The relationship between semantic and episodic memory: evidence from a case of severe anterograde amnesia.

It is increasingly being recognized that new declarative, consciously accessible information can be learned in anterograde amnesia, but it is not clear whether this learning is supported by episodic or semantic memory. We report a case of a 55-year-old man who experienced severe amnesia after limited damage to the medial temporal lobe following neurosurgical complications. His general cognitive performance and knowledge of new French words and public events that occurred before and after the onset of amnesia were assessed. Performance remained satisfactory on post-morbid vocabulary and public events, with a drop in performance observed for very recent public events only, while knowledge of very recent vocabulary was comparable to that of the control subjects. The implications of these findings for our understanding of the underlying learning mechanisms are discussed. This is the first report of acquisition of consciously accessible postmorbid knowledge of public events in a patient with severe amnesia.

An association of cognitive impairment with diabetes and retinopathy in end stage renal disease patients under peritoneal dialysis.

BACKGROUND: Diabetes and retinopathy have been considered as risk factors of cognitive impairment (CI) in previous studies. We investigated both of these two factors and their relationship with global and specific cognitive functions in end stage renal disease patients under peritoneal dialysis (PD). METHODS: In this multicenter cross-sectional study, 424 clinically stable patients were enrolled from 5 PD units, who performed PD for at least three months and completed fundoscopy examination if they had diabetes. Global cognitive function was measured using the Modified Mini-Mental State Examination (3MS), Trail-Making Test forms A and B for executive function, and subtests of the Battery for the Assessment of Neuropsychological Status for immediate and delayed memory, visuospatial skills, and language ability. RESULTS: PD Patients with DM and Retinopathy had significantly higher prevalence of CI, executive dysfunction, impaired immediate memory and visuospatial skill, compared with patients in non-DM group. By multivariate logistic regression analyses, DM and retinopathy rather than DM only were significantly associated with increased risk for CI, executive dysfunction, impaired immediate memory and visuospatial skill, odds ratios(ORs) and 95% confidence intervals were 2.09[1.11,3.92], 2.89[1.55,5.37], 2.16 [1.15,4.06] and 2.37[1.32,4.22], respectively (all P < 0.05). CONCLUSIONS: Diabetic PD patients with retinopathy were at two times risk for overall cognitive impairment, executive dysfunction, impaired immediate memory and visuospatial skill as compared to non-diabetic PD patients.

The circuitry of abulia: insights from functional connectivity MRI.

BACKGROUND: Functional imaging and lesion studies have associated willed behavior with the anterior cingulate cortex (ACC). Abulia is a syndrome characterized by apathy and deficiency of motivated behavior. Abulia is most frequently associated with ACC damage, but also occurs following damage to subcortical nuclei (striatum, globus pallidus, thalamic nuclei). We present resting state functional connectivity MRI (fcMRI) data from an individual who suffered a stroke leading to abulia. We hypothesized that, although structural imaging revealed no damage to the patient's ACC, fcMRI would uncover aberrant function in this region and in the relevant cortical networks. METHODS: Resting state correlations in the patient's gray matter were compared to those of age-matched controls. Using a novel method to identify abnormal patterns of functional connectivity in single subjects, we identified areas and networks with aberrant connectivity. RESULTS: Networks associated with memory (default mode network) and executive function (cingulo-opercular network) were abnormal. The patient's anterior cingulate was among the areas showing aberrant functional connectivity. In a rescan 3 years later, deficits remained stable and fcMRI findings were replicated. CONCLUSIONS: These findings suggest that the aberrant functional connectivity mapping approach described may be useful for linking stroke symptoms to disrupted network connectivity.

Persistent anterograde amnesia following limbic encephalitis associated with antibodies to the voltage-gated potassium channel complex.

OBJECTIVE: Limbic encephalitis (LE) associated with antibodies to the voltage-gated potassium channel complex (VGKC) is a potentially reversible cause of cognitive impairment. Despite the prominence of cognitive dysfunction in this syndrome, little is known about patients' neuropsychological profile at presentation or their long-term cognitive outcome. METHODS: We used a comprehensive neuropsychological test battery to evaluate cognitive function longitudinally in 19 patients with VGKC-LE. RESULTS: Before immunotherapy, the group had significant impairment of memory, processing speed and executive function, whereas language and perceptual organisation were intact. At follow-up, cognitive impairment was restricted to the memory domain, with processing speed and executive function having returned to the normal range. Residual memory function was predicted by the antibody titre at presentation. CONCLUSIONS: The results show that, despite broad cognitive dysfunction in the acute phase, patients with VGKC-LE often make a substantial recovery with immunotherapy but may be left with permanent anterograde amnesia.

Prevalence and clinical characteristics of unremembered nocturnal eating in diabetic subjects: Kurume sleep trouble in obesity and metabolic disorders (KUSTOMED) study.

Nighttime food intake is associated with weight gain and higher HbA1c levels. We experienced night eaters who have no memory of their nocturnal eating in the morning. In this study, the curious night eating behavior was designated as "unremembered nocturnal eating syndrome (UNES)". We screened 1,169 patients with diabetes for sleep quality and abnormal eating behavior at night using the Pittsburgh Sleep Quality Index questionnaire with an additional question regarding UNES. When abnormal nocturnal eating behavior was noted, detailed clinical information was extracted from interviews with the patients. We identified 9 patients who experienced UNES. They had a higher BMI compared with subjects who reported no such episodes. Among them, 6 patients who consumed food at night without memory 2-5 times per month or more had significantly higher HbA1c levels. Continuous glucose monitoring in a patient with type 1 diabetes revealed an abrupt elevation of glucose levels from midnight when some foods were consumed. Eight of the 9 patients were taking benzodiazepine and/or non-benzodiazepine hypnotic agents when they experienced the episodes. The prevalence of UNES was 0.8% in all subjects and 4% in those taking hypnotic drugs. The ratio of hypnotic drug use in subjects with UNES was significantly higher than for individuals without UNES (89% vs. 17%, p<0.0001). Although UNES seems to be etiologically heterogeneous, hypnotics-induced parasomnia and/or anterograde amnesia may be associated with the behavior. UNES is not rare in diabetic patients on hypnotic medicine and may be a hidden cause of unexpected morning hyperglycemia.

Compensating for anterograde amnesia: a new training method that capitalizes on emerging smartphone technologies.

Following a neuropathological event, individuals left with moderate-to-severe memory impairment are unable to reliably form new memories. The most common challenges involve the capacity to perform a task in the future and to consciously recall a recent event. Disruption of these memory processes leaves the individual trapped in the present, unable to stay on track, and alienated from ongoing events. Memory research has demonstrated that implicit memory is often preserved despite severe explicit memory impairment and that preserved memory systems can provide avenues for acquiring new skills and knowledge. A within-subject single-case A1-B1-A2-B2 experimental design was used to introduce an established theory-driven training program of technology use for individuals with moderate-to-severe memory impairment. We describe its application to enabling RR, an individual with memory impairment postcolloid cyst removal, to independently support her memory using a commercial smartphone. RR showed successful outcome on both objective and qualitative measures of memory functioning. Moreover, she demonstrated consistent and creative generalization of acquired smartphone skills across a broad range of real-life memory-demanding circumstances. Our findings suggest that individuals with moderate-to-severe memory impairment are able to capitalize on emerging commercial technology to support their memory.

Successful life outcome and management of real-world memory demands despite profound anterograde amnesia.

We describe the case of Angie, a 50-year-old woman with profound amnesia (General Memory Index = 49, Full Scale IQ = 126) following a closed head injury in 1985. This case is unique in comparison to other cases reported in the literature in that, despite the severity of her amnesia, she has developed remarkable real-world life abilities, shows impressive self-awareness and insight into the impairment and sparing of various functional memory abilities, and exhibits ongoing maturation of her identity and sense of self following amnesia. The case provides insights into the interaction of different memory and cognitive systems in handling real-world memory demands and has implications for rehabilitation and for successful life outcome after amnesia.

Widespread cognitive impairment in psychogenic anterograde amnesia.

A 34-year-old man without a past history of any psychiatric or neurological disorder developed severe anterograde amnesia following a psychological trauma. Initial assessment of neuropsychological functions 3 months after the psychological trauma indicated severe memory deficits for acquiring new information in both verbal and visual modalities with widespread cognitive deficits in attention, executive functions, and intellectual ability. Importantly, working and remote memory were intact. The case illustrates that psychogenic anterograde amnesia might be associated with a wider range of cognitive deficits. Possible neurobiological explanations are discussed to explain large cognitive impairments associated with anterograde psychogenic amnesia.

Recovery of long-term anterograde amnesia, but not retrograde amnesia, after initiation of an anti-epileptic drug in a case of transient epileptic amnesia.

Transient epileptic amnesia (TEA) is characterised by recurrent brief episodes of amnesia and atypical amnesic symptoms, known as long-term anterograde amnesia and dense retrograde amnesia. It has been proposed that an antiepileptic drug (AED) can prevent not only epileptiform activity, but also accelerated forgetting. However, there have been no reports regarding the effects of such drugs on retrograde amnesia. We found that an AED prevented accelerated forgetting, but not dense retrograde amnesia, suggesting that accelerated forgetting in TEA was treatable, but retrograde amnesia was an irreversible process.

Just lying there, remembering: improving recall of prose in amnesic patients with mild cognitive impairment by minimising interference.

The hallmark of amnesia is poor explicit long-term memory along with normal short-term memory. It is often stated that information encountered by amnesic patients is forgotten within 1 minute of presentation. However, previous work has not distinguished between forgetting as a function of time versus the interfering material occupying that time. We show that there is a marked benefit of reduced interference in amnesic patients with mild cognitive impairment (MCI), a condition that is characterised by anterograde amnesia in the absence of other neuropsychological deficits and carries an increased risk for Alzheimer's disease. The result suggests that long-term memory is encoded in these patients to a greater extent than had been realised but that their memory is highly vulnerable to interference.

Loss of spatial learning in a patient with topographical disorientation in new environments.

The case is described of a patient who, following cerebral hypoxia, developed severe difficulty in orienting himself in new environments in the context of a mild global amnesic syndrome. Some episodes he related suggested that his main difficulty was remembering the spatial/directional value of landmarks he recognised. A neuroradiological examination documented severe bilateral atrophy of the hippocampi associated with atrophic changes in the cerebral hemispheres, most marked in the dorsal regions. Neuropsychological and experimental evaluation showed a severe deficit of spatial learning with substantially preserved ability to learn verbal and visual-object information. He was also virtually unable to learn a route in a maze task based exclusively on spatial data, but the availability of visual cues substantially improved his learning. Finally, he performed within normal limits on various tests investigating knowledge acquired premorbidly regarding famous buildings, routes in the town he had been living in since childhood, and geography. Topographical disorientation may be subtended by a specific difficulty in storing the spatial/directional value of visual landmarks in novel environments. The hippocampus appears to be involved in the acquisition of new topographical spatial knowledge.

Severe anterograde amnesia with extensive hippocampal degeneration in a case of rapidly progressive frontotemporal dementia.

Frontotemporal dementia (FTD) is usually characterized as a spectrum of relatively slowly progressive disorders with largely focal frontal or temporal presentations. The development of clinical and research criteria for discriminating FTD from Alzheimer's disease has relied, in part, on the relative preservation of episodic memory in FTD. We present a patient with FTD who, in addition to the more typical behavioural and language deficits, had a profound anterograde amnesia at the time of diagnosis. Neuroimaging confirmed atrophy of frontal and temporal lobes bilaterally, most marked in the anterior left temporal region. At post-mortem, non-Alzheimer pathology resulting in devastating cell loss was revealed in the hippocampi, as well as in the frontal and temporal cortex, thus providing neuroanatomical corroboration of the episodic memory deficit. Progression of the disease was extraordinarily rapid, with just 2 years between reported onset and time of death. This case demonstrates that the pattern of FTD may include severe anterograde amnesia as a prominent and early consequence of the disease.