Hallucinatory palinopsia | |
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Specialty | Ophthalmology |
Hallucinatory palinopsia is a subtype of palinopsia, a visual disturbance defined as the persistent or recurrence of a visual image after the stimulus has been removed. [1] Palinopsia is a broad term describing a group of symptoms which is divided into hallucinatory palinopsia and illusory palinopsia. [2] Hallucinatory palinopsia refers to the projection of an already-encoded visual memory and is similar to a complex visual hallucination: the creation of a formed visual image where none exists.
Hallucinatory palinopsia usually arises from posterior cortical lesions or seizures and can be the presenting symptom of a serious neurological disease. Hallucinatory palinopsia describes afterimages or scenes that are formed, long-lasting, high resolution, and isochromatic. The palinoptic images are not typically reliant on environmental parameters and often present with homonymous visual field deficits. Hallucinatory palinopsia occurs unpredictably and the persistent images can appear anywhere in the visual field, regardless of the location of the original stimulus. A patient will often have only a few episodes of hallucinatory palinopsia. Visual perseveration is synonymous with palinopsia. The term is from Greek: palin for "again" and opsia for "seeing".
Formed image perseveration refers to a single, stationary object that remains fixed in one's visual field. These pathological afterimages look realistic and have the same color and clarity as the original stimulus. The palinopsia lasts at least 15 seconds, but may persist for hours or days. For example, a patient sees a cat, and an identical copy of the cat remains fixed in the field of view for 30 minutes. [3] A patient commonly complains of the perseverated fingers of an examiner. These afterimages often occur in visual field deficits but may occur anywhere in the visual field, regardless of the location of the original stimulus. The generation of the afterimages is not affected by external conditions such as the length of fixation, brightness of the stimulus, contrast, or motion. The palinoptic image can appear immediately after seeing the original image or may be delayed in time.
Scene perseveration refers to seeing a previously-viewed, short stereotyped scene that continuously replays for several minutes. For example, a patient might view a person throwing a ball, and then an hour later, perceives the same action sequence repeated many times. [4] The palinoptic scene usually has the same color and clarity as the original. Our understanding of visual memory considers a short scene as a unit of memory, similar to an image, [5] thus scene perseveration is probably mechanistically related to formed image perseveration.
Categorical incorporation refers to seeing an object or feature and superimposing it onto comparable objects or people. For example, after seeing a person wearing a hat, that same hat is seen on each subsequent person seen. Or a person sees the spire of a building and then incorporates it to the top of other structures seen. [6] The palinoptic images have the same characteristics as the original stimulus, and episodes of categorical incorporation usually last a few minutes. Categorical incorporation highlights the brain's use of constructs to process stimuli.
Patterned visual spread describes the spread of a pattern in a field of view. For example, a patient sees a checkered pattern on a lamp, which then contiguously spreads to objects such as the floor or a desk. Contextual clues do not influence patterned visual spread, distinguishing it from categorical incorporation.
Of the published cases of palinopsia from posterior cortical lesions or seizures, 93% described hallucinatory palinopsia. [2] Hallucinatory palinopsia may be caused by many types of posterior cortical lesions such as neoplasms, [7] infarctions, [8] hemorrhages, [9] arteriovenous malformations, [3] aneurysm, [1] abscesses, [10] and tuberculomas. [11] Hallucinatory palinopsia from seizures may be secondary to a focal cortical lesion or may be secondary to a non-structural disturbance. Causes of seizures that are reported to cause palinopsia include metabolic disturbances (hyperglycemia, [12] carnitine deficiency [13] ), ion channel disturbances, [14] Creutzfeldt–Jakob disease, [15] and seizures of unknown cause. [16]
Hallucinatory palinopsia is a dysfunction of visual memory, caused by localized cortical hyperexcitability or hyperactivity in the posterior visual pathway. Post-geniculate cortical lesions or seizures may cause cortical deafferentation, focal cortical irritation, and epileptic discharges, the proposed mechanisms of hallucinatory palinopsia.
Palinopsia can occur from posterior visual pathway (post-geniculate) deafferentation, which causes homonymous visual field deficits. This mechanism is thought to be similar to the deafferentation hyperexcitability seen in visual release hallucinations (Charles Bonnet syndrome), [17] which are distinguished from palinopsia by whether the formed image or scene previously occurred. It is hypothesized that deafferentation hyperexcitability is the cause of neuropathic pain. Molecular changes from deafferentation include an increase in presynaptic neurotransmitter vesicles and heightened post-synaptic receptor sensitivity. [18]
Palinopsia from epileptic discharges, confirmed by electroencephalogram, have been reported during a seizure aura, during the seizure, or post-ictally. [4] The seizures can originate anywhere in the posterior visual pathway, depending on the location of the pathology. Palinoptic seizures rarely generalize, and there are case reports of palinopsia as the only symptom of a visual seizure. [4] If seizures are persistent, then continual palinoptic episodes often occur.
Palinopsia, which occurs after neurosurgical procedures or cerebrovascular accidents, has been partly attributed to focal cortical irritation. [17] [19] Symptoms are associated with perilesional hyperperfusion, which may reflect focal cortical instability and subsequent hyperactivity. [9]
While there are reports of palinopsia from each individual mechanism, there is usually a combination of the aforementioned mechanisms. For example, hallucinatory palinopsia may present in a patient with seizure symptoms and visual field deficits, after a neurosurgical procedure. [20] This suggests that the cortical hyperactivity from each mechanism is additive. "Hallucinatory palinopsia, once considered a disorder of the nondominant parieto-occipital lobe, has since been shown to occur from lesions in the dominant or non-dominant temporal, parietal, or occipital lobes. … The predominance of lesions in certain cortical areas is more likely due to the uneven distribution or functional variation of visual cortex-hippocampal neurons. ... All of the hallucinatory palinopsia symptoms occur concomitantly in a patient with one lesion, which supports current evidence that objects, features, and scenes are all units of visual memory, perhaps at different levels of processing. This alludes to neuroanatomical integration in visual memory creation and storage." [2]
Palinopsia necessitates a full ophthalmologic and neurologic history and physical exam. Hallucinatory palinopsia warrants automated visual field testing and neuroimaging since the majority of hallucinatory palinopsia is caused by posterior cortical lesions and seizures. It is generally easy to diagnose the underlying cause of hallucinatory palinopsia. The medical history typically includes concerning symptoms, and neuroimaging usually reveals cortical lesions. In patients with hallucinatory palinopsia and unremarkable neuroimaging, blood tests or clinical history often hints at the cause. The practitioner should be considering visual seizures in these cases.
Palinopsia from cerebrovascular accidents generally resolves spontaneously, and treatment should be focused on the vasculopathic risk factors. Palinopsia from neoplasms, AVMs, or abscesses require treatment of the underlying condition, which usually also resolves the palinopsia. Palinopsia due to seizures generally resolves after correcting the primary disturbance and/or treating the seizures. In persistent hallucinatory palinopsia, a trial of an anti-epileptic drug can be attempted. Anti-epileptics reduce cortical excitability and could potentially treat palinopsia caused by cortical deafferentation or cortical irritation. Patients with idiopathic hallucinatory palinopsia should have close follow-up. [2]
A hallucination is a perception in the absence of an external stimulus that has the qualities of a real perception. 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. Many hallucinations happen also during sleep paralysis.
The occipital lobe is one of the four major lobes of the cerebral cortex in the brain of mammals. The name derives from its position at the back of the head, from the Latin ob, 'behind', and caput, 'head'.
Visual snow syndrome (VSS) is an uncommon neurological condition in which the primary symptom is that affected individuals see persistent flickering white, black, transparent, or coloured dots across the whole visual field. Other common symptoms are palinopsia, enhanced entoptic phenomena, photophobia, and tension headaches. The condition is typically always present and has no known cure, as viable treatments are still under research. Astigmatism, although not presumed connected to these visual disturbances, is a common comorbidity. As well, migraine and tinnitus are common comorbidities which are both associated with a more severe presentation of the syndrome. TMJ may also be a common comorbidity.
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.
Visual memory describes the relationship between perceptual processing and the encoding, storage and retrieval of the resulting neural representations. Visual memory occurs over a broad time range spanning from eye movements to years in order to visually navigate to a previously visited location. Visual memory is a form of memory which preserves some characteristics of our senses pertaining to visual experience. We are able to place in memory visual information which resembles objects, places, animals or people in a mental image. The experience of visual memory is also referred to as the mind's eye through which we can retrieve from our memory a mental image of original objects, places, animals or people. Visual memory is one of several cognitive systems, which are all interconnected parts that combine to form the human memory. Types of palinopsia, the persistence or recurrence of a visual image after the stimulus has been removed, is a dysfunction of visual memory.
Palinopsia is the persistent recurrence of a visual image after the stimulus has been removed. Palinopsia is not a diagnosis; it is a diverse group of pathological visual symptoms with a wide variety of causes. Visual perseveration is synonymous with palinopsia.
An aura is a perceptual disturbance experienced by some with epilepsy or migraine. An epileptic aura is a seizure.
Cortical blindness is the total or partial loss of vision in a normal-appearing eye caused by damage to the brain's occipital cortex. Cortical blindness can be acquired or congenital, and may also be transient in certain instances. Acquired cortical blindness is most often caused by loss of blood flow to the occipital cortex from either unilateral or bilateral posterior cerebral artery blockage and by cardiac surgery. In most cases, the complete loss of vision is not permanent and the patient may recover some of their vision. Congenital cortical blindness is most often caused by perinatal ischemic stroke, encephalitis, and meningitis. Rarely, a patient with acquired cortical blindness may have little or no insight that they have lost vision, a phenomenon known as Anton–Babinski syndrome.
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 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.
Cerebral achromatopsia is a type of color-blindness caused by damage to the cerebral cortex of the brain, rather than abnormalities in the cells of the eye's retina. It is often confused with congenital achromatopsia but underlying physiological deficits of the disorders are completely distinct. A similar, but distinct, deficit called color agnosia exists in which a person has intact color perception but has deficits in color recognition, such as knowing which color they are looking at.
Foix–Chavany–Marie Syndrome (FCMS), also known as bilateral opercular syndrome, is a neuropathological disorder characterized by paralysis of the facial, tongue, pharynx, and masticatory muscles of the mouth that aid in chewing. The disorder is primarily caused by thrombotic and embolic strokes, which cause a deficiency of oxygen in the brain. As a result, bilateral lesions may form in the junctions between the frontal lobe and temporal lobe, the parietal lobe and cortical lobe, or the subcortical region of the brain. FCMS may also arise from defects existing at birth that may be inherited or nonhereditary. Symptoms of FCMS can be present in a person of any age and it is diagnosed using automatic-voluntary dissociation assessment, psycholinguistic testing, neuropsychological testing, and brain scanning. Treatment for FCMS depends on the onset, as well as on the severity of symptoms, and it involves a multidisciplinary approach.
Posterior cortical atrophy (PCA), also called Benson's syndrome, is a rare form of dementia which is considered a visual variant or an atypical variant of Alzheimer's disease (AD). The disease causes atrophy of the posterior part of the cerebral cortex, resulting in the progressive disruption of complex visual processing. PCA was first described by D. Frank Benson in 1988.
Lesions in the visual pathway affect vision most often by creating deficits or negative phenomena, such as blindness, visual field deficits or scotomas, decreased visual acuity and color blindness. On occasion, they may also create false visual images, called positive visual phenomena. These images can be a result of distortion of incoming sensory information leading to an incorrect perception of a real image called an illusion. When the visual system produces images which are not based on sensory input, they can be referred to as hallucinations. The visual phenomena may last from brief moments to several hours, but they also can be permanent. They are generally associated with other symptoms but occasionally are isolated. Conditions causing these phenomena include disruptions in the visual input along the pathways lesions in the extracortical visual system, migraines, seizures, toxic-metabolic encephalopathy, psychiatric conditions and sleep apnea, among others. The mechanisms underlying positive visual phenomena are not yet well understood. Possible mechanisms may be: 1) defect in the sensory input causing compensatory upregulation of the visual cortex, 2) faulty visual processing in which inputs are normal but lesions result in an inappropriate pattern of cortical excitation, 3)variants of normal visual processing. Of all forms of hallucination, visual hallucinations are the least likely to be associated with psychiatric disorders. For example most patients with visual hallucinations do not have schizophrenia and most patients with schizophrenia do not have visual hallucinations.
Tuber cinereum hamartoma is a benign tumor in which a disorganized collection of neurons and glia accumulate at the tuber cinereum of the hypothalamus on the floor of the third ventricle. It is a congenital malformation, included on the spectrum of gray matter heterotopias. Formation occurs during embryogenesis, typically between days 33 and 41 of gestation. Size of the tumor varies from one to three centimeters in diameter, with the mean being closer to the low end of this range. It is estimated to occur at a frequency of one in one million individuals.
Ulegyria is a diagnosis used to describe a specific type of cortical scarring in the deep regions of the sulcus that leads to distortion of the gyri. Ulegyria is identified by its characteristic "mushroom-shaped" gyri, in which scarring causes shrinkage and atrophy in the deep sulcal regions while the surface gyri are spared. This condition is most often caused by hypoxic-ischemic brain injury in the perinatal period. The effects of ulegyria can range in severity, although it is most commonly associated with cerebral palsy, mental retardation and epilepsy. N.C. Bresler was the first to view ulegyria in 1899 and described this abnormal morphology in the brain as “mushroom-gyri." Although ulegyria was first identified in 1899, there is still limited information known or reported about the condition.
People with epilepsy may be classified into different syndromes based on specific clinical features. These features include the age at which seizures begin, the seizure types, and EEG findings, among others. Identifying an epilepsy syndrome is useful as it helps determine the underlying causes as well as deciding what anti-seizure medication should be tried. Epilepsy syndromes are more commonly diagnosed in infants and children. Some examples of epilepsy syndromes include benign rolandic epilepsy, childhood absence epilepsy and juvenile myoclonic epilepsy. Severe syndromes with diffuse brain dysfunction caused, at least partly, by some aspect of epilepsy, are also referred to as epileptic encephalopathies. These are associated with frequent seizures that are resistant to treatment and severe cognitive dysfunction, for instance Lennox-Gastaut syndrome and West syndrome.
Illusory palinopsia is a subtype of palinopsia, a visual disturbance defined as the persistence or recurrence of a visual image after the stimulus has been removed. Palinopsia is a broad term describing a heterogeneous group of symptoms, which is divided into hallucinatory palinopsia and illusory palinopsia. Illusory palinopsia is likely due to sustained awareness of a stimulus and is similar to a visual illusion: the distorted perception of a real external stimulus.
Cerebral diplopia or polyopia describes seeing two or more images arranged in ordered rows, columns, or diagonals after fixation on a stimulus. The polyopic images occur monocular bilaterally and binocularly, differentiating it from ocular diplopia or polyopia. The number of duplicated images can range from one to hundreds. Some patients report difficulty in distinguishing the replicated images from the real images, while others report that the false images differ in size, intensity, or color. Cerebral polyopia is sometimes confused with palinopsia, in which multiple images appear while watching an object. However, in cerebral polyopia, the duplicated images are of a stationary object which are perceived even after the object is removed from the visual field. Movement of the original object causes all of the duplicated images to move, or the polyopic images disappear during motion. In palinoptic polyopia, movement causes each polyopic image to leave an image in its wake, creating hundreds of persistent images (entomopia).
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 visual pathway consists of structures that carry visual information from the retina to the brain. Lesions in that pathway cause a variety of visual field defects. In the visual system of human eye, the visual information processed by retinal photoreceptor cells travel in the following way:
Retina→Optic nerve→Optic chiasma →Optic tract→Lateral geniculate body→Optic radiation→Primary visual cortex