Topographical Disorientation Research Articles

Behavioural and cognitive mechanisms of Developmental Topographical Disorientation

Individuals affected by Developmental Topographical Disorientation (DTD) get lost on a daily basis, even in the most familiar of surroundings such as their neighbourhood, the building where they have worked for many years, and, in extreme cases, even in their own homes. Individuals with DTD report a lifelong selective inability to orient despite otherwise well-preserved general cognitive functions, and the absence of any acquired brain injury or neurological condition, with general intelligence reported to be within the normal range. To date, the mechanisms underlying such a selective developmental condition remain unknown. Here, we report the findings of a 10-year-long study investigating the behavioural and cognitive mechanisms of DTD in a large sample of 1211 cases. We describe the demographics, heritability pattern, self-reported and objective spatial abilities, and some personality traits of individuals with DTD as compared to a sample of 1624 healthy controls; importantly, we test the specific hypothesis that the presence of DTD is significantly related to the inability of the individuals to form a mental representation of the spatial surroundings (i.e., a cognitive map). We found that individuals with DTD reported relatively greater levels of neuroticism and negative affect, and rated themselves more poorly on self-report measures of memory and imagery skills related to objects, faces, and places. While performing interactive tasks, as a group, the individuals with DTD performed slightly worse on a scene-based perspective-taking task, and, notably struggled to solve tasks that demand the generation and use of a cognitive map. These novel findings help define the phenotype of DTD, and lay the foundation for future studies of the neurological and genetic mechanisms of this lifelong condition.

‘No, I don't know where that is. All I know is my room.’: Topographical disorientation and user‐centred requirements for in‐hospital navigation aids of people with dementia and their caregivers

AbstractBackgroundHospitals are complex buildings in which the risk of disorientation is high with negative consequences on financial and staff resources due to missed appointments and increased need of support. Existing indoor navigation aids, such as accompanying services, spatial design or technical assistance, often miss the intended users’ needs. Based on the value sensitive design approach, this study asked people with dementia (PwD) and their caregivers about the requirements in‐hospital navigation aids should fulfil for them to be considered useful.MethodWe conducted semi‐structured interviews with two stakeholder groups of stationary dementia care: PwD (n = 10, agemean = 83,9 years) and informal caregivers (n = 5, agemean = 73,8 years). In the interviews, we asked for previous experiences including barriers for the use of existing navigation aids. We analysed underlying values and requirements for improved technologies. The interviews were evaluated using qualitative content analysis according to Mayring.ResultAccompanying services by hospital staff or relatives were considered by respondents to be the most helpful support for in‐hospital orientation. Barriers for the use of existing in‐hospital navigation aids are their low visibility and lacking need of usage due to restricted mobility of in‐patients with dementia. Interestingly, some PwD are willing to use technical assistance for navigation support although their digital competencies are considered to be very low by caregivers. For both stakeholder groups, trust is an important value in technical devices for navigation. PwD highlighted independence as further important value while caregivers prioritized safety. Both respondents groups wanted a device that could be worn on the body and that gave simple instructions for finding rooms via visual or acoustic signals.ConclusionPwD and caregivers can specify needs for in‐hospital navigation aids and their requirements differ only slightly due to varying values. User‐centred design potentially helps to improve emerging technologies for patient‐centred stationary care.

Egocentric and Allocentric Spatial Cognition in Amnestic Mild Cognitive Impairment and Early Alzheimer’s Disease

Background: Topographical disorientation is one of the early symptoms of Alzheimer’s disease (AD). The nature of this symptom, however, remains unclear. Objective: The aim of the study was to investigate egocentric and allocentric spatial cognition in patients with amnestic mild cognitive impairment (aMCI) and early AD. Participants and Methods: The participants consisted of normal healthy volunteers (n = 23), patients with aMCI (n = 26), and patients with early AD (n = 22). We administered the card placing test (CPT), in which a subject was required to recreate an array of 3 cards, each of which was randomly placed on 8 grids around the individual, before (part A) and after (part B) the individual’s rotation. With this design, the CPT can reveal an individual’s ability to represent spatial information either egocentrically (CPT-A) or allocentrically (CPT-B). A qualitative analysis of errors in performing the CPT was also conducted. Results: Compared with the controls, the aMCI patients showed significantly poorer CPT-B performance, while there was no significant difference in CPT-A performance between these 2 groups. In contrast, the AD patients demonstrated significantly poorer performance on both the CPT-A and CPT-B than the controls and aMCI patients. There was no significant difference in the profile of errors on the CPT-B between the controls and aMCI patients, whereas there was a notable difference in those on the CPT-A between the controls and AD patients and the aMCI and AD patients. Conclusion: Allocentric spatial cognition is selectively impaired in aMCI patients, while an egocentric spatial cognition is additionally impaired in AD patients.

Selective defects of face familiarity associated to a left temporo-occipital lesion

Acquired prosopagnosia is usually a consequence of bilateral or right hemisphere lesions and is often associated with topographical disorientation and dyschromatopsia. Left temporo-occipital lesions sometimes result in a face recognition disorder but in a context of visual object agnosia with spared familiarity feelings for faces, usually in left-handers. We describe a patient with a left temporo-occipital hemorrhagic lesion unexpectedly resulting in a deficit of face familiarity, which could represent a mild form of associative prosopagnosia. Our patient failed to feel familiarity feelings even with very well-known famous faces but had neither visual object agnosia nor defects with semantics or naming of celebrities. This was confirmed even when the patient was re-tested a year later. We speculate that a graded lateralization of face processing could be at the basis of occasional cases of prosopagnosia.

The differential effect of normal and pathological aging on egocentric and allocentric spatial memory in navigational and reaching space.

Topographical disorientation (TD) refers to a particular condition which determines the loss of spatial orientation, both in new and familiar environments. TD and spatial memory impairments occur relatively early as effect of cognitive decline in aging, even in prodromal stages of dementia, namely mild cognitive impairment (MCI). (a) To show that components linked to the recall of familiar spatial knowledge are relatively spared with respect to the learning of unfamiliar ones in normal aging, while they are not in MCI, and (b) to investigate gender differences for their impact on egocentric and allocentric frames of reference. Forty young participants (YC), 40 healthy elderly participants (HE), 40 elderly participants with subjective memory complaints (SMC), and 40 elderly with probable MCI were administeredwith egocentric and allocentric familiar tasks, based on the map of their hometown, and with egocentric and allocentric unfamiliar tasks, based on new material to be learned. A series of general linear models were used to analyze data. No group differences were found on egocentric task based on familiar information. MCI performed worse than the other groups on allocentric tasks based on familiar information (YC = HE = SMC > MCI). Significant differences emerged between groups on egocentric and allocentric tasks based on unfamiliar spatial information (YC > HE = SMC > MCI). A gender difference was found, favoring men on allocentric unfamiliar task. Familiarity of spatial memory traces can represent a protective factor for retrospective components of TD in normal aging. Conversely, using newly learned information for assessment may lead to overestimating TD severity.

A Novel Training Program to Improve Human Spatial Orientation: Preliminary Findings.

The ability to form a mental representation of the surroundings is a critical skill for spatial navigation and orientation in humans. Such a mental representation is known as a “cognitive map” and is formed as individuals familiarize themselves with the surrounding, providing detailed information about salient environmental landmarks and their spatial relationships. Despite evidence of the malleability and potential for training spatial orientation skills in humans, it remains unknown if the specific ability to form cognitive maps can be improved by an appositely developed training program. Here, we present a newly developed computerized 12-days training program in a virtual environment designed specifically to stimulate the acquisition of this important skill. We asked 15 healthy volunteers to complete the training program and perform a comprehensive spatial behavioral assessment before and after the training. We asked participants to become familiar with the environment by navigating a small area before slowly building them up to navigate within the larger and more complex environment; we asked them to travel back and forth between environmental landmarks until they had built an understanding of where those landmarks resided with respect to one another. This process repeated until participants had visited every landmark in the virtual town and had learned where each landmark resided with respect to the others. The results of this study confirmed the feasibility of the training program and suggested an improvement in the ability of participants to form mental representations of the spatial surrounding. This study provides preliminary findings on the feasibility of a 12-days program in training spatial orientation skills. We discuss the utility and potential impact of this training program in the lives of the many individuals affected by topographical disorientation as a result of an acquired or developmental condition.

Topographical Disorientation: Clinical and Theoretical Significance of Long-Lasting Improvements Following Imagery-Based Training.

Neuropsychological studies on acquired topographical disorientation have provided useful insights into the contribution of different brain regions to human navigation. However, little is known about the possibility to restore navigational skills after brain damage. Here we describe the case of No Longer Lost (NLL), a 49-year-old man who complained of severe topographical disorientation following traumatic brain injury. Extensive neuropsychological evaluation at baseline revealed selective episodic memory deficits and topographical disorientation. NLL underwent 8-week imagery-based treatment (IBT) inspired by current cognitive models of human spatial navigation. After IBT, NLL improved topographical skills and episodic memory. From a clinical point of view, the present study describes a model-based intervention for topographical disorientation. From a theoretical point of view, it provides new insights into the cognitive models of human spatial navigation and straightforward evidence about common phylogenetic roots of brain mechanisms devoted to spatial navigation and memory.

Topographic Disorientation in Patients with Brain Damage

This article describes the symptoms, pathogenesis, and variants of topographic disorientation. Methods for the diagnosis and rehabilitation of patients are presented. Studies have led to the conclusion that identification of the variant of topographic disorientation and determination of the strategy for compensating for this disorder have great importance for completing the neurorehabilitation of patients with stroke and other focal brain damage.

Dementia trajectory for patients with logopenic variant primary progressive aphasia.

The timing of progression of logopenic variant primary progressive aphasia (lvPPA) to severe dementia has not been elucidated. To address this shortcoming, 10 patients with lvPPA were continuously followed. Patients were assessed with the annual rate of change in the Clinical Dementia Rating (CDR) sum of boxes and period from lvPPA onset to the onset of benchmark signs, including mild, moderate, or severe dementia, episodic memory deficits, topographical disorientation, difficulties with using controls for electronic appliances, and conceptual apraxia. When severe dementia was evident, we also investigated the incidence of severe cognitive and behavioral signs such as neologistic jargon, difficulties in recognizing family members, pica, and mirror sign. The mean time for patients to reach a particular CDR was as follows: CDR of 1, 4.1 ± 1.3years post-onset; CDR 2, 5.7 ± 1.6years; CDR 3, 7.3 ± 1.6years. The annual rate of change in the CDR sum of boxes was 3.4 ± 1.1, corresponding to 1.7years for the CDR to increase by 1.0. Difficulties with using electronic controls began at 3.3 ± 1.6years, episodic memory deficits at 4.0 ± 2.0years, topographical disorientation at 5.2 ± 2.1years, and conceptual apraxia at 5.5 ± 2.1years. For patients who progressed to severe dementia, six could not recognize family members, five exhibited pica, three experienced mirror sign, and one developed neologistic jargon. Our results suggest that patients with lvPPA progress rapidly to dementia and develop conceptual apraxia, episodic memory deficits, visuospatial deficits, and semantic memory deficits.

Does spatial cognitive style affect how navigational strategy is planned?

People orient themselves in the environment using three different, hierarchically organized, spatial cognitive styles: landmark, route, and survey. Landmark style is based on a representation encompassing only visual information (terrain features); route style is based on a representation that connects landmarks and routes using an egocentric (body-centred) frame of reference; survey style is based on a global map-like representation that mainly involves an allocentric (world-centred) frame of reference. This study was aimed at investigating whether individual spatial cognitive style affected the way to plan a path when searching for a lost object. Participants with landmark, route, and survey style were assessed with an ecological navigational planning task (the Key Search Task), which required planning a strategy to search for the lost key in a hypothetical wide squared field. Results showed that spatial cognitive styles were associated to different navigational planning strategies, although the time to complete the Key Search Task was comparable across the styles. As revealed by the Key Search Task score, survey style individuals were the best navigational planners, route style individuals were less efficient and landmark style individuals were the least efficient. These results suggest that spatial cognitive style has effects on navigational planning. Implications for clinical settings, such as for developmental topographical disorientation, are discussed.

Pure topographical disorientation in novel environments without anterograde amnesia: a case study

ABSTRACTTopographical disorientation (TD) in novel environments is considered to be a part of anterograde amnesia. A 56-year-old woman presented with pure TD only in novel environments following limbic encephalitis. She could not remember directions inside the hospital on weekly outpatient visits; however, her verbal and visual anterograde memories were normal. In the test of learning photographs of scenes, faces, and objects, only her scores for landscapes were worse than those in healthy controls. These findings suggested that her TD specific to landscapes and directions in novel environments was caused by category-specific memory impairment related to bilateral hippocampal and parahippocampal dysfunction.

Remote spatial and autobiographical memory in cases of episodic amnesia and topographical disorientation

A number of theories have postulated that there is a strong relationship between episodic memory and spatial processes mediated by the hippocampus. Evidence for episodic amnesia following damage to the medial temporal lobes is extensive, but less is known about the types of spatial memory affected by damage to these regions. In this study, we compared episodic memory with detailed scene memory, landmark recognition and schematic (map-based) spatial memory in a group of individuals with amnesia related to damage to the medial temporal lobes (MTL) including the hippocampus. We compared their performance to matched controls, and to an individual with topographical disorientation, a selective spatial memory deficit relating to more posterior temporal and occipital lobe damage. For individuals with MTL lesions, impairments to scene memory were comparable to those in episodic memory. Landmark recognition was impaired only for less familiar landmarks, and schematic spatial memory was not impaired compared to controls. Despite the absence of hippocampal damage, the individual with topographical disorientation, like the MTL amnesic patients, demonstrated impairments to scene memory and recognition of less familiar landmarks, and intact schematic spatial memory, but with less severe episodic memory loss. These results highlight the similarities between detailed scene memory and episodic memory, including their reliance on the medial temporal lobe, and suggest that more schematic forms of spatial memory may be unaffected by medial temporal damage. In addition, the results suggest that damage to more posterior temporal or occipital regions that leads to spatial memory deficits may entail some impairment to episodic memory even if the hippocampus is spared.

Cingulate infarction: A neuropsychological and neuroimaging study

Background and purposeIschemic lesions rarely affect the cingulate cortex (CC) in isolation, restricting human lesion/behavioural change correlational analysis. The aim of this study was to determine clinical, neuropsychological and neuroimaging features of isolated cingulate infarcts. MethodsWe studied, 3800 patients with first-ever ischemic stroke included in our Stroke Registry between 2012 and 2018. Among them we studied 7 patients with an acute isolated cingulate infarct confirmed by MRI. ResultsAmong all patients, 7 patients (0.01%) showed ischemic lesions in the territory of cingulate cortex territory, allowing us to delineate 2 substantial distributions; (1) Anterior cingulate cortex (ACC) infarction (4 patients [57%]) was presented low vigilance level with apathy, mutism, deficits in executive function, attention, and disturbances of working, episodic and verbal memory; (2) Posterior cingulate cortex (PCC) infarction (3 patients [43%]) developed topographic disorientation, visual memory deficit and affective-emotional behavioural changes. ConclusionsAccording rarely seen CC infarction events, we suggest that anterior and posterior CC are functionally separated and differences in clinical presentation are explained by considering; ACC plays a role in executive functions, episodic and working memory, set maintenance, and PCC is focused on spatial and verbal attention, and memory system. We considered that different patterns of cingulate infarcts are the result of variation in cingulate arterial supply or suggest a source of embolism.

Is Losing One's Way a Sign of Cognitive Decay? Topographical Memory Deficit as an Early Marker of Pathological Aging.

Spatial navigation tasks reveal small differences between normal and pathological aging and may thus disclose potential neuropsychological predictors of neurodegenerative diseases. The aim of our study was to investigate which navigational skills are compromised in the early phase of pathological aging as well as the extent to which they are compromised. We performed an extensive neuropsychological evaluation based on working memory and learning tasks (i.e., Corsi Block-Tapping Test and Walking Corsi Test) involving both reaching and navigational vista spaces. We also assessed spatial navigation skills in the real world by asking participants to perform route-learning and landmark-recognition tasks. We conducted a cross-sectional study on nineteen patients with a diagnosis of mild cognitive impairment (MCI) who displayed either an isolated memory deficit (single-domain amnestic MCI, MCIsd; N = 3) or a memory deficit associated with deficits in other cognitive functions (multi-domain MCI, MCImd; N = 16) as well as on nineteen healthy control participants. The groups' performances were compared by means of mixed factorial ANOVA and two-sample t-tests. We found that patients with MCI performed worse than controls, especially when they were required to learn spatial positions within the navigational vista space. Route-learning within the real environment was also impaired whereas landmark-recognition was spared. The same pattern of results emerged in the MCImd subgroup. Moreover, single case analyses on MCIsd patients revealed a dissociation between learning of spatial positions within navigational vista space and within reaching space. These results suggest that topographical learning is compromised in the early phase of MCIsd and MCImd and that spatial navigation tasks may be used to better characterize topographical disorientation in MCI patients as well as for the early diagnosis of pathological aging.

Memory for familiar locations: The impact of age, education and cognitive efficiency on two neuropsychological allocentric tasks.

This research aims to reconsider and support the use of spatial tasks based on familiar geographical information in the neuropsychological assessment of topographical (dis)orientation. Performance on two spatial tasks based on familiar information -l andmark positioning on a map and map of Italy - were compared in two studies assessing allocentric orientation among young and healthy elderly with different levels of education (Study 1) and elderly with and without probable cognitive impairment (Study 2). Results from Study 1 showed that the map of Italy task was affected by education, while the landmark positioning on a map was not. Results of Study 2 showed that both tasks were sensitive to different levels of cognitive functioning in a sample of community-dwelling seniors. Overall, spatial tasks based on mental representation of the hometown environment may be an important supplement in the assessment of allocentric topographical disorientation, discriminating typical from atypical aging.

Perspective taking to assess topographical disorientation: Group study and preliminary normative data

Difficulty in navigating in a well-known environment (i.e., topographical disorientation) is an incapacitating syndrome. Despite the relatively high frequency after a right cerebral lesion, there is currently a dearth of neuropsychological tests to assess it. We propose a completely new test, the third in a battery of three, which assesses perspective taking, a crucial process in spatial orientation. In this test, inspired from Piaget and Inhelder Three Mountains, the subject must imagine a change in perspective in two conditions: after a rotational or a linear displacement. This task was proposed to 63 patients with a right cerebral lesion and a control group (N = 112). Results show that a right cerebral lesion impairs perspective taking, more specifically when rotation is involved. In addition, the pattern of responses in patients indicates that they have a general deficit in the ability of decentering. Furthermore, we established preliminary normative data taking into account the effect of age and education (there was no effect of gender) on performance. These data indicate that this new test is a good tool in clinical practice for assessing spatial orientation and determining more precisely the symptoms underlying topographical disorientation.

Pack in or go on: Topographic disorientation induced by bupropion sustained release tablet

The mechanism of effect of bupropion is unknown. The authors report a case of bupropion-associated topographic disorientation during bupropion treatment for smoking cessation. A 44-year-old female patient with a history not knowing where she is, the inability to recognize the surrounding buildings was admitted to neurology department. During her admission, her symptoms disappeared and she did not continue her medication. There was no abnormality in the investigation. It was concluded that the onset of symptoms was dose dependent. Bupropion may cause unusual neuropsychiatric behaviors. This case suggests that topographic disorientation may be the primary symptom of bupropion toxicity.

Atypical non-progressive semantic impairment following allogeneic bone marrow transplantation in a patient with Waldenström’s macroglobulinemia: A case report

Objective: A case report of a 74-year-old male presenting with an atypical multimodal semantic impairment. The patient was diagnosed with Waldenström macroglobulinemia (WM) for which he received allogeneic bone marrow transplantation (BMT) due to disease progression. Following BMT, he developed a sudden onset of semantic difficulties that have remained unchanged for eight years. No other cognitive functions have been affected and his activities of daily living remain fully preserved.Method: The patient was assessed at our neuropsychology unit with six neuropsychological evaluations over an 8-years follow-up period following BMT. Additional semantic tests were administered during the last three evaluations. Four MRI scans (at age 62, 66, 69 and 74) and 18F-FDG PET (at age 74) were obtained.Results: The patient presents a multimodal semantic impairment, including naming impairment, visual agnosia, prosopoanomia, associative prosopagnosia, topographical disorientation and impaired retrograde memory for public events. MRI scans and 18F-FDG PET revealed bilateral symmetrical atrophy (temporal > frontal) and inferior bilateral temporal lobe hypometabolism, respectively. Neuroradiological examination was unremarkable prior to BMT.Conclusion: Clinical diagnosis remains a challenge given the focal and stable nature of his deficits. We hypothesize that the BMT procedure might have resulted in the temporal lobe damage and subsequent semantic impairment. We recommend obtaining a thorough neuropsychological evaluation of patients who receive allogenic BMT, both prior to and following transplant.

Posterior Cortical Atrophy: A longitudinal neurocognitive case study.

Posterior Cortical Atrophy (PCA) is a neurodegenerative disease that first develops in the occipital-parietal-temporal cortex. While its major pathological cause is the same as that of Alzheimer's disease (e.g. Tang-Wai & colleagues), the development of the cognitive changes over its course is quite different. Here we present a 6-year longitudinal account of the neurocognitive profile of a highly intelligent man (BG), starting from the year of his diagnosis to that of his death. Despite his normal visual acuity and visual fields, BG initially reported reading difficulties. Early in his disease, he had deficits in visual attention, processing speed and recognition. His recognition difficulties first presented as alexia without agraphia, followed by topographical disorientation, prosopagnosia, simultagnosia, and object agnosia. Next, his visuo-constructional, arithmetic skills, and visual memory gradually declined. Relative to his severe visuo-perceptual and recognition difficulties, his abilities to reach for objects remained functional. Serial MRI brains demonstrated progressive left-more-than-right occipital, temporal and parietal atrophy with relative sparing of the hippocampi. Four years following diagnosis, BG could no longer perform any visual tasks and started to develop significant expressive language difficulties. Over the full course of the disease, his motor abilities, verbal recognition and verbal logical memory remained functional, and his practical reasoning normal. BG's case illustrates that the neurodegenerative process involve in PCA can first affect the visuo-cognitive functions typical of the ventral system, extend to all functions dependent on the posterior areas, and eventually to language areas, all while sparing areas responsible for practical and logical verbal reasoning. Meeting abstract presented at VSS 2018

Topographical disorientation in aging. Familiarity with the environment does matter.

Topographical disorientation (TD) refers to navigational impairment as an effect of aging or brain damage. Decreases in navigational performance with aging are more due to deficits in the ability to mentally represent space in an object-centered (allocentric) than in a self-centered (egocentric) format. Familiarity/remoteness of spatial memory traces can represent a protective factor for TD in aging. Conversely, using newly learned information for assessment may lead to overestimating TD severity as it combines two contributing factors: heading (allocentric) disorientation and anterograde agnosia. A supplementary evaluation of TD with aging according to ecological spatial tasks is recommended. The core tasks should focus on landmark positioning, both on a blind map (allocentric) and along a route (egocentric) of the hometown so as to disentangle spatial memory for familiar/remote information from decline due to recent encoding of information.