopy) are presented. The consistent finding across these different qualitative and quantitative examinations of VC's brain has shown that there is primarily structural and functional abnormality located selectively in the hippocampus bilaterally. Marked impairments in both verbal and non-verbal recall and recognition standardized memory tests were documented in the context of VC's intact cognitive profile and normal semantic memory. The results of five new experimental recognition memory tests tapping recollection and familiarity using verbal, topographical (buildings and landscapes) and unknown human faces memoranda revealed striking differential effects according to the type of stimuli used. A receiver operating characteristic analysis revealed that VC's recollective- and familiarity-based recognition processes were well preserved for unknown human faces. In contrast, recollective-based recognition for verbal and topographical material was at floor. Familiarity-based recognition was also impaired, significantly below controls for verbal and buildings memoranda and quite weak, although not reaching significance, for landscapes. These data suggest that the hippocampus is involved in recollective processes of verbal and topographical stimuli. It also plays an appreciable role in familiarity processes for these stimuli. However, recollection and familiarity of human faces appear not to depend on this region.     

Impaired memory for scenes but not faces in developmental hippocampal amnesia: a case study

Item recognition for unfamiliar faces and scenes was tested in Jon, who has developmental amnesia resulting from bilateral hippocampal pathology. Performance and confidence judgements in healthy adults showed that both tests were equated for difficulty and had similar receiver operating characteristics (ROCs). Jon's performance on the faces test was indistinguishable from the controls, in both sensitivity and the shape of the ROC curve. In contrast his performance on the scenes test was markedly poor, and his ROC was inconsistent with both a standard dual process (DP; recollection and familiarity) model and an unequal variance signal detection model of recognition memory. Jon's data were as well fitted as controls' data by a DP model that included two recollection parameters, but required counter-intuitive parameter values corresponding to normal recollection and impaired familiarity, which likely reflect an idiosyncratic use of confidence judgements when his memory for the material is weak. The results highlight a limitation in using ROCs to estimate recollection and familiarity in patients who may have developed compensatory strategies for material that they have difficulty remembering (scenes, but not faces in this case). Overall, these data are difficult to reconcile with domain-general accounts of the hippocampal role in memory, including dual process models and the declarative model. Instead, the data indicate that the hippocampus plays a preferential role in the processing of topographical memoranda over faces memoranda.     

Imagining fictitious and future experiences: Evidence from developmental amnesia

Patients with bilateral hippocampal damage acquired in adulthood who are amnesic for past events have also been reported to be impaired at imagining fictitious and future experiences. One such patient, P01, however, was found to be unimpaired on these tasks despite dense amnesia and 50% volume loss in both hippocampi. P01 might be an atypical case, and in order to investigate this we identified another patient with a similar neuropsychological profile. Jon is a well-characterised patient with developmental amnesia and 50% volume loss in his hippocampi. Interestingly both Jon and P01 retain some recognition memory ability, and show activation of residual hippocampal tissue during fMRI. Jon's ability to construct fictitious and future scenarios was compared with the adult-acquired cases previously reported on this task and control participants. In contrast to the adult-acquired cases, but similar to P01, Jon was able to richly imagine both fictitious and future experiences in a comparable manner to control participants. Moreover, his constructions were spatially coherent. We speculate that the hippocampal activation during fMRI noted previously in P01 and Jon might indicate some residual hippocampal function which is sufficient to support their preserved ability to imagine fictitious and future scenarios.     

Autobiographical memory and the self in a case of transient epileptic amnesia

Transient epileptic amnesia (TEA) is characterized by deficits in autobiographical memory (AM). One of the functions of AM is to maintain the self, suggesting that the self may undergo changes as a result of memory loss in temporal lobe epilepsy. To examine this, we used a modification of a task used to assess the relationship between self and memory (the IAM task) in a single case, E.B. Despite complaints of AM loss, E.B. had no difficulty in producing a range of self-images (e.g., I am a husband) and collections of self-defining AMs in support of these statements. E.B. produced fewer episodic memories at times of self-formation, but this did not seem to impact on the maintenance of self. The results support recent work suggesting the self may be maintained in the absence of episodic memory. The application of tasks such as that used here will further elucidate AM impairment in temporal lobe epilepsy.     

Hippocampal activation during episodic and semantic memory retrieval: comparing category production and category cued recall

Whether or not the hippocampus participates in semantic memory retrieval has been the focus of much debate in the literature. However, few neuroimaging studies have directly compared hippocampal activation during semantic and episodic retrieval tasks that are well matched in all respects other than the source of the retrieved information. In Experiment 1, we compared hippocampal fMRI activation during a classic semantic memory task, category production, and an episodic version of the same task, category cued recall. Left hippocampal activation was observed in both episodic and semantic conditions, although other regions of the brain clearly distinguished the two tasks. Interestingly, participants reported using retrieval strategies during the semantic retrieval task that relied on autobiographical and spatial information; for example, visualizing themselves in their kitchen while producing items for the category kitchen utensils. In Experiment 2, we considered whether the use of these spatial and autobiographical retrieval strategies could have accounted for the hippocampal activation observed in Experiment 1. Categories were presented that elicited one of three retrieval strategy types, autobiographical and spatial, autobiographical and nonspatial, and neither autobiographical nor spatial. Once again, similar hippocampal activation was observed for all three category types, regardless of the inclusion of spatial or autobiographical content. We conclude that the distinction between semantic and episodic memory is more complex than classic memory models suggest.     

Retrieval mechanisms for autobiographical memories: insights from the frontal variant of frontotemporal dementia

Very few studies have investigated autobiographical memory in the frontal variant of frontotemporal dementia (fv-FTD). The aim of this study was therefore to unravel the mechanisms of autobiographical memory disruption in general and in the anterograde and retrograde components of amnesia in particular, in patients suffering from fv-FTD. An autobiographical memory task assessing overall (AM) and strictly episodic memories (EM) from five lifetime periods covering the entire lifespan revealed the absence of a temporal gradient for both scores, suggesting the existence of a retrieval deficit. An analysis of the correlation between these two scores and a general cognitive assessment of executive function, working, episodic (i.e. new learning ability) and semantic memory, and behavioural changes highlighted the considerable involvement of executive function, semantic memory and, to a lesser degree, episodic memory and behavioural changes. Moreover, step-wise regression analyses performed on the EM score revealed that the executive function was a better predictor of the retrograde component than of the anterograde component, which was linked principally to new episodic learning ability. All these results confirm the impact of executive dysfunction on autobiographical deficits in fv-FTD, and suggest that the mechanisms at the root of autobiographical memory disruption may also involve difficulties in new episodic learning and semantic storage, though this may be due to the fact that we studied an advanced form of fv-FTD.     

Long-term amnesia: a review and detailed illustrative case study

Long-term amnesia is a slowly developing form of anterograde amnesia accompanied by retrograde amnesia of variable severity (Kapur, 1996; 1997) often associated with damage to the anterior temporal neocortex and epileptic seizures. The precise neural and functional deficits that underlie this condition are unknown. A patient, JL, who has this condition following a closed-head injury, is described in detail. Her injury caused bilateral anterior temporal neocortex damage that was more extensive on the left and right-sided damage to the perirhinal and orbitofrontal cortices. The hippocampus appeared to be intact bilaterally. Epilepsy developed within two years of JL's injury. Apart from her memory impairments, JL's cognitive functions, including high-level visual perception, attention, semantic memory and executive functions were well preserved. Her memory also seemed well preserved for at least 30 minutes following encoding. The one exception was the patient's relatively greater impairment at difficult visual recognition tests for which verbalization may not have been an effective strategy. This problem may have been caused by JL's right-sided perirhinal and orbitofrontal cortex damage. Her recall and recognition was clearly impaired after a three-week delay. She also showed a retrograde amnesia, which appeared to be milder than her remote post-morbid memory deficit. JL's remote memory was preserved for information first encountered in either the pre- or post-morbid period provided the information had received sufficient rehearsal over long periods of time. Her long-term amnesia may have been caused by anterior temporal neocortex damage, possibly in association with her epileptic seizures. Whether the condition is heterogeneous, involves a deficit in slow consolidation, disruption of unconsolidated memories, or blockage of maintenance or disruption of insufficiently rehearsed memories whether or not these have been slowly consolidated is discussed.     

The case of K.C.: contributions of a memory-impaired person to memory theory

K.C. has been investigated extensively over some 20 years since a motorcycle accident left him with widespread brain damage that includes large bilateral hippocampal lesions, which caused a remarkable case of memory impairment. On standard testing, K.C.'s anterograde amnesia is as severe as that of any other case reported in the literature, including H.M. However, his ability to make use of knowledge and experiences from the time before his accident shows a sharp dissociation between semantic and episodic memory. A good deal of his general knowledge of the world, including knowledge about himself, is preserved, but he is incapable of recollecting any personally experienced events. In displaying such "episodic amnesia," which encompasses an entire lifetime of personal experiences, K.C. differs from many other amnesic cases. Here, we document for the first time the full extent of K.C.'s brain damage using MRI-based quantitative measurements. We then review the many investigations with K.C. that have contributed to our understanding not only of episodic and semantic memory but also to the development of other aspects of memory theory. These include the distinction between implicit and explicit memory, the prospect of new learning in amnesia, and the fate of recent and remote memory for autobiographical and public events, people, and spatial locations.     

Functional retrograde amnesia: a multiple case study

Functional retrograde amnesia (RA) is a rare pathology and has been rarely studied in detail across different patients. We extensively examined five functional RA patients and compared their neuropsychological profile including anterograde and retrograde memory performance, executive functions, emotional processing, and formally assessed psychiatric symptoms. Across patients, neuropsychological deficits beyond RA were most consistently seen in executive functions and attention suggesting that these dysfunctions contribute to the remote memory deficit. In a majority of the patients, problems in social cognition and emotional behaviour were reflected in Theory of Mind deficits and accompanying psychiatric symptoms. Aberrances in a measure of social desirability were detected, pointing to repressive tendencies in three out of the five patients. Future studies of functional RA patients may investigate more specifically which frontal-lobe associated (dys-) functions contribute to the memory retrieval deficit. Moreover, studying more closely the interaction between social cognition, repressive personality style and memory inhibition in this disease seems worthwhile pursuing.     

Psychological therapy for psychogenic amnesia: Successful treatment in a single case study

Psychogenic amnesia is widely understood to be a memory impairment of psychological origin that occurs as a response to severe stress. However, there is a paucity of evidence regarding the effectiveness of psychological therapy approaches in the treatment of this disorder. The current article describes a single case, “Ben”, who was treated with formulation-driven psychological therapy using techniques drawn from cognitive behavioural therapy (CBT) and acceptance and commitment therapy (ACT) for psychogenic amnesia. Before treatment, Ben exhibited isolated retrograde and anterograde memory impairments. He received 12 therapy sessions that targeted experiential avoidance followed by two review sessions, six weeks and five months later. Ben's retrograde and anterograde memory impairments improved following therapy to return to within the “average” to “superior” ranges, which were maintained at follow-up. Further experimental single case study designs and larger group studies are required to advance the understanding of the effectiveness and efficacy of psychological therapy for psychogenic amnesia.     

Growing up with bilateral hippocampal atrophy: from childhood to teenage

The respective roles of the medial temporal lobe (MTL) structures in memory are controversial. Some authors put forward a modular account according to which episodic memory and recollection-based processes are crucially dependent on the hippocampal formation whereas semantic acquisition and familiarity-based processes rely on the adjacent parahippocampal gyri. Others defend a unitary view.We report the case of VJ, a boy with developmental amnesia of most likely perinatal onset diagnosed at the age of 8. Magnetic resonance imaging (MRI), including quantitative volumetric measurements of the hippocampal formation and of the entorhinal, perirhinal, and temporopolar cortices, showed severe, bilateral atrophy of the hippocampal formation, fornix and mammillary bodies; by contrast, the perirhinal cortex was within normal range and the entorhinal and temporopolar cortex remained within two standard deviations (SDs) from controls’ mean. We examined the development of his semantic knowledge from childhood to teenage as well as his recognition and cued recall memory abilities. On tasks tapping semantic memory, VJ increased his raw scores across years at the same rate as children from large standardisation samples, except for one task; he achieved average performance, consistent with his socio-educational background. He performed within normal range on 74% of recognition tests and achieved average to above average scores on 42% of them despite very severe impairment on 82% of episodic recall tasks. Both faces and landscapes-scenes gave rise to above average scores when tested with coloured stimuli. Cued recall, although impaired, was largely superior to free recall.This case supports a modular account of the MTL with episodic, but not semantic memory depending on the hippocampal formation. Furthermore, the overall pattern of findings is consistent with evidence from both brain-damaged and neuroimaging studies indicating that recollection requires intact hippocampal formation and familiarity relies, at least partly, on the adjacent temporal lobe cortex.