Signs and symptoms
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| Anosodiaphoria | |
|---|---|
| Specialty | Neurology |
Anosodiaphoria is a condition in which a person who has a brain injury seems indifferent to the existence of their impairment. Anosodiaphoria is specifically used in association with indifference to paralysis. It is a somatosensory agnosia, or a sign of neglect syndrome. [1] It might be specifically associated with defective functioning of the frontal lobe of the right hemisphere. [2]
Joseph Babinski first used the term anosodiaphoria in 1914 to describe a disorder of the body schema in which patients verbally acknowledge a clinical problem (such as hemiparesis) but fail to be concerned about it. [3] Anosodiaphoria follows a stage of anosognosia, in which there may be verbal, explicit denial of the illness, and after several days to weeks, develop the lack of emotional response. [4] Indifference is different from denial because it implies a lack of caring on the part of the patient, who otherwise acknowledges his or her deficit.[ citation needed ]
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A few possible explanations for anosodiaphoria exist:
Other explanations include reduced emotional experience, impaired emotional communication, alexithymia, behavioral abnormalities, dysexecutive syndrome, and the frontal lobes. [6]
Anosodiaphoria occurs after stroke of the brain. 27% of patients with an acute hemispheric stroke had the stroke in the right hemisphere, while 2% have it in their left. [7]
Anosodiaphoria is thought to be related to unilateral neglect, a condition often found after damage to the non-dominant (usually the right) hemisphere of the cerebral cortex in which patients seem unable to attend to, or sometimes comprehend, anything on a certain side of their body (usually the left).[ citation needed ]
The frontal lobe is thought to be the primary area for the lack of emotional insight seen in anosodiaphoria, such as in frontotemporal dementia. A recent 2011 study done by Mendez and Shapira found that people with frontotemporal dementia also had a loss of insight more properly described at "frontal anosodiaphoria", a lack of concern for proper self-appraisal. Patients were found to have a lack of emotional updating, or concern for having an illness; an absence of an emotional self-referent tagging of information on their disorder, which they think is possibly from disease in the ventromedial prefrontal cortex, anterior cingulate-anterior insula area, especially on the right. [8]
Indifference to illness may have an adverse impact on a patient's engagement in neurological rehabilitation, cognitive rehabilitation and physical rehabilitation. Patients are not likely to implement rehabilitation for a condition about which they are indifferent. Although anosognosia often resolves in days to weeks after stroke, anosodiaphoria often persists. [9] Therefore, the therapist has to be creative in their rehabilitation approach in order to maintain the interest of the patient.[ citation needed ]
Aphasia is an inability to comprehend or formulate language because of damage to specific brain regions. The major causes are stroke and head trauma; prevalence is hard to determine but aphasia due to stroke is estimated to be 0.1–0.4% in the Global North. Aphasia can also be the result of brain tumors, brain infections, or neurodegenerative diseases.
Anosognosia is a condition in which a person with a disability is cognitively unaware of having it due to an underlying physical or psychological condition. Anosognosia can result from physiological damage to brain structures, typically to the parietal lobe or a diffuse lesion on the fronto-temporal-parietal area in the right hemisphere, and is thus a neuropsychiatric disorder. A deficit of self-awareness, it was first named by the neurologist Joseph Babinski in 1914. Phenomenologically, anosognosia has similarities to denial, which is a psychological defense mechanism; attempts have been made at a unified explanation. Anosognosia is sometimes accompanied by asomatognosia, a form of neglect in which patients deny ownership of body parts such as their limbs. The term is from Ancient Greek ἀ- a-, 'without', νόσος nosos, 'disease' and γνῶσις gnōsis, 'knowledge'. It is also considered a disorder that makes the treatment of the patient more difficult, since it may affect negatively the therapeutic relationship.
Frontotemporal dementia (FTD), or frontotemporal degeneration disease, or frontotemporal neurocognitive disorder, encompasses several types of dementia involving the progressive degeneration of frontal and temporal lobes. FTDs broadly present as behavioral or language disorders with gradual onsets. Common signs and symptoms include significant changes in social and personal behavior, apathy, blunting of emotions, and deficits in both expressive and receptive language. Currently, there is no cure for FTD, but there are treatments that help alleviate symptoms.
Hemispatial neglect is a neuropsychological condition in which, after damage to one hemisphere of the brain, a deficit in attention and awareness towards the side of space opposite brain damage is observed. It is defined by the inability of a person to process and perceive stimuli towards the contralesional side of the body or environment. Hemispatial neglect is very commonly contralateral to the damaged hemisphere, but instances of ipsilesional neglect have been reported.
In neurology, abulia, or aboulia, refers to a lack of will or initiative and can be seen as a disorder of diminished motivation (DDM). Abulia falls in the middle of the spectrum of diminished motivation, with apathy being less extreme and akinetic mutism being more extreme than abulia. The condition was originally considered to be a disorder of the will, and aboulic individuals are unable to act or make decisions independently; and their condition may range in severity from subtle to overwhelming. In the case of akinetic mutism, many patients describe that as soon as they "will" or attempt a movement, a "counter-will" or "resistance" rises up to meet them.
Witzelsucht is a set of pure and rare neurological symptoms characterized by a tendency to make puns, or tell inappropriate jokes or pointless stories in socially inappropriate situations. It makes one unable to read sarcasm.
In psychology, disinhibition is a lack of restraint manifested in disregard of social conventions, impulsivity, and poor risk assessment. Disinhibition affects motor, instinctual, emotional, cognitive, and perceptual aspects with signs and symptoms similar to the diagnostic criteria for mania. Hypersexuality, hyperphagia, and aggressive outbursts are indicative of disinhibited instinctual drives.
Semantic dementia (SD), also known as semantic variant primary progressive aphasia (svPPA), is a progressive neurodegenerative disorder characterized by loss of semantic memory in both the verbal and non-verbal domains. However, the most common presenting symptoms are in the verbal domain. Semantic dementia is a disorder of semantic memory that causes patients to lose the ability to match words or images to their meanings. However, it is fairly rare for patients with semantic dementia to develop category specific impairments, though there have been documented cases of it occurring. Typically, a more generalized semantic impairment results from dimmed semantic representations in the brain.
Progressive nonfluent aphasia (PNFA) is one of three clinical syndromes associated with frontotemporal lobar degeneration. PNFA has an insidious onset of language deficits over time as opposed to other stroke-based aphasias, which occur acutely following trauma to the brain. The specific degeneration of the frontal and temporal lobes in PNFA creates hallmark language deficits differentiating this disorder from other Alzheimer-type disorders by the initial absence of other cognitive and memory deficits. This disorder commonly has a primary effect on the left hemisphere, causing the symptomatic display of expressive language deficits and sometimes may disrupt receptive abilities in comprehending grammatically complex language.
Reduplicative paramnesia is the delusional belief that a place or location has been duplicated, existing in two or more places simultaneously, or that it has been 'relocated' to another site. It is one of the delusional misidentification syndromes; although rare, it is most commonly associated with acquired brain injury, particularly simultaneous damage to the right cerebral hemisphere and to both frontal lobes.
Frontal lobe disorder, also frontal lobe syndrome, is an impairment of the frontal lobe of the brain due to disease or frontal lobe injury. The frontal lobe plays a key role in executive functions such as motivation, planning, social behaviour, and speech production. Frontal lobe syndrome can be caused by a range of conditions including head trauma, tumours, neurodegenerative diseases, neurodevelopmental disorders, neurosurgery and cerebrovascular disease. Frontal lobe impairment can be detected by recognition of typical signs and symptoms, use of simple screening tests, and specialist neurological testing.
Dysexecutive syndrome (DES) consists of a group of symptoms, usually resulting from brain damage, that fall into cognitive, behavioural and emotional categories and tend to occur together. The term was introduced by Alan Baddeley to describe a common pattern of dysfunction in executive functions, such as planning, abstract thinking, flexibility and behavioural control. It is thought to be Baddeley's hypothesized working memory system and the central executive that are the hypothetical systems impaired in DES. The syndrome was once known as frontal lobe syndrome; however 'dysexecutive syndrome' is preferred because it emphasizes the functional pattern of deficits over the location of the syndrome in the frontal lobe, which is often not the only area affected.
The neuroanatomy of memory encompasses a wide variety of anatomical structures in the brain.
Anton syndrome, also known as Anton-Babinski syndrome and visual anosognosia, is a rare symptom of brain damage occurring in the occipital lobe. Those who have it are cortically blind, but affirm, often quite adamantly and in the face of clear evidence of their blindness, that they are capable of seeing. Failing to accept being blind, people with Anton syndrome dismiss evidence of their condition and employ confabulation to fill in the missing sensory input. It is named after the neurologist Gabriel Anton. Only 28 cases have been published.
Amorphosynthesis, also called a hemi-sensory deficit, is a neuropsychological condition in which a patient experiences unilateral inattention to sensory input. This phenomenon is frequently associated with damage to the right cerebral hemisphere resulting in severe sensory deficits that are observed on the contralesional (left) side of the body. A right-sided deficit is less commonly observed and the effects are reported to be temporary and minor. Evidence suggests that the right cerebral hemisphere has a dominant role in attention and awareness to somatic sensations through ipsilateral and contralateral stimulation. In contrast, the left cerebral hemisphere is activated only by contralateral stimuli. Thus, the left and right cerebral hemispheres exhibit redundant processing to the right-side of the body and a lesion to the left cerebral hemisphere can be compensated by the ipsiversive processes of the right cerebral hemisphere. For this reason, right-sided amorphosynthesis is less often observed and is generally associated with bilateral lesions.
Phonagnosia is a type of agnosia, or loss of knowledge, that involves a disturbance in the recognition of familiar voices and the impairment of voice discrimination abilities in which the affected individual does not suffer from comprehension deficits. Phonagnosia is an auditory agnosia, an acquired auditory processing disorder resulting from brain damage, other auditory agnosias include cortical deafness and auditory verbal agnosia also known as pure word deafness.
Right hemisphere brain damage (RHD) is the result of injury to the right cerebral hemisphere. The right hemisphere of the brain coordinates tasks for functional communication, which include problem solving, memory, and reasoning. Deficits caused by right hemisphere brain damage vary depending on the location of the damage.
Cerebellar cognitive affective syndrome (CCAS), also called Schmahmann's syndrome is a condition that follows from lesions (damage) to the cerebellum of the brain. It refers to a constellation of deficits in the cognitive domains of executive function, spatial cognition, language, and affect resulting from damage to the cerebellum. Impairments of executive function include problems with planning, set-shifting, abstract reasoning, verbal fluency, and working memory, and there is often perseveration, distractibility and inattention. Language problems include dysprosodia, agrammatism and mild anomia. Deficits in spatial cognition produce visual–spatial disorganization and impaired visual–spatial memory. Personality changes manifest as blunting of affect or disinhibited and inappropriate behavior. These cognitive impairments result in an overall lowering of intellectual function. CCAS challenges the traditional view of the cerebellum being responsible solely for regulation of motor functions. It is now thought that the cerebellum is responsible for monitoring both motor and nonmotor functions. The nonmotor deficits described in CCAS are believed to be caused by dysfunction in cerebellar connections to the cerebral cortex and limbic system.
The mini-SEA is a neuropsychological battery aiming to evaluate the impairment of the social and emotional cognition. Developed by Maxime Bertoux in 2012, the mini-SEA has been preferentially designed for the assessment, follow-up and diagnosis of neurodegenerative diseases such as the frontotemporal dementia, but is more generally designed to evaluate the integrity of the frontal lobes.
Asomatognosia is a neurological disorder characterized as loss of recognition or awareness of part of the body. The failure to acknowledge, for example, a limb, may be expressed verbally or as a pattern of neglect. The limb may also be attributed to another person, a delusion known as somatoparaphrenia. However, they can be shown their limb and this error is temporarily corrected. Some authors have focused on the prevalence of hemispatial neglect in such patients.