Topographical disorientation
Encyclopedia
Topographical disorientation is a cognitive disorder marked by the inability to orient in the surrounding. This disorder may result from the inability to make use of selective spatial information (e.g., environmental landmarks) or to orient by means of specific cognitive strategies such as the ability to form a mental representation of the environment, also known as a cognitive map
Cognitive map
Cognitive maps are a type of mental processing composed of a series of psychological transformations by which an individual can acquire, code, store, recall, and decode information about the relative locations and attributes of phenomena in their everyday or metaphorical spatial environment.The...

.

Diagnosis

Topographical Disorientation is usually diagnosed with the use of a comprehensive battery of neuropsychological tests combined with a variety of orientation tasks performed by the participants in both virtual and real surroundings. Performance on certain tests can identify underlying neurological disorder
Neurological disorder
A neurological disorder is a disorder of the body's nervous system. Structural, biochemical or electrical abnormalities in the brain, spinal cord, or in the nerves leading to or from them, can result in symptoms such as paralysis, muscle weakness, poor coordination, loss of sensation, seizures,...

s and verify the disorientation as a selective impairment. Brain imaging
Neuroimaging
Neuroimaging includes the use of various techniques to either directly or indirectly image the structure, function/pharmacology of the brain...

 is used to determine regions of brain damage
Brain damage
"Brain damage" or "brain injury" is the destruction or degeneration of brain cells. Brain injuries occur due to a wide range of internal and external factors...

, if any. Navigational skills can be assessed by tests pertaining to memory
Indirect tests of memory
Indirect memory tests assess the retention of information without direct reference to the source of information. Participants are given tasks designed to elicit knowledge that was acquired incidentally or unconsciously and is evident when performance shows greater inclination towards items...

, visual-perceptual abilities
Visual perception
Visual perception is the ability to interpret information and surroundings from the effects of visible light reaching the eye. The resulting perception is also known as eyesight, sight, or vision...

, object recognition, mental rotation
Mental rotation
Mental rotation is the ability to rotate mental representations of two-dimensional and three-dimensional objects.-Introduction:Mental rotation is somewhat localized to the right cerebral hemisphere. It is thought to take place largely in the same areas as perception...

, imagery abilities, and spatial abilities. More direct testing of navigation involves asking the patient to describe a route
Route
Route may refer to:* Route or thoroughfare for transportation* Route number or road number*Trade route, a commonly used path for the passage of goods*Scenic route, a thoroughfare designated as scenic based on the scenery through which it passes...

, read a map
Map
A map is a visual representation of an area—a symbolic depiction highlighting relationships between elements of that space such as objects, regions, and themes....

, draw a map, follow a route, or point out landmarks.

Acquired topographical disorientation

Acquired Topographical Disorientation refers to the inability to orient in the surrounding as a result of focal brain damage
Focal and diffuse brain injury
Focal and diffuse brain injury are ways to classify brain injury: focal injury occurs in a specific location, while diffuse injury occurs over a more widespread area. It is common for both focal and diffuse damage to occur as the result of the same event; many traumatic brain injuries have aspects...

.
Acquired Topographical Disorientation has been studied for decades using case studies
Case study
A case study is an intensive analysis of an individual unit stressing developmental factors in relation to context. The case study is common in social sciences and life sciences. Case studies may be descriptive or explanatory. The latter type is used to explore causation in order to find...

 of patient
Patient
A patient is any recipient of healthcare services. The patient is most often ill or injured and in need of treatment by a physician, advanced practice registered nurse, veterinarian, or other health care provider....

s who have selectively lost their ability to find their way within large-scale, locomotor environments. Several dozen case reports of topographical disorientation have been presented over the last century. Studying these people will aid in the understanding of the complex, multi-component behavior of navigation. General cognitive impairments such as amnesia
Amnesia
Amnesia is a condition in which one's memory is lost. The causes of amnesia have traditionally been divided into categories. Memory appears to be stored in several parts of the limbic system of the brain, and any condition that interferes with the function of this system can cause amnesia...

, dementia
Dementia
Dementia is a serious loss of cognitive ability in a previously unimpaired person, beyond what might be expected from normal aging...

, intellectual disability
Intellectual disability
Intellectual disability is a broad concept encompassing various intellectual deficits, including mental retardation , deficits too mild to properly qualify as MR, various specific conditions , and problems acquired later in life through acquired brain injuries or neurodegenerative diseases like...

, hemispatial neglect
Hemispatial neglect
Hemispatial neglect, also called hemiagnosia, hemineglect, unilateral neglect, spatial neglect, unilateral visual inattention, hemi-inattention or neglect syndrome is a neuropsychological condition in which, after damage to one hemisphere of the brain, a deficit in attention to and awareness of...

 as well as diseases affecting brain function such as meningitis
Meningitis
Meningitis is inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. The inflammation may be caused by infection with viruses, bacteria, or other microorganisms, and less commonly by certain drugs...

, Alzheimer's disease
Alzheimer's disease
Alzheimer's disease also known in medical literature as Alzheimer disease is the most common form of dementia. There is no cure for the disease, which worsens as it progresses, and eventually leads to death...

, stroke
Stroke
A stroke, previously known medically as a cerebrovascular accident , is the rapidly developing loss of brain function due to disturbance in the blood supply to the brain. This can be due to ischemia caused by blockage , or a hemorrhage...

 can result in topographic disorientation.

Egocentric disorientation

Egocentric disorientation is marked by the inability to represent the location of objects with respect to self. This is usually due to lesion
Lesion
A lesion is any abnormality in the tissue of an organism , usually caused by disease or trauma. Lesion is derived from the Latin word laesio which means injury.- Types :...

s in the posterior parietal lobe. Patients experience no difficulty recognizing or naming people or objects. They are unable to accurately reach for visual objects or state the relationship between two objects (above, below, left, right, nearer or farther).
In a case study presented by Stark and colleagues, a patient named GW described the inability to accurately reach for visual targets despite normal vision. She had no difficulty recognizing and naming objects presented to her, but was unable to point to locations of targets defined by visual
Visual system
The visual system is the part of the central nervous system which enables organisms to process visual detail, as well as enabling several non-image forming photoresponse functions. It interprets information from visible light to build a representation of the surrounding world...

, proprioceptive
Proprioception
Proprioception , from Latin proprius, meaning "one's own" and perception, is the sense of the relative position of neighbouring parts of the body and strength of effort being employed in movement...

, or audio
Auditory system
The auditory system is the sensory system for the sense of hearing.- Outer ear :The folds of cartilage surrounding the ear canal are called the pinna...

 input. The loss of a egocentric spatial representation system left her unable to position herself in space. Most indicative of her disability is that she often turned in the wrong direction when greeted by someone who she was not facing

Heading disorientation

Heading disorientation is marked by the inability to represent direction of orientation with respect to external environment. This is usually due to lesions in the posterior cingulate
Posterior cingulate
The posterior cingulate cortex is the backmost part of the cingulate cortex, lying behind the anterior cingulate cortex.This is the upper part of the "limbic lobe". The cingulate cortex is made up of an area around the midline of the brain...

. Patients show no signs of visuo-spatial agnosia
Agnosia
Agnosia is a loss of ability to recognize objects, persons, sounds, shapes, or smells while the specific sense is not defective nor is there any significant memory loss...

. Patients are able to determine their location using landmarks, but are unable to determine which direction to proceed from those landmarks in order to reach their destination. They are also impaired in map drawing tasks and are unable to describe routes between familiar locations.
Takahashi and colleagues presented three cases of focal brain damage
Focal and diffuse brain injury
Focal and diffuse brain injury are ways to classify brain injury: focal injury occurs in a specific location, while diffuse injury occurs over a more widespread area. It is common for both focal and diffuse damage to occur as the result of the same event; many traumatic brain injuries have aspects...

 to the right retrosplenial region
Retrosplenial region
The retrosplenial region is a brain area and part of the cingulate cortex. It is defined by Brodmann area 26, Brodmann area 29 and Brodmann area 30.-Function:...

 through a cerebral hemorrhage that caused a loss in sense of direction. All three patients showed normal perception, were able to identify familiar buildings and landscapes, were able to determine and remember locations of objects that could be seen from where they were standing, but were unable to recall direction from selective familiar landmarks. Symptoms of topographical disorientation disappeared in all three patients after two months.

Landmark agnosia

Landmark agnosia
Agnosia
Agnosia is a loss of ability to recognize objects, persons, sounds, shapes, or smells while the specific sense is not defective nor is there any significant memory loss...

 is marked by the inability to recognize salient
Salient
Salient may refer to:* See Salients, re-entrants and pockets for the battlefield feature* Salient , part of a discrete territory projecting out of the main portion, bordered by foreign territory on three sides, into which it projects...

 environmental stimuli such as landmarks. This is usually due to lesions in the lingual gyrus
Lingual gyrus
The lingual gyrus of the occipital lobe lies between the calcarine sulcus and the posterior part of the collateral sulcus; behind, it reaches the occipital pole; in front, it is continued on to the tentorial surface of the temporal lobe, and joins the parahippocampal gyrus...

. Patients are able to draw detailed maps and visualize
Mental image
A mental image is an experience that, on most occasions, significantly resembles the experience of perceiving some object, event, or scene, but occurs when the relevant object, event, or scene is not actually present to the senses...

 places familiar to them before the illness. They can distinguish between classes of buildings, such as house or skyscraper, but are unable to identify specific buildings, such as their own house or famous landmarks. Patients can navigate using strictly spatial information and specific details of landmarks such as house number or door color.
C. A. Pallis described a patient, A.H., who presented with color
Achromatopsia
Achromatopsia , is a medical syndrome that exhibits symptoms relating to at least five separate individual disorders. Although the term may refer to acquired disorders such as color agnosia and cerebral achromatopsia, it typically refers to an autosomal recessive congenital color vision disorder,...

, face
Prosopagnosia
Prosopagnosia is a disorder of face perception where the ability to recognize faces is impaired, while the ability to recognize other objects may be relatively intact...

 and landmark agnosia as a result of a cerebral embolism.

Anterograde disorientation

Anterograde
Anterograde amnesia
Anterograde amnesia is a loss of the ability to create new memories after the event that caused the amnesia, leading to a partial or complete inability to recall the recent past, while long-term memories from before the event remain intact. This is in contrast to retrograde amnesia, where memories...

 disorientation is marked by the inability to orient in new environments. This is usually due to lesions in the parahippocampus. Patients were able to navigate through and draw maps of environments learned at least 6 months before the damage.

Teng and colleagues tested a profoundly amnesic patient who has complete bilateral damage to the hippocampus
Hippocampus
The hippocampus is a major component of the brains of humans and other vertebrates. It belongs to the limbic system and plays important roles in the consolidation of information from short-term memory to long-term memory and spatial navigation. Humans and other mammals have two hippocampi, one in...

 and extensive damage to adjacent structures in the medial temporal lobe
Temporal lobe
The temporal lobe is a region of the cerebral cortex that is located beneath the Sylvian fissure on both cerebral hemispheres of the mammalian brain....

. The patient was able to recall the spatial layout of his hometown where he moved away from 50 years ago, before he became amnesic. The patient has no knowledge of his current neighborhood, where he moved to after his injury. The finding indicates that the medial temporal lobe is not needed for the retrieval
Knowledge retrieval
Knowledge Retrieval seeks to return information in a structured form, consistent with human cognitive processes as opposed to simple lists of data items...

 of spatial maps learned prior to injury. However, the hippocampus and other surrounding structures are essential for the formation of long-term declarative memories
Declarative memory
Declarative memory is one of two types of long term human memory. It refers to memories which can be consciously recalled such as facts and knowledge. Its counterpart is known as non-declarative or Procedural memory, which refers to unconscious memories such as skills...

, including spatial memories.

Developmental topographical disorientation

Developmental Topographical Disorientation (also known as DTD) refers to the inability to orient since childhood
Childhood
Childhood is the age span ranging from birth to adolescence. In developmental psychology, childhood is divided up into the developmental stages of toddlerhood , early childhood , middle childhood , and adolescence .- Age ranges of childhood :The term childhood is non-specific and can imply a...

 despite any brain damage, neurological condition or general cognitive defects. Individuals affected by DTD are unable to generate a mental representation of the environment (i.e. a cognitive map) and therefore unable to make use of it while orienting (a process that usually people go through while orienting). Not to be confused with healthy individuals who have a poor sense of direction, individuals affected by DTD get lost in very familiar surroundings, such as their house
House
A house is a building or structure that has the ability to be occupied for dwelling by human beings or other creatures. The term house includes many kinds of different dwellings ranging from rudimentary huts of nomadic tribes to free standing individual structures...

 or neighborhood, daily.

Case one

A woman in Vancouver
Vancouver
Vancouver is a coastal seaport city on the mainland of British Columbia, Canada. It is the hub of Greater Vancouver, which, with over 2.3 million residents, is the third most populous metropolitan area in the country,...

, referred to as Pt1, presented with topographical disorientation in absence of any structural lesions. Despite normal cognitive development
Cognitive development
Cognitive development is a field of study in neuroscience and psychology focusing on a child's development in terms of information processing, conceptual resources, perceptual skill, language learning, and other aspects of brain development and cognitive psychology compared to an adult's point of...

, she has never been able to orient in the environment. Further testing showed that she was able to follow route based, landmark based, and verbal directions to reach a destination in an urban environment. In map based testing, Pt1 was able to follow a route traced on a map but she was unable to draw a detailed schematic map of her house or any other familiar surrounding. Detailed orientation tests revealed that she had a selective impairment in the ability to create mental representation of the environments (i.e., cognitive maps). This inability did not allow Pt1 to become familiar with the locations (and spatial relationships) of different landmarks in the surrounding, resulting in getting lost at every attempt.

Other cases

F.G. is a normally developed 22 year old male with more severe symptoms than Pt1. He has no medical history of motor, neurological, or cognitive developmental delays or of neurological or psychiatric diseases. He has been unable to find his bearings in an environment since childhood. F.G. is unable to form a cognitive map, identify landmarks, derive verbal instructions used in navigation, retain a previously learned path, or read a map. He is able to accomplish rudimentary navigational tasks, such as those in a virtual environment, by adopting a verbal strategy. F.G. is unable to segregate and identify a landmark in his environment or determine location or directional information from a landmark he recognizes.

Emergent Disorder

Developmental Topographical Disorientation has been recently documented in 120 new cases using an online assessment
Assessment
Educational assessment is the process of documenting, usually in measurable terms, knowledge, skills, attitudes and beliefs. Assessment can focus on the individual learner, the learning community , the institution, or the educational system as a whole...

.
Participants in this study reported getting lost daily or 1 to 5 times a week in familiar surroundings such as their neighborhood or house and having this difficulty since childhood. They have no other cognitive difficulties that affect daily activities. The online tests evaluated their object and face recognition ability, landmark recognition, ability to recall directional information, left/right orientation, and ability to reverse a route to return to starting position. The final two tests involved the formation and use of a cognitive map. The test provided objective confirmation that DTD is related to the inability to create cognitive maps. Future research will include in-person testing to further evaluate orientation skills in a real-world environment along with neuropsychological evaluation
Neuropsychological assessment
Neuropsychological assessment was traditionally carried out to assess the extent of impairment to a particular skill and to attempt to locate an area of the brain which may have been damaged after brain injury or neurological illness...

 and structural imaging
Neuroimaging
Neuroimaging includes the use of various techniques to either directly or indirectly image the structure, function/pharmacology of the brain...

 to rule out cognitive defects.

Treatment

Treatment for topographical disorientation have been achieved through a case by case basis. Prognosis is largely dependent on the organic cause. Neuropsychological assessment followed by an assessment of unaffected cognitive abilities can be employed in therapy. Treatment for recovering navigational skills require strengthening unaffected navigational strategies to bypass defective ones.

Case study

In a case study presented by Incoccia and colleagues
, a young woman known as MGC showed an inability to orient herself in her environment or use a map. She presented with a cerebral malformation bilaterally involving retrorolandic
Central sulcus
-External links:* via the Neuroscience Information Framework...

 regions, but developed to normal cognitive levels. Unlike most cases of topographical disorientation, where patients become impaired after their navigational skill was completely developed, the congenital
Congenital disorder
A congenital disorder, or congenital disease, is a condition existing at birth and often before birth, or that develops during the first month of life , regardless of causation...

 malformation prevented MGC to develop her navigation skills.

Evaluation

An extensive neuropsychological assessment of general intelligence
Intelligence
Intelligence has been defined in different ways, including the abilities for abstract thought, understanding, communication, reasoning, learning, planning, emotional intelligence and problem solving....

, attention
Attention
Attention is the cognitive process of paying attention to one aspect of the environment while ignoring others. Attention is one of the most intensely studied topics within psychology and cognitive neuroscience....

, memory
Memory
In psychology, memory is an organism's ability to store, retain, and recall information and experiences. Traditional studies of memory began in the fields of philosophy, including techniques of artificially enhancing memory....

, and visuo
Visual perception
Visual perception is the ability to interpret information and surroundings from the effects of visible light reaching the eye. The resulting perception is also known as eyesight, sight, or vision...

-spatial
Depth perception
Depth perception is the visual ability to perceive the world in three dimensions and the distance of an object. Depth sensation is the ability to move accurately, or to respond consistently, based on the distances of objects in an environment....

 perception was conducted on MGC.
MGC did not show any signs of apraxia
Apraxia
Apraxia is a disorder caused by damage to specific areas of the cerebrum. Apraxia is characterized by loss of the ability to execute or carry out learned purposeful movements, despite having the desire and the physical ability to perform the movements...

, hemispatial neglect
Hemispatial neglect
Hemispatial neglect, also called hemiagnosia, hemineglect, unilateral neglect, spatial neglect, unilateral visual inattention, hemi-inattention or neglect syndrome is a neuropsychological condition in which, after damage to one hemisphere of the brain, a deficit in attention to and awareness of...

, visual imagery defect or agnosia for faces
Prosopagnosia
Prosopagnosia is a disorder of face perception where the ability to recognize faces is impaired, while the ability to recognize other objects may be relatively intact...

, colors
Color agnosia
Color agnosia , is a medical or psychological condition that prevents a person from correctly associating hue names with common objects. The sufferer retains the ability of distinguishing hues...

, or objects. She was further tested on her navigational skills using real navigational tasks in experimental and ecological environment
City
A city is a relatively large and permanent settlement. Although there is no agreement on how a city is distinguished from a town within general English language meanings, many cities have a particular administrative, legal, or historical status based on local law.For example, in the U.S...

, map drawing, and cognitive abilities relevant for navigation. MGC did not perform within normal range for visuo-spatial memory, mental rotation, and line orientation tasks.

Training

MGC was trained to develop a navigational strategy that she could independently implement to reach unfamiliar places by following routes never before taken. MGC's topographical abilities cannot be restored, but rather alternative strategies, such as language-based navigation, were developed. She was trained to use verbal instructions to follow a path and describe paths in novel environments. After extensive practice, MGC was re-evaluated and showed great improvement in her navigational skills along with increased self-confidence, enthusiasm, and positive outlook.

See also

  • Cognitive map
    Cognitive map
    Cognitive maps are a type of mental processing composed of a series of psychological transformations by which an individual can acquire, code, store, recall, and decode information about the relative locations and attributes of phenomena in their everyday or metaphorical spatial environment.The...

  • Navigation
    Navigation
    Navigation is the process of monitoring and controlling the movement of a craft or vehicle from one place to another. It is also the term of art used for the specialized knowledge used by navigators to perform navigation tasks...

  • Path integration
    Path integration
    Path integration is the name given to the method thought to be used by animals for dead reckoning.Charles Darwin and J.J. Murphy first postulated an inertially-based navigation system in animals in 1873...

  • Hippocampus
    Hippocampus
    The hippocampus is a major component of the brains of humans and other vertebrates. It belongs to the limbic system and plays important roles in the consolidation of information from short-term memory to long-term memory and spatial navigation. Humans and other mammals have two hippocampi, one in...

  • Place cells
  • Grid cells
    Grid cells
    A grid cell is a type of neuron that has been found in the brains of rats and mice; and it is likely to exist in other animals including humans...

  • Head direction cells
    Head direction cells
    Many mammals possess neurons called head direction cells, which are active only when the animal's head points in a specific direction within an environment. These neurons fire at a steady rate Many mammals possess neurons called head direction (HD) cells, which are active only when the animal's...

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