Topographical Disorientation Research Articles

A Neural Basis for Developmental Topographic Disorientation.

Individuals with developmental topographic disorientation (DTD) have a life-long impairment in spatial navigation in the absence of brain damage, neurological conditions, or basic perceptual or memory deficits. Although progress has been made in identifying brain regions that subserve normal navigation, the neural basis of DTD is unknown. Using functional and structural neuroimaging and detailed statistical analyses, we investigated the brain regions typically involved in navigation and scene processing in a representative DTD individual, J.N. Although scene-selective regions were identified, closer scrutiny indicated that these areas, specifically the retrosplenial cortex (RSC), were functionally disrupted in J.N. This comprehensive examination of a representative DTD individual provides insight into the neural basis of DTD and the role of the RSC in navigation-related processing.

Assessment of Topographical Disorientation: First Application of New Tests and Case Report

A right posterior cerebral lesion can lead to an inability to orient and can consequently interfere with daily-life autonomy. Despite the wide literature about navigation abilities, it is still difficult to assess topographical disorientation (TD) because of the interindividual specificity of spatial knowledge and the diversity of symptoms. We describe here a set of new tests evaluating spatial cognition in a patient with TD presenting difficulties in navigating inside the hospital and in his hometown more than 3 years after his diffuse ischemic right Sylvian stroke. These tests assess mental imagery (global and specifically spatial imagery), perspective change, and the ability to recall spatial relations in familiar environments. The evaluation revealed difficulties with all the tests (but not in global mental imagery) in this patient when compared with matched controls. Hence, these new tests seem specific and affected by a right hemispheric lesion. The observed deficits can explain, at least partially, the spatial orientation difficulties experienced by this patient in the hospital and familiar environments. In conclusion, these tests could be appropriate tools for the assessment of visuospatial and spatial processes.

Tastes Like Music: 17 Quirks of the Brain and Body by Maria Birmingham (review)

Reviewed by: Tastes Like Music: 17 Quirks of the Brain and Body by Maria Birmingham Deborah Stevenson Birmingham, Maria Tastes Like Music: 17 Quirks of the Brain and Body; illus. by Monika Melnychuk. OwlKids, 2014 40p Trade ed. ISBN 978-1-77147-010-0 $17.95 Paper ed. ISBN 978-1-77147-083-4 $11.95 R Gr. 4–8 This slender nonfiction title offers a quick and lively tour through some unusual (if not necessarily uncommon) physiological characteristics, ranging from neurological challenges such as developmental topographical disorientation (people can’t place where they are in their own neighborhoods or even houses) to sensory traits such as supertasting to growth irregularities like hyperdontia (extra teeth). Each spread offers a brief overview of a characteristic in energetic titled paragraphs, with lively sidebars providing more information and, often, a short interview with someone possessing the feature. While not all characteristics are equally compelling in their presentation, Birmingham (who interviews herself in the section on anosmia) does an unusually fine job of her overviews, making them genuinely fresh and informative while keeping them to an accessible length. This is a quick pick that balances legitimate scientific interest with believe-it-or-not appeal and an unthreatening mass-market look; readers will want to share details out loud with one another, while younger kids reading at an advanced level and reluctant readers willing to zip past the occasional technical term will both find this rewarding. Attractively edgy cartooning provides spot art in every spread, sometimes with bonus anatomical diagrams. An index is included; while there are unfortunately no notes, the acknowledgments of interviewees and consultants suggests creditable primary research. [End Page 248] Copyright © 2015 The Board of Trustees of the University of Illinois

Please keep calm: investigating hippocampal function without stress.

Please keep calm: investigating hippocampal function without stress.

Finding my own way: an fMRI single case study of a subject with developmental topographical disorientation

Developmental topographical disorientation (DTD) causes impaired spatial orientation and navigation from early childhood with no evidence of cerebral damage. Using fMRI and a landmark sequencing task, we investigated the hypothesis that Dr Wai’s abnormal cerebral activation pattern was related to his peculiar behavioral profile. Although Dr Wai was able to correctly perform landmark sequencing, he showed a lack of activity in regions activated in all control subjects and activity in areas that were not activated in any control subject. These results are discussed in light of cognitive and functional model of navigation, with relevant implications for DTD physiology.

Topographical disorientation after ischemic mini infarct in the dorsal hippocampus: whispers in silence.

Silent focal ischemic mini infarcts in the brain are thought to cause no clinically overt symptoms. Some populations of hippocampal cells are particularly sensitive to ischemic events, however, rendering hippocampal functions especially vulnerable to ischemia-induced deficits. The present study investigated whether an otherwise silent ischemic mini infarct in the hippocampus (HPC) can produce impairments in spatial performance in rats. Spatial performance was assessed in the ziggurat task (ZT) using a 10-trial spatial learning protocol for 4 days prior to undergoing hippocampal ischemic lesion or sham surgery. Hippocampal silent ischemia was induced by infusion of endothelin-1 (ET-1), a potent vasoconstrictor, into either the dorsal or the ventral hippocampus (dHPC and vHPC). When tested postoperatively in the ZT using a standard testing protocol for 8 days, rats with hippocampal lesions exhibited no spatial deficit. Although spatial learning and memory in the ZT were not affected by the ET-1-induced silent ischemia, rats with dHPC stroke showed more returns when navigating the ZT as opposed to the vHPC rats. Comparison of region-specific HPC lesions in the present study indicated that dorsal hippocampal function is critically required for topographic orientation in a complex environment. Topographic disorientation as reflected by enhanced return behaviors may represent one of the earliest predictors of cognitive decline after silent ischemic insult that may be potentially traced with sensitive clinical examination in humans.

Developmental topographical disorientation and decreased hippocampal functional connectivity.

Developmental topographical disorientation (DTD) is a newly discovered cognitive disorder in which individuals experience a lifelong history of getting lost in both novel and familiar surroundings. Recent studies have shown that such a selective orientation defect relies primarily on the inability of the individuals to form cognitive maps, i.e., mental representations of the surrounding that allow individuals to get anywhere from any location in the environment, although other orientation skills are additionally affected. To date, the neural correlates of this developmental condition are unknown. Here, we tested the hypothesis that DTD may be related to ineffective functional connectivity between the hippocampus (HC; known to be critical for cognitive maps) and other brain regions critical for spatial orientation. A group of individuals with DTD and a group of control subjects underwent a resting-state functional magnetic resonance imaging (rsfMRI) scan. In addition, we performed voxel-based morphometry to investigate potential structural differences between individuals with DTD and controls. The results of the rsfMRI study revealed a decreased functional connectivity between the right HC and the prefrontal cortex (PFC) in individuals with DTD. No structural differences were detected between groups. These findings provide evidence that ineffective functional connectivity between HC and PFC may affect the monitoring and processing of spatial information while moving within an environment, resulting in the lifelong selective inability of individuals with DTD to form cognitive maps that are critical for orienting in both familiar and unfamiliar surroundings.

A selective egocentric topographical working memory deficit in the early stages of Alzheimer's disease: a preliminary study.

The aim of this study was to determine whether an egocentric topographical working memory (WM) deficit is present in the early stages of Alzheimer's disease (AD) with respect to other forms of visuospatial WM. Further, we would investigate whether this deficit could be present in patients having AD without topographical disorientation (TD) signs in everyday life assessed through an informal interview to caregivers. Seven patients with AD and 20 healthy participants performed the Walking Corsi Test and the Corsi Block-Tapping Test. The former test requires memorizing a sequence of places by following a path and the latter is a well-known visuospatial memory task. Patients with AD also performed a verbal WM test to exclude the presence of general WM impairments. Preliminary results suggest that egocentric topographical WM is selectively impaired, with respect to visuospatial and verbal WM, even without TD suggesting an important role of this memory in the early stages of AD.

Rehabilitation in a complex case of topographical disorientation

This paper describes the rehabilitation process of a patient with severe topographical disorientation. The study demonstrates the sustained effects of a tailor-made, meticulous rehabilitation programme based on the gradual development of compensatory strategies. The patient (RB) had a memory impairment specific to environmental landmarks. He was able to recognise objects in his environment, but was unable to identify any salient object as a landmark and was also unable to derive any directional information from a chosen landmark. As such, his topographical disorientation syndrome was complex in that he had elements of both landmark agnosia and a heading disorientation, as described by Aguirre and D'Esposito (1999). Because of this dual damage to the exocentric framework, the tools and methods used in RB's rehabilitation programme were all based on his intact egocentric frame of reference. Remarkable training effects were found for routes he used frequently. After years of training he could walk these routes without the aid of the written information he had used previously, which can be interpreted as a form of implicit learning. In the 12 years we followed this patient some transfer occurred, as the patient was ultimately able to identify his own landmarks. However, RB remains dependent on other people to construct new routes for him on the basis of these landmarks.

Looking for the compass in a case of developmental topographical disorientation: A behavioral and neuroimaging study

Developmental topographical disorientation (DTD) is the presence of navigational deficits in the context of normal intellectual ability and in the absence of any perinatal, neurological, or psychiatric disorder. As only three cases of DTD have been fully described thus far, we are still unable to draw definitive conclusions about its nature and relationship with other visuospatial competencies, such as mental rotation. The case of Mr. L.A., a 38-year-old man with no history of neurological or psychiatric disorders, sheds some light on these open questions. A neuropsychological assessment including IQ, memory, visuospatial, visuoconstructive, and navigational tests showed that Mr. L.A. has pure navigational deficits affecting both route knowledge and cognitive map processing. Unlike previously described cases of DTD, Mr. L.A. was not affected by any other visuospatial or visuoconstructive deficits. In a functional magnetic resonance imaging (fMRI) task involving the recall of route knowledge, Mr. L.A. showed activation in the occipital areas, involved in low-level perceptual analysis of the stimuli, and showed no activation in the areas activated in controls with regard to route knowledge. The present case suggests that different types of DTD exist, which are characterized by different navigational difficulties and anomalous/lacking functional brain activities in specific navigational networks.

Seeing scenes: topographic visual hallucinations evoked by direct electrical stimulation of the parahippocampal place area.

In recent years, functional neuroimaging has disclosed a network of cortical areas in the basal temporal lobe that selectively respond to visual scenes, including the parahippocampal place area (PPA). Beyond the observation that lesions involving the PPA cause topographic disorientation, there is little causal evidence linking neural activity in that area to the perception of places. Here, we combined functional magnetic resonance imaging (fMRI) and intracranial EEG (iEEG) recordings to delineate place-selective cortex in a patient implanted with stereo-EEG electrodes for presurgical evaluation of drug-resistant epilepsy. Bipolar direct electrical stimulation of a cortical area in the collateral sulcus and medial fusiform gyrus, which was place-selective according to both fMRI and iEEG, induced a topographic visual hallucination: the patient described seeing indoor and outdoor scenes that included views of the neighborhood he lives in. By contrast, stimulating the more lateral aspect of the basal temporal lobe caused distortion of the patient's perception of faces, as recently reported (Parvizi et al., 2012). Our results support the causal role of the PPA in the perception of visual scenes, demonstrate that electrical stimulation of higher order visual areas can induce complex hallucinations, and also reaffirm direct electrical brain stimulation as a tool to assess the function of the human cerebral cortex.

I find my way in a maze but not in my own territory! Navigational processing in developmental topographical disorientation.

Developmental Topographical Disorientation (DTD) is a selective difficulty in way-finding showed by individuals with normal intellectual ability. We aimed to clarify whether getting lost even in familiar contexts could be because of a variety of developmental deficits that may affect the acquisition of one or more navigational competencies. Two DTD individuals were submitted to neuropsychological assessment and MRI exam, to verify the absence of further cognitive deficits and brain abnormalities; navigational and visuospatial tasks, and Radial Arm Maze (RAM) paradigms to assess spatial competencies; and switching paradigms to assess backward inhibition processes in spatial- and no-spatial contexts. DTD performances were compared with those of controls matched for gender, age, and education. DTD participants were not able to retrace a previously showed route, but they could recognize previously seen landmarks. They performed RAM paradigms without significant differences from controls and adopted complex navigational strategies as the cognitive mapping. It is interesting that DTD participants exhibited a peculiar alteration of Backward Inhibition, a mechanism that allows flexibly adapting to continuously changing environments. These findings demonstrate that getting lost for DTD participants did not imply the lack of basic navigational strategies and was not related to visuospatial or spatial memory deficits. We discuss the hypothesis that Backward Inhibition might play a role in updating environment representation during navigation.

The lost ability to find the way: Topographical disorientation after a left brain lesion.

We report the case of a patient (M.S.) who, after a left brain damage in posteromedial areas, showed a deficit in determining the direction of any destination with respect to his current position or to external frames (heading disorientation). Given that spatial cognition includes a wide range of cooperating abilities, we deemed that M.S.'s spatial disorientation could be ascribed to specific alterations within this multicomponent system where landmarks and spatial frames of reference contribute to organize information for different purposes. M.S. and 12 healthy elderly people (NCs) were submitted to an extensive neuropsychological assessment and to 2 ad hoc spatial tasks: (a) Object-Location Memory Task (what, where, and their binding); and (b) spatial memory task combining categorical (nonmetric)/coordinate (metric) relations with egocentric/allocentric frames of reference (in verbal and visuomotor conditions). M.S.'s performance was compared with that of NCs by means of a modified t test to small control sample size. M.S. met difficulty in positional processing and binding but not in object recognition. M.S. showed a selective deficit in the coordinate component in verbal (combined with both egocentric and allocentric frames) and visuomotor (only with the egocentric frame) spatial judgment tasks. In contrast, the categorical component looked always preserved in both frames of reference. The left posteromedial brain areas contribute in combining and translating metric relations according to frames of reference and in using these representations to guide actions according to an egocentric perspective.

Supplemental Material for I Find My Way in a Maze But Not in My Own Territory! Navigational Processing in Developmental Topographical Disorientation

Supplemental Material for I Find My Way in a Maze But Not in My Own Territory! Navigational Processing in Developmental Topographical Disorientation

Dromosagnosia, or why some people lose their sense of direction while driving

We coined a new word, “dromosagnosia”, from the Greek words, dromos (“way, road”) + agnosia, to describe the loss of direction while driving, an orientation disorder similar to but different from pure topographic disorientation. Historically, human beings have moved more quickly, from using domesticated animals to high speed vehicles, and this may be beyond the brain’s ability to react. Without the benefit of an automatic navigation system, automobiles are associated with more problems of dromosagnosia than are fast-moving aircraft or ships. Previous studies have noted that some areas of the brain are associated with spatial orientation, spatial memory, and even emotion, and abnormalities there could exacerbate the loss of sense of direction. We hypothesize that some people are especially disadvantaged from these brain differences and emotional disturbances when driving their cars. Functional magnetic resonance imaging (fMRI) and event-related potentials (ERP) studies combined with a virtual reality driving simulation might be used to find the areas of the brain related to dromosagnosia. Future applications: some people with dromosagnosia might benefit from special remedial training and a driving safety support system to avoid potential problems.

Cognitive and behavioral deficits following bilateral thalamic stroke: A longitudinal study

We describe behavioral and neuropsychological outcome of a patient (N.S.), who showed a bilateral paramedian thalamic ischemic lesion, with particular reference to the longitudinal evolution of topographical disorientation (TD) and confabulations. We report clinical neuropsychological/behavioral data over a 43-month follow-up. The results show early after the stroke a severe amnesic-confabulatory syndrome with dysexecutive deficits, associated with memory disorders both for visuo-spatial and verbal materials and TD both for known and new places. Behavioral disinhibition and anosognosia for cognitive deficits were also observed.All cognitive impairments have been recovered during the long-term follow-up.Bilateral paramedian thalamic infarcts often lead to severe and long-lasting neurological and cognitive impairments. Only a few cases showed good recovery. Our patient represents an interesting and uncommon case of bilateral paramedian thalamic syndrome with a significant neuropsychological recovery.

Retrosplenial Amnesia without Topographic Disorientation Caused by a Lesion in the Nondominant Hemisphere

We report the case of a 68-year-old right-handed man who was admitted to our hospital because of sudden onset of headache. On admission, he presented with left homonymous hemianopsia, disorientation, and recent memory disturbance; however, he had normal remote memory and digit span. He was able to recall the room layout of his house and describe the route from the nearest station to his home on a map. However, at the hospital, he sometimes lost his way because of amnesia. Computed tomography (CT) and magnetic resonance imaging revealed a subcortical hematoma in the right occipital forceps and the parietal lobe, involving the cingulate isthmus. Single-photon emission CT imaging showed reduced perfusion not only in the retrosplenial region but also in the right thalamus. These findings suggested that the retrosplenial amnesia might have been caused by the interruption of hippocampal input into the anterior thalamus.

Bilateral basal ganglia lesions as initial manifestation of CNS invasion in adult T-cell leukemia

We describe the case of a 67-year-old man who exhibited unsteadiness in walking, topographical disorientation, and urinary incontinence. Neurological examination revealed somnolence and mild weakness in the lower limbs with slight rigidity in the upper limbs. Cerebrospinal fluid examination showed pleocytosis with “flower cells” and an extremely high level of soluble interleukin-2 receptor. T2-weighted brain imaging revealed symmetrical high-intensity lesions in the bilateral caudate putamen. Positron emission tomography demonstrated intense uptake of 2-[fluorine-18]-fluoro-2-deoxy-d-glucose in the same region. He was diagnosed with central nervous system invasion by adult T-cell leukemia (ATL) and received chemotherapy. Interestingly, chemotherapy ameliorated the lesions and terminally caused the gray matter itself to atrophy in the bilateral caudate nuclei, which may be evidence for the direct infiltration of ATL tumors.

The Dide–Botcazo syndrome: Forgotten and misunderstood

Bilateral infarcts of the posterior cerebral arteries are associated with a range of visual and memory deficits. In 1902, Dide and Botcazo presented a clinico-pathological case study linking visual field defects, topographical disorientation, retro-anterograde amnesia and alexia with bilateral medial occipito-temporal lesions. Based on the findings they suggested the occipital lobe and inferior longitudinal fasciculus played an important role in memory. The combination of deficits was subsequently referred to on occasion as Dide–Botcazo syndrome but the term was largely forgotten until revived in the 1980s. More recently, some authors have included visual anosognosia – Anton's syndrome – in the syndrome, a feature that was not in the original case report. Here we present a historical review of Dide–Botcazo syndrome, illustrated with a recent case with almost identical clinical features to that described by Dide and Botcazo. Although Dide and Botcazo's theory of occipital amnesia has been superseded by developments in our understanding of the neurobiology of memory, it seems fitting to remember in some way their description of a clinical association of visual and memory deficits. We suggest Dide–Botcazo syndrome be used to describe a variant of vascular dementia, where visual field deficits are associated with memory impairment and, depending on the location of the vascular lesions, visual perceptual dysfunction, topographic, imagery or dreaming deficits.

Topographic disorientation following mesial left temporo-occipital hemorrhage

A 52-year-old left-handed man with a history of uncontrolled hypertension presented complaining of “memory problems.” Further description of his symptoms, however, revealed relatively isolated dysfunction in finding his way around previously known routes (audio clip at neurology.org/cp), despite recognition of landmarks. This was sudden in onset and nonprogressive. Formal neuropsychological testing revealed moderate impairment in verbal greater than visual memory, with additional impairments on tasks of working memory, processing speed, mental flexibility, and phonemic word fluency. Color naming and face recognition were normal. Brain MRI showed a mesial left temporo-occipital hemorrhage with extension into the left parahippocampal region (figure, A and B). Multiple subcortical T2 and fluid-attenuated inversion recovery hyperintensities were also evident (figure, B). Gradient-echo revealed multiple areas of hemosiderin, evidence of microbleeds (figure, C). A diagnosis of topographic disorientation due to mesial left temporal hemorrhage was made.