Dissociative Amnesia: Remembrances Under Cover.

The existence or questionability of "repressed memories" can be discussed as being a matter of definition. It seems, however, far-fetched to consider all "lost" memories as caused by encoding problems, brain damage, forgetfulness, failure to disclose events, and so on. We argue that dissociative amnesia (DA) (or "psychogenic amnesia," or "functional amnesia," or, as we favor to call it, "mnestic block syndrome") is caused by psychic alterations, but ultimately they can be traced to changes in the physiology of the brain, as we are of the opinion that all memory processes-positive or negative-alter brain functions, sometimes more permanently, sometimes transiently. We have proven this idea using functional imaging techniques, in particular fluoro-deoxy-d-glucose positron emission tomography. Having investigated dozens of patients with severe and long-lasting DA conditions, we believe it to be disrespectful to many (but not to all) of the affected patients to question their disease condition, which can be proven to be not caused by feigning, malingering, or direct brain damage.

The need for a unified framework: How Tulving's framework of memory systems, memory processes, and the SPI-model can guide and sharpen the understanding of déjà vu and involuntary autobiographical memories and add to conceptual clarity.

Barzykowski and Moulin link déjà vu and involuntary autobiographical memories to the process of retrieval. They make no reference to Tulving's SPI-model. In this, it is proposed that information is acquired serially (S), stored in parallel (P), and retrieved independently (I). This model offers an alternative, elegant, view of involuntary autobiographical memory retrieval, as well as of déjà vus.

Urbach-Wiethe disease in a young patient without apparent amygdala calcification.

Urbach-Wiethe disease is an extremely rare genetically-based syndrome which usually leads to dermatological and neurological changes. Neurologically, the amygdaloid region is primarily bilaterally affected. Therefore, several functions modulated by the amygdala are changed in patients with Urbach-Wiethe disease. As the neurological alterations evolve only gradually, it is particularly important to determine the cognitive and brain status of a juvenile. The patient described here was seen briefly at age 9 and tested neuropsychologically at age 19; furthermore, computer tomography and magnetic resonance imaging of his head was done. There were no important abnormalities in the brain, which is unusual in the light of previous data from other patients. On the cognitive level, the patient was generally within normal limits. However, he had mild problems in attention and concentration, and more prominent problems in emotional processing domain, and in personality dimensions. It is concluded that amygdala calcifications in Urbach-Wiethe disease take place progressively-possibly underpinned by genetic and gender variables; this can subsequently allow psychosocial-social factors (such a proper education and socialization) and biological factors (compensatory neuroplasticity) to retard and diminish the development of socio-emotional and cognitive deteriorations, though the outcome of questionnaires indicates that such patients may develop substantial concerns as to their future life and well-being.

Behavioral, neurological, and psychiatric frailty of autobiographical memory.

Autobiographical-episodic memory is considered to be the most complex of the five long-term memory systems. It is autonoetic, which means, self-reflective, relies on emotional colorization, and needs the features of place and time; it allows mental time traveling. Compared to the other four long-term memory systems-procedural memory, priming, perceptual, and semantic memory-it develops the latest in phylogeny and ontogeny, and is the most vulnerable of the five systems, being easily impaired by brain damage and psychiatric disorders. Furthermore, it is characterized by its fragility and proneness to distortion due to environmental influences and subsequent information. On the brain level, a distinction has to be made between memory encoding and consolidating, memory storage, and memory retrieval. For encoding, structures of the limbic system, with the hippocampus in its center, are crucial, for storage of widespread cortical networks, and for retrieval again a distributed recollection network, in which the prefrontal cortex plays a crucial role, is engaged. Brain damage and psychiatric diseases can lead to what is called "focal retrograde amnesia." In this context, the clinical picture of dissociative or functional or psychogenic amnesia is central, as it may result in autobiographical-emotional amnesia of the total past with the consequence of an impairment of the self as well. The social environment therefore can have a major impact on the brain and on autobiographical-episodic memory processing. This article is categorized under: Psychology > Memory.

Stress- and trauma-related blockade of episodic-autobiographical memory processing.

Memory disorders without a direct neural substrate still belong to the riddles in neuroscience. Although they were for a while dissociated from research and clinical arenas, risking becoming forgotten diseases, they sparked novel interests, paralleling the refinements in functional neuroimaging and neuropsychology. Although Endel Tulving has not fully embarked himself on exploring this field, he had published at least one article on functional amnesia (Schacter et al., 1982) and ignited a seminal article on amnesia with mixed etiology (Craver et al., 2014). Most importantly, the research of Endel Tulving has provided the researchers and clinicians in the field of dissociative or functional amnesia with the best framework for superiorly understanding these disorders through the lens of his evolving concept of episodic memory and five long term memory systems classification, which he developed and advanced. Herein we use the classification of long-term memory systems of Endel Tulving as well as his concepts and views on autonoetic consciousness, relationships between memory systems and relationship between episodic memory and emotion to describe six cases of dissociative amnesia that put a challenge for researchers and clinicians due to their atypicality. We then discuss their possible triggering and maintaining mechanisms, pointing to their clinical heterogeneity and multifaceted causally explanatory frameworks.

Quo vadis 'episodic memory'? - Past, present, and perspective.

The term 'episodic memory' was coined by Endel Tulving, who also created a classification in several memory systems. This classification is presented, and it is described which predecessors existed for the partition of memory into systems. The 'episodic memory system' is discussed as being in general equivalent with the 'episodic-autobiographical memory system'. It is seen as an emotionally colorized system. A special paragraph is devoted to the 'perceptual memory system', as this was not included in Tulving's previous schemes of memory systems. More recent sub-categorizations of the 'episodic memory system' are presented and a perspective on the future of the episodic memory system is developed.

Episodic memory is emotionally laden memory, requiring amygdala involvement.

The memory impairment of neurological and psychiatric patients is seen as occurring mainly in the autobiographical-episodic memory domain and this is considered to depend on limbic structures such as the amygdala or the septal nuclei. Especially the amygdala is a hub for giving an emotional flavor to personal memories. Bastin et al. fail to include the amygdala in their integrative memory model.

Is self-report sleepiness associated with cognitive performance in temporal lobe epilepsy?

OBJECTIVES: Sleepiness and cognitive impairment are common symptoms observed in patients with epilepsy. We investigate whether self-reported sleepiness is associated with cognitive performance in patients with refractory mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). Seventy-one consecutive patients with MTLE-HS were evaluated with the Stanford Sleepiness Scale (SSS) before neuropsychological evaluation. Their mean SSS scores were compared with controls. Each cognitive test was compared between patients with (SSS ≥ 3) or without sleepiness (SSS < 3). Imbalances were controlled by regression analysis. Patients reported a significantly higher degree of sleepiness than controls (p < 0.0001). After multiple linear regression analysis, only one test (RAVLT total) remained associated with self-reported sleepiness. CONCLUSION: Self-reported sleepiness was significantly higher in MTLE-HS patients than controls, but did not affect their cognitive performance. If confirmed in other populations, our results may have implications for decision making about sleepiness screening in neuropsychological settings.

Is self-report sleepiness associated with cognitive performance in temporal lobe epilepsy? / A sonolência auto-relatada está associada ao desempenho cognitivo na epilepsia do lobo temporal?

ABSTRACT Sleepiness and cognitive impairment are common symptoms observed in patients with epilepsy. We investigate whether self-reported sleepiness is associated with cognitive performance in patients with refractory mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). Seventy-one consecutive patients with MTLE-HS were evaluated with the Stanford Sleepiness Scale (SSS) before neuropsychological evaluation. Their mean SSS scores were compared with controls. Each cognitive test was compared between patients with (SSS ≥ 3) or without sleepiness (SSS < 3). Imbalances were controlled by regression analysis. Patients reported a significantly higher degree of sleepiness than controls (p < 0.0001). After multiple linear regression analysis, only one test (RAVLT total) remained associated with self-reported sleepiness. Conclusion: Self-reported sleepiness was significantly higher in MTLE-HS patients than controls, but did not affect their cognitive performance. If confirmed in other populations, our results may have implications for decision making about sleepiness screening in neuropsychological settings.

RESUMO A sonolência e o comprometimento cognitivo são queixas comuns na epilepsia. Investigamos se a sonolência relatada pelo paciente está associada ao desempenho cognitivo na epilepsia do lobo temporal mesial refratária com esclerose do hipocampo (ELTM-EH). 71 pacientes com ELTM-EH foram avaliados pela Escala de Sonolência de Stanford (ESS) antes da avaliação neuropsicológica. A média na ESS foi comparada com a de controles. Cada teste foi comparado entre os pacientes com sonolência (ESS ≥ 3) ou sem sonolência (ESS <3). Diferenças foram controladas por regressão logística múltipla. Os pacientes relataram uma sonolência maior do que os controles (p <0,0001). Após a regressão, a sonolência relatada pelos pacientes mostrou-se associada a apenas um teste (RAVLT total). Os pacientes com ELTM-EH referem mais sonolência do que os controles, mas esta não foi associada com a cognição. Se confirmado em outras populações, nossos resultados implicarão na tomada de decisão sobre o impacto da sonolência no contexto neuropsicológico.

Neural correlates of free recall of "famous events" in a "hypermnestic" individual as compared to an age- and education-matched reference group.

BACKGROUND: Memory performance of an individual (within the age range: 50-55 years old) showing superior memory abilities (protagonist PR) was compared to an age- and education-matched reference group in a historical facts ("famous events") retrieval task. RESULTS: Contrasting task versus baseline performance both PR and the reference group showed fMRI activation patterns in parietal and occipital brain regions. The reference group additionally demonstrated activation patterns in cingulate gyrus, whereas PR showed additional widespread activation patterns comprising frontal and cerebellar brain regions. The direct comparison between PR and the reference group revealed larger fMRI contrasts for PR in right frontal, superior temporal and cerebellar brain regions. CONCLUSIONS: It was concluded that PR generally recruits brain regions as normal memory performers do, but in a more elaborate way, and furthermore, that he applied a memory-strategy that potentially includes executively driven multi-modal transcoding of information and recruitment of implicit memory resources.

Psychological causes of autobiographical amnesia: A study of 28 cases.

Autobiographical amnesia is found in patients with focal or diffuse brain damage ("organic amnesia"), but also without overt brain damage (at least when measured with conventional brain imaging methods). This last condition is usually named dissociative amnesia at present, and was originally described as hysteria. Classically and traditionally, dissociative amnesia is seen as a disorder that causes retrograde amnesia in the autobiographical domain in the aftermath of incidents of major psychological stress or trauma. In the present study one of the probably largest published collections of patients (28) with psychogenically caused autobiographical amnesia, who were assessed with comprehensive neuropsychological tests, will be described and documented in order to identify variables which are central for the occurrence of dissociative amnesia. The presented cases demonstrate that autobiographical amnesia without direct brain damage can have very mixed clinical presentations, causes and consequences. The described cases of psychogenic amnesia are clustered according to a number of manifestations and features, which include a reduced effort to perform cognitively at a normal level, a forensic background, anterograde (instead of retrograde) autobiographical amnesia, the fugue condition, concurrent somatic diseases, and their appearance in childhood and youth. It is concluded that autobiographical amnesia of a psychogenic origin may occur within a variety of symptom pictures. For all patients, it probably serves a protective function by offering them a mechanism to exit a life situation which appears to them unmanageable or adverse.

Validation of diagnostic tests for depressive disorder in drug-resistant mesial temporal lobe epilepsy.

PURPOSE: This study aimed to evaluate the diagnostic accuracy of the Hamilton Rating Scale for Depression (HRSD), the Beck Depression Inventory (BDI), the Hospital Anxiety and Depression Scale (HADS), and the Hospital Anxiety and Depression Scale-Depression subscale (HADS-D) as diagnostic tests for depressive disorder in drug-resistant mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). METHODS: One hundred three patients with drug-resistant MTLE-HS were enrolled. All patients underwent a neurological examination, interictal and ictal video-electroencephalogram (V-EEG) analyses, and magnetic resonance imaging (MRI). Psychiatric interviews were based on DSM-IV-TR criteria and ILAE Commission of Psychobiology classification as a gold standard; HRSD, BDI, HADS, and HADS-D were used as psychometric diagnostic tests, and receiver operating characteristic (ROC) curves were used to determine the optimal threshold scores. RESULTS: For all the scales, the areas under the curve (AUCs) were approximately 0.8, and they were able to identify depression in this sample. A threshold of ≥9 on the HRSD and a threshold of ≥8 on the HADS-D showed a sensitivity of 70% and specificity of 80%. A threshold of ≥19 on the BDI and HADS-D total showed a sensitivity of 55% and a specificity of approximately 90%. The instruments showed a negative predictive value of approximately 87% and a positive predictive value of approximately 65% for the BDI and HADS total and approximately 60% for the HRSD and HADS-D. CONCLUSIONS: HRSD≥9 and HADS-D≥8 had the best balance between sensitivity (approximately 70%) and specificity (approximately 80%). However, with these thresholds, these diagnostic tests do not appear useful in identifying depressive disorder in this population with epilepsy, and their specificity (approximately 80%) and PPV (approximately 55%) were lower than those of the other scales. We believe that the BDI and HADS total are valid diagnostic tests for depressive disorder in patients with MTLE-HS, as both scales showed acceptable (though not high) specificity and PPV for this type of study.

The nature of the semantic/episodic memory distinction: A missing piece of the "working through" process.

The relations between the semantic and episodic-autobiographical memory systems are more complex than described in the target article. We argue that understanding the noetic/autonoetic distinction provides critical insights into the foundation of the delineation between the two memory systems. Clarity with respect to the criteria for classification of these two systems, and the evolving conceptualization of episodic memory, can further neuroscientifically informed therapeutic approaches.

Medial temporal lobe activation during autobiographical context memory retrieval of time and place and its dependency upon recency.

The contribution of the medial temporal lobe (MTL) to the retrieval of autobiographical memories is widely accepted. Results of former patient studies and functional imaging studies suggest different involvement of the MTL during the retrieval of autobiographical context information and the remoteness of these. Varying recency, the MTL contribution during chronological and locational autobiographical context information processing was investigated in this study. Thirteen males (mean = 25 years) judged the event's place or time in a two-choice recognition task. Subjects made significantly more errors on chronological judgments. Retrieval of chronological information activated the left MTL, while retrieval of locational information activated the MTL bilaterally. Retrieval of more recent than remote context information activated especially the right MTL. Our results underline different MTL contributions on the retrieval of autobiographical context information, depending on the content and on the remoteness of the event that took place.

Impairments in Episodic-Autobiographical Memory and Emotional and Social Information Processing in CADASIL during Mid-Adulthood.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) - is the most common genetic source of vascular dementia in adults, being caused by a mutation in NOTCH3 gene. Spontaneous de novo mutations may occur, but their frequency is largely unknown. Ischemic strokes and cognitive impairments are the most frequent manifestations, but seizures affect up to 10% of the patients. Herein, we describe a 47-year-old male scholar with a genetically confirmed diagnosis of CADASIL (Arg133Cys mutation in the NOTCH3 gene) and a seemingly negative family history of CADASIL illness, who was investigated with a comprehensive neuropsychological testing battery and neuroimaging methods. The patient demonstrated on one hand severe and accelerated deteriorations in multiple cognitive domains such as concentration, long-term memory (including the episodic-autobiographical memory domain), problem solving, cognitive flexibility and planning, affect recognition, discrimination and matching, and social cognition (theory of mind). Some of these impairments were even captured by abbreviated instruments for investigating suspicion of dementia. On the other hand the patient still possessed high crystallized (verbal) intelligence and a capacity to put forth a façade of well-preserved intellectual functioning. Although no definite conclusions can be drawn from a single case study, our findings point to the presence of additional cognitive changes in CADASIL in middle adulthood, in particular to impairments in the episodic-autobiographical memory domain and social information processing (e.g., social cognition). Whether these identified impairments are related to the patient's specific phenotype or to an ascertainment bias (e.g., a paucity of studies investigating these cognitive functions) requires elucidation by larger scale research.

A Bayesian latent group analysis for detecting poor effort in a sample of cognitively impaired patients.

Using a Bayesian latent group analysis in a simulation design, we recently showed a high diagnostic accuracy when assessing effort in the context of malingered memory deficits. We here further evaluate our Bayesian model in a sample of cognitively impaired patients. The main analysis showed both high sensitivity and specificity, thus corroborating a high diagnostic accuracy of the model. Additional analysis showed variations on effort estimates after changes in malingering base rates. Variations affected sensitivity, but not specificity, which is in line with typical findings in malingering research. These data suggest that Bayesian analyses may complement and improve existing effort measures.

The impact of age on prefrontal cortex integrity during spatial working memory retrieval.

Healthy aging is accompanied by a decline in spatial working memory that is related to functional cerebral changes within the spatial working memory network. In the last decade, important findings were presented concerning the location (e.g., prefrontal), kind (e.g., 'underactivation,' 'overactivation'), and meaning (e.g., functional deficits, compensation) of these changes. Less is known about how functional connections between specific brain regions are affected by age and how these changes are related to behavioral performance. To address these issues, we used functional magnetic resonance imaging to examine retrieval-related brain activation and functional connectivity in 18 younger individuals and 18 older individuals. We assessed working memory with a modified version of the Corsi Block-Tapping test, which requires the storage and reproduction of spatial target sequences. Analyses of group differences in brain activation and functional connectivity included comparisons between younger individuals, older individuals, older high-performers, and older low-performers. In addition, we conducted a functional connectivity analysis by using a seed region approach. In comparison to younger individuals, older individuals showed lower right-hemispheric dorsolateral prefrontal activation and lower functional connectivity between the right dorsolateral prefrontal cortex and the bilateral orbitofrontal cortex. Older high-performers showed higher right dorsolateral and anterior prefrontal cortex activation than older low-performers, as well as higher functional connectivity between these brain regions. The present results suggest age-related reductions of prefrontal activation during spatial working memory retrieval. Moreover, task-related functional connectivity appears to be lower in older adults. Performance accuracy in older adults is associated with right dorsolateral and anterior prefrontal cortex activation, and with the functional connection between these regions.

Deficient symbol processing in Alzheimer disease.

Symbols and signs have been suggested to improve the orientation of patients suffering from Alzheimer disease (AD). However, there are hardly any studies that confirm whether AD patients benefit from signs or symbols and which symbol characteristics might improve or impede their symbol comprehension. To address these issues, 30 AD patients and 30 matched healthy controls performed a symbol processing task (SPT) with 4 different item categories. A repeated-measures analysis of variance was run to identify impact of different item categories on performance accuracy in both the experimental groups. Moreover, SPT scores were correlated with neuropsychological test scores in a broad range of other cognitive domains. Finally, diagnostic accuracy of the SPT was calculated by a receiver-operating characteristic curve analysis. Results revealed a global symbol processing dysfunction in AD that was associated with semantic memory and executive deficits. Moreover, AD patients showed a disproportional performance decline at SPT items with visual distraction. Finally, the SPT total score showed high sensitivity and specificity in differentiating between AD patients and healthy controls. The present findings suggest that specific symbol features impede symbol processing in AD and argue for a diagnostic benefit of the SPT in neuropsychological assessment.