Prosopometamorphopsia

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Prosopometamorphopsia (sometimes known as "demon face syndrome" [1] [2] ) is a visual disorder characterized by altered perceptions of faces. In the perception of a person with the disorder, facial features are distorted in a variety of ways including drooping, swelling, discoloration, and shifts of position. Prosopometamorphopsia is distinct from prosopagnosia, which is characterised by the inability to recognise faces. About 75 cases of prosopometamorphopsia have been reported in the scientific literature. [3] In about half of the reported cases, features on both sides of the face appear distorted. In the other half of cases, distortions are restricted to one side of the face (left or right) and this condition is called hemi-prosopometamorphopsia.

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Presentation

Generally, faces tend to appear distorted to a person who is affected by this condition. [4] Those who suffer from this condition are able to recognise faces normally but perceive them as strangely disfigured. These facial hallucinations are usually described as ugly, and have prominent eyes and teeth. [5] Some have described the faces as having a cartoon-like quality. Faces have been known to be perceived as contorted and as having displaced features. [6] For example, one patient described a person's face as having a nose deviated to the side, the mouth lying at a diagonal and one eyebrow being higher than the other. [6] Prosopometamorphopsia may either involve perceptions of the whole face or only one side of the face (usually after right hemisphere damage). [7]

Causes

The definitive cause for prosopometamorphopsia is still unknown. However, several potential theories have been expressed in the literature in this area. Generally, this condition has been associated with damage or abnormalities in various brain areas (temporal, occipital, parietal, and frontal lobes). [4] The development of prosopometamorphopsia has been recorded to be a manifestation of epilepsy in some cases. [4] Hyperactivity in the core or distributed face areas (without lesions) may be connected to causation.

Functional imaging studies in humans have identified an area in the fusiform gyrus which is selectively activated by stimulation when exposed to faces called the fusiform face area (FFA). [5] Another area known to be activated by face stimuli is the superior temporal sulcus (STS). This region is particularly active when having to process facial expressions, especially the expressive features related to the eyes. Therefore, it has been suggested that the prominent eyes (which is a typical feature in the facial perceptions) in the distortions recorded is more in accordance with increased activity (which would cause an overrepresentation of the eyes) within the STS rather than the fusiform face area. [5]

Other studies, however, have found that stimulation of the posterior and mid-fusiform face selective regions in a patient with medication-resistant epilepsy resulted in perceptions consistent with that of facial metamorphoses (patient noted that the experimenter's face started to droop). [4] This study found that the perceived distortions correlated with signal changes in the patient's FFA, and not in the STS.

Diagnosis

Classification

Prosopometamorphopsia is considered a face hallucination and is included under the umbrella of complex visual hallucinations. [8] Unlike other forms of hallucinations such as peduncular hallucinosis or Charles Bonnet syndrome, prosopometamorphopsia does not predominate at a particular time of day; it is a constant experience. [8] However, patients with Charles Bonnet syndrome have noted descriptions of prosopometamorphopsia. [6] This form of perceptual distortion along with others such as macropsia and micropsia (alteration of perceived object size) and palinopia (spatial and temporal varieties and polyopia) are classified under the category termed metamorphopsia. [9] These facial distortions can occur to either hallucinated perceptions or true (non-hallucinated) perceptions. [6] It is attributed to structural brain changes or functional disorders like epilepsy, migraine or eye diseases. [10]

Treatment

Antidepressants such as citalopram and the antipsychotic quetiapine have been recorded as unable to facilitate improvements for these symptoms. [10] Valproic acid was initially used to treat the woman who hallucinated the dragon-like faces and this alleviated her symptoms entirely, however, she went on to develop auditory hallucinations as a side effect. [10] She was subsequently prescribed rivastigmine instead which reduced the auditory hallucinations and reduced her visual symptoms.

The 75-year-old woman in the previous study was treated with intravenous heparin infusion and coumadization over a period of 10 days which enabled the alleviation of her visual symptoms almost entirely. [7] The type of treatment may vary depending on the cause of the facial distortions.

Case studies

One study reported a 24-year-old woman who developed prosopometamorphopsia after a childbirth. [9] Initially she developed severe migraines and blurred vision mainly in her right visual hemifield and was in a confused state. The visual disturbances persisted long after the migraine had subsided several hours later. She described the left half of people's faces as "out of place" and would see these distortions irrespective of whether the faces were familiar or unknown. However, she was able to visualise the faces of familiar people in her mind without the distortions. She also did not report perceiving distortions in stimuli other than faces and demonstrated the same patterns a year after the first assessments. It was discovered that this woman had a left hemisphere lesion which resulted in distortions of the left half of the faces to which she was exposed. The unilateral aspect of the defect suggests that the early stages of face processing occurs in parallel mechanisms across both hemispheres and the right hemisphere then integrates the information that results in a unitary face representation. [9]

Another study examined a 75-year-old woman who suffered from a sudden onset of nausea, dizziness and blurred vision. [7] The central part of faces, especially the nose and mouth, were described as being out of shape. She claimed that noses looked narrow and lengthened toward the mouth which looked small and rounded regardless of whether the faces were familiar to her or not. She was found not to have any other impairments in her visuoperceptual performances, nor did she have any cognitive or psychiatric impairments. A T2-weighted brain MRI revealed an infarction in the right medial temporooccipital lobe including the parahippocampal gyrus (complement of the FFA).

A 52-year-old woman suffered from a lifelong history of seeing faces morph into dragon-like faces and reported hallucinating similar faces several times a day. [10] Initially she would recognise the actual faces but after a while they would become black, grew long pointy ears and a protruding snout, displayed reptile-like skin and had large protruding eyes in bright colours. She would see these faces coming towards her several times in a day from objects like electric sockets. She has also had these hallucinations in the dark. She had previously suffered from recurrent headaches, passage hallucinations (to see movements in the corner of the eyes) and zoopsia (she saw large ants crawling over her hands). An MRI of the brain showed minor white-matter abnormalities near the lentiform nucleus and in the semioval center. The visual perceptions she had experienced were attributed to unusual electrophysiological activity in the regions of the brain that are specialised for face and colour in the ventral occipito-temporal cortex. [10]

A 44-year-old woman reported to have begun seeing facial distortions. [4] She perceived that people's faces would change to look more like caricatures of them and her perception worsened over time. She had a history of epilepsy in childhood and had suffered a concussion several years before having this condition, though no medical evidence of seizure was found during distortions. She reported that occasionally she would experience a pixelated vision, like television static and mentioned that these symptoms occurred several times a week with each event lasting from a few minutes to a few hours.

Related Research Articles

<span class="mw-page-title-main">Hallucination</span> Perception that only seems real

A hallucination is a perception in the absence of an external stimulus that has the compelling sense of reality. Hallucinations are vivid, substantial, and are perceived to be located in external objective space. Hallucination is a combination of two conscious states of brain wakefulness and REM sleep. They are distinguishable from several related phenomena, such as dreaming, which does not involve wakefulness; pseudohallucination, which does not mimic real perception, and is accurately perceived as unreal; illusion, which involves distorted or misinterpreted real perception; and mental imagery, which does not mimic real perception, and is under voluntary control. Hallucinations also differ from "delusional perceptions", in which a correctly sensed and interpreted stimulus is given some additional significance.

<span class="mw-page-title-main">Micropsia</span> Medical condition

Micropsia is a condition affecting human visual perception in which objects are perceived to be smaller than they actually are. Micropsia can be caused by optical factors, by distortion of images in the eye, by changes in the brain, and from psychological factors. Dissociative phenomena are linked with micropsia, which may be the result of brain-lateralization disturbance.

<span class="mw-page-title-main">Alice in Wonderland syndrome</span> Neurological disorder that distorts perception of objects size and distance

Alice in Wonderland syndrome (AIWS), also known as Todd's syndrome or dysmetropsia, is a neurological disorder that distorts perception. People with this syndrome may experience distortions in their visual perception of objects, such as appearing smaller (micropsia) or larger (macropsia), or appearing to be closer (pelopsia) or farther (teleopsia) than they are. Distortion may also occur for senses other than vision.

Macropsia is a neurological condition affecting human visual perception, in which objects within an affected section of the visual field appear larger than normal, causing the person to feel smaller than they actually are. Macropsia, along with its opposite condition, micropsia, can be categorized under dysmetropsia. Macropsia is related to other conditions dealing with visual perception, such as aniseikonia and Alice in Wonderland Syndrome. Macropsia has a wide range of causes, from prescription and illicit drugs, to migraines and (rarely) complex partial epilepsy, and to different retinal conditions, such as epiretinal membrane. Physiologically, retinal macropsia results from the compression of cones in the eye. It is the compression of receptor distribution that results in greater stimulation and thus a larger perceived image of an object.

The Fregoli delusion is a rare disorder in which a person holds a delusional belief that different people are in fact a single person who changes appearance or is in disguise. The syndrome may be related to a brain lesion and is often of a paranoid nature, with the delusional person believing themselves persecuted by the person they believe is in disguise.

<span class="mw-page-title-main">Temporal lobe</span> One of the four lobes of the mammalian brain

The temporal lobe is one of the four major lobes of the cerebral cortex in the brain of mammals. The temporal lobe is located beneath the lateral fissure on both cerebral hemispheres of the mammalian brain.

<span class="mw-page-title-main">Face perception</span> Cognitive process of visually interpreting the human face

Facial perception is an individual's understanding and interpretation of the face. Here, perception implies the presence of consciousness and hence excludes automated facial recognition systems. Although facial recognition is found in other species, this article focuses on facial perception in humans.

<span class="mw-page-title-main">Fusiform gyrus</span> Gyrus of the temporal and occipital lobes of the brain

The fusiform gyrus, also known as the lateral occipitotemporal gyrus,is part of the temporal lobe and occipital lobe in Brodmann area 37. The fusiform gyrus is located between the lingual gyrus and parahippocampal gyrus above, and the inferior temporal gyrus below. Though the functionality of the fusiform gyrus is not fully understood, it has been linked with various neural pathways related to recognition. Additionally, it has been linked to various neurological phenomena such as synesthesia, dyslexia, and prosopagnosia.

<span class="mw-page-title-main">Bálint's syndrome</span> Medical condition

Bálint's syndrome is an uncommon and incompletely understood triad of severe neuropsychological impairments: inability to perceive the visual field as a whole (simultanagnosia), difficulty in fixating the eyes, and inability to move the hand to a specific object by using vision. It was named in 1909 for the Austro-Hungarian neurologist and psychiatrist Rezső Bálint who first identified it.

<span class="mw-page-title-main">Aura (symptom)</span> Symptom of epilepsy and migraine

An aura is a perceptual disturbance experienced by some with epilepsy or migraine. An epileptic aura is actually a minor seizure.

Focal seizures are seizures that affect initially only one hemisphere of the brain. The brain is divided into two hemispheres, each consisting of four lobes – the frontal, temporal, parietal and occipital lobes. A focal seizure is generated in and affects just one part of the brain – a whole hemisphere or part of a lobe. Symptoms will vary according to where the seizure occurs. When seizures occur in the frontal lobe, the patient may experience a wave-like sensation in the head. When seizures occur in the temporal lobe, a feeling of déjà vu may be experienced. When seizures are localized to the parietal lobe, a numbness or tingling may occur. With seizures occurring in the occipital lobe, visual disturbances or hallucinations have been reported.

Visual agnosia is an impairment in recognition of visually presented objects. It is not due to a deficit in vision, language, memory, or intellect. While cortical blindness results from lesions to primary visual cortex, visual agnosia is often due to damage to more anterior cortex such as the posterior occipital and/or temporal lobe(s) in the brain.[2] There are two types of visual agnosia: apperceptive agnosia and associative agnosia.

<span class="mw-page-title-main">Posterior cerebral artery</span> Artery which supplies blood to the occipital lobe of the brain

The posterior cerebral artery (PCA) is one of a pair of cerebral arteries that supply oxygenated blood to the occipital lobe, part of the back of the human brain. The two arteries originate from the distal end of the basilar artery, where it bifurcates into the left and right posterior cerebral arteries. These anastomose with the middle cerebral arteries and internal carotid arteries via the posterior communicating arteries.

Akinetopsia, also known as cerebral akinetopsia or motion blindness, is a term introduced by Semir Zeki to describe an extremely rare neuropsychological disorder, having only been documented in a handful of medical cases, in which a patient cannot perceive motion in their visual field, despite being able to see stationary objects without issue. The syndrome is the result of damage to visual area V5, whose cells are specialized to detect directional visual motion. There are varying degrees of akinetopsia: from seeing motion as frames of a cinema reel to an inability to discriminate any motion. There is currently no effective treatment or cure for akinetopsia.

Phantosmia, also called an olfactory hallucination or a phantom odor, is smelling an odor that is not actually there. This is intrinsically suspicious as the formal evaluation and detection of relatively low levels of odour particles is itself a very tricky task in air epistemology. It can occur in one nostril or both. Unpleasant phantosmia, cacosmia, is more common and is often described as smelling something that is burned, foul, spoiled, or rotten. Experiencing occasional phantom smells is normal and usually goes away on its own in time. When hallucinations of this type do not seem to go away or when they keep coming back, it can be very upsetting and can disrupt an individual's quality of life.

<span class="mw-page-title-main">Fusiform face area</span> Part of the human visual system that is specialized for facial recognition

The fusiform face area is a part of the human visual system that is specialized for facial recognition. It is located in the inferior temporal cortex (IT), in the fusiform gyrus.

Prosopamnesia is a selective neurological impairment in the ability to learn new faces. There is a special neural circuit for the processing of faces as opposed to other non-face objects. Prosopamnesia is a deficit in the part of this circuit responsible for encoding perceptions as memories.

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) is a pure but rare form of idiopathic occipital epilepsy that affects otherwise normal children and adolescents. It is classified amongst benign idiopathic childhood focal epilepsies such as rolandic epilepsy and Panayiotopoulos syndrome.

<span class="mw-page-title-main">Occipital epilepsy</span> Medical condition

Occipital epilepsy is a neurological disorder that arises from excessive neural activity in the occipital lobe of the brain that may or may not be symptomatic. Occipital lobe epilepsy is fairly rare, and may sometimes be misdiagnosed as migraine when symptomatic. Epileptic seizures are the result of synchronized neural activity that is excessive, and may stem from a failure of inhibitory neurons to regulate properly.

The occipital face area (OFA) is a region of the human cerebral cortex which is specialised for face perception. The OFA is located on the lateral surface of the occipital lobe adjacent to the inferior occipital gyrus. The OFA comprises a network of brain regions including the fusiform face area (FFA) and posterior superior temporal sulcus (STS) which support facial processing.

References

  1. ↑ Talia Naquin (March 22, 2024). "Demon face syndrome pictures". Fox 8 . Retrieved April 25, 2024.
  2. ↑ Sarah Al-Arshani (March 28, 2024). "What causes prosopometamorphopsia, the rare 'demon-face syndrome' PMO". USA Today . Retrieved April 25, 2024.
  3. ↑ Duchaine, Brad. "Understanding Prosopometamorphopsia (PMO)".
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