Made by DATEXIS (Data Science and Text-based Information Systems) at Beuth University of Applied Sciences Berlin
Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
Funded by The Federal Ministry for Economic Affairs and Energy; Grant: 01MD19013D, Smart-MD Project, Digital Technologies
Proposed diagnostic criteria for the "visual snow" syndrome:
- Dynamic, continuous, tiny dots in the entire visual field.
- At least one additional symptom:
- Palinopsia (visual trailing and afterimages)
- Enhanced entoptic phenomena (floaters, photopsia, blue field entoptic phenomenon, self-light of the eye)
- Photophobia
- Tinnitus
- Impaired night vision
- Symptoms are not consistent with typical migraine aura.
- Symptoms are not attributed to another disorder (ophthalmological, drug abuse).
Migraine and migraine with aura are common comorbidities. However, comorbid migraine worsens some of the additional visual symptoms and tinnitus seen in "visual snow" syndrome. This might bias research studies by patients with migraine being more likely to offer study participation than those without migraine due to having more severe symptoms. In contrast to migraine, comorbidity of typical migraine aura does not appear to worsen symptoms.
Patients with visual "snow" have normal equivalent input noise levels: Visual snow is a poorly understood symptom. Patients report seeing "snow", much like the visual noise on a TV screen after transmission ends. Some hypothesize that what the patients see as "snow" is their own intrinsic visual noise. Dennis Pelli and others' measurements assess whether visual-snow patients have increased levels of intrinsic visual noise.
Of all of the visual distortions, micropsia has the largest variety of causes.
Micropsia can occur during the aura phase of a migraine attack, a phase that often precedes the onset of a headache and is commonly characterized by visual disturbances. Micropsia, along with hemianopsia, quadrantopsia, scotoma, phosphene, teicopsia, metamorphopsia, macropsia, teleopsia, diplopia, dischromatopsia, and hallucination disturbances, is a type of aura that occurs immediately before or during the onset of a migraine headache. The symptom usually occurs less than thirty minutes before the migraine headache begins and lasts for five to twenty minutes. Only 10-20% of children with migraine headaches experience auras. Visual auras such as micropsia are most common in children with migraines.
There are a few cases of palinopsia with many of the same features as hallucinatory palinopsia (formed image perseveration) but with some important differences. The formed perseverated image may only last a couple seconds or may be black or translucent. These variants usually lack the realistic clarity of hallucinatory palinopsia, and the generation of the palinoptic images is affected by fixation time, motion, stimulus intensity, or contrast. These variants probably represent an overlap in hallucinatory and illusory palinopsia but are included in illusory palinopsia since they often co-exist with the other illusory symptoms.
Visual trailing describes an object in motion leaving frozen copies in its wake. These motion-induced afterimages may be discontinuous such as in a film reel or may be blurred together such as in a long-exposure photograph. If discontinuous, the patient also usually reports akinetopsia. The perseverated images last a few seconds and are usually identical in color and shape to the original stimulus. Most cases describe visual trails during movement of an object, although there are also reports from the movement of the observer's head or eyes.
Palinopsia (Greek: "palin" for "again" and "opsia" for "seeing") 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.
In 2014, Gersztenkorn and Lee comprehensively reviewed all cases of palinopsia in the literature and subdivided it into two clinically relevant groups: illusory palinopsia and hallucinatory palinopsia. Hallucinatory palinopsia, usually due to seizures or posterior cortical lesions, describes afterimages that are formed, long-lasting, and high resolution. Illusory palinopsia, usually due to migraines, head trauma, prescription drugs, or hallucinogen persisting perception disorder (HPPD), describes afterimages that are affected by ambient light and motion and are unformed, indistinct, or low resolution.
Retinal migraine is associated with transient monocular visual loss (scotoma) in one eye lasting less than one hour. During some episodes, the visual loss may occur with no headache and at other times throbbing headache on the same side of the head as the visual loss may occur, accompanied by severe light sensitivity and/or nausea. Visual loss tends to affect the entire monocular visual field of one eye, not both eyes. After each episode, normal vision returns.
It may be difficult to read and dangerous to drive a vehicle while retinal migraine symptoms are present.
Retinal migraine is a different disease than scintillating scotoma, which is a visual anomaly caused by spreading depression in the occipital cortex at the back of the brain, not in the eyes nor any component thereof. Unlike in retinal migraine, a scintillating scotoma involves repeated bouts of temporary diminished vision or blindness and affects vision from both eyes, upon which sufferers may see flashes of light, zigzagging patterns, blind spots, or shimmering spots or stars.
Quadrantanopia, quadrantanopsia, or quadrant anopia refers to an anopia affecting a quarter of the field of vision.
It can be associated with a lesion of an optic radiation. While quadrantanopia can be caused by lesions in the temporal and parietal lobes, it is most commonly associated with lesions in the occipital lobe.
Palinopsia is a pathological symptom and should be distinguished from physiological afterimages, a common and benign phenomenon. Physiological afterimages appear when viewing a bright stimulus and shifting visual focus. For example, after staring at a computer screen and looking away, a vague afterimage of the screen remains in the visual field. A stimulus consistently produces the same afterimage, which is dependent on the stimulus intensity and contrast, the time of fixation, and the retinal adaptation state. Physiological afterimages are usually the complementary color of the original stimulus (negative afterimage), while palinoptic afterimages are usually the same color as the original stimulus (positive afterimage). There is some ambiguity between illusory palinopsia and physiological afterimages since there are not concrete symptomatic criteria which determines if an afterimage is pathological.
Retinal migraine (also known as ophthalmic migraine, and ocular migraine) is a retinal disease often accompanied by migraine headache and typically affects only one eye. It is caused by ischaemia or vascular spasm in or behind the affected eye.
The terms "retinal migraine" and "ocular migraine" are often confused with "visual migraine", which is a far-more-common symptom of vision loss, resulting from the aura phase of the common migraine. The aura phase of migraine can occur with or without a headache. Ocular or retinal migraines happen in the eye, so only affect the vision in that eye, while visual migraines occur in the brain, so affect the vision in both eyes together. Visual migraines result from cortical spreading depression and are also commonly termed scintillating scotoma.
Common causes of scotomata include demyelinating disease such as multiple sclerosis (retrobulbar neuritis), damage to nerve fiber layer in the retina (seen as cotton wool spots) due to hypertension, toxic substances such as methyl alcohol, ethambutol and quinine, nutritional deficiencies, vascular blockages either in the retina or in the optic nerve, stroke or other brain injury, and macular degeneration, often associated with aging. Scintillating scotoma is a common visual aura in migraine. Less common, but important because they are sometimes reversible or curable by surgery, are scotomata due to tumors such as those arising from the pituitary gland, which may compress the optic nerve or interfere with its blood supply.
Rarely, scotomata are . One important variety of bilateral scotoma may occur when a pituitary tumour begins to compress the optic chiasm (as distinct from a single optic nerve) and produces a bitemporal paracentral scotoma, and later, when the tumor enlarges, the scotomas extend out to the periphery to cause the characteristic bitemporal hemianopsia. This type of visual-field defect tends to be obvious to the person experiencing it but often evades early objective diagnosis, as it is more difficult to detect by cursory clinical examination than the classical or textbook bitemporal peripheral hemianopia and may even elude sophisticated electronic modes of visual-field assessment.
In a pregnant woman, scotomata can present as a symptom of severe preeclampsia, a form of pregnancy-induced hypertension. Similarly, scotomata may develop as a result of the increased intracranial pressure that occurs in malignant hypertension.
The scotoma is also caused by the aminoglycoside antibiotics mainly by Streptomycin.
Homonymous denotes a condition which affects the same portion of the visual field of each eye.
Homonymous inferior quadrantanopia is a loss of vision in the same lower quadrant of visual field in both eyes whereas a homonymous superior quadrantanopia is a loss of vision in the same upper quadrant of visual field in both eyes.
A lesion affecting one side of the temporal lobe may cause damage to the inferior optic radiations (known as the temporal pathway or Meyer's loop) which can lead to superior quadrantanopia on the contralateral side of both eyes (colloquially referred to as "pie in the sky"); if the superior optic radiations (parietal pathway) are lesioned, the visual loss occurs on the inferior contralateral side of both eyes and is referred to as an inferior quadrantanopia.
Symptom-producing, or pathological, scotomata may be due to a wide range of disease processes, affecting any part of the visual system, including the retina (in particular its most sensitive portion, the macula), the optic nerve and even the visual cortex. A pathological scotoma may involve any part of the visual field and may be of any shape or size. A scotoma may include and enlarge the normal blind spot. Even a small scotoma that happens to affect central or macular vision will produce a severe visual disability, whereas a large scotoma in the more peripheral part of a visual field may go unnoticed by the bearer because of the normal reduced optical resolution in the peripheral visual field.
Closed-eye hallucinations and closed-eye visualizations (CEV) are a distinct class of hallucination. These types of hallucinations generally only occur when one's eyes are closed or when one is in a darkened room. They can be a form of phosphene. Some people report closed-eye hallucinations under the influence of psychedelics. These are reportedly of a different nature than the "open-eye" hallucinations of the same compounds.
Photophobia is a symptom of abnormal intolerance to visual perception of light. As a medical symptom, photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of actual physical sensitivity of the eyes, though the term is sometimes additionally applied to abnormal or irrational fear of light such as heliophobia. The term "photophobia" comes from the Greek φῶς ("phōs"), meaning "light", and φόβος ("phóbos"), meaning "fear". Photophobia is a common symptom of visual snow.
Neurological causes for photophobia include:
- Autism spectrum disorders
- Chiari malformation
- Occipital Neuralgia
- Dyslexia
- Encephalitis including Myalgic encephalomyelitis aka Chronic fatigue syndrome
- Meningitis
- Trigeminal disturbance causes central sensitization (hence, multiple other associated hypersensitivities. Causes can be bad bite, infected tooth, etc.
- Subarachnoid haemorrhage
- Tumor of the posterior cranial fossa
Image burn-in occurs when very bright objects lie in one's field of vision, and should not be confused with closed-eye hallucinations. Visual burn-in from bright lights is visible for a few minutes after closing the eyes, or by blinking repeatedly, but the burn-in effect slowly fades away as the retina recovers, whereas the waking-consciousness CEV noise will not disappear if observed continuously over a period of time.
In binasal hemianopsia, vision is missing in the inner (nasal or medial) half of both the right and left visual fields. Information from the nasal visual field falls on the temporal (lateral) retina. Those lateral retinal nerve fibers do not cross in the optic chiasm. Calcification of the internal carotid arteries can impinge the uncrossed, lateral retinal fibers leading to loss of vision in the nasal field.
Note: Clinical testing of visual fields (by confrontation) can produce a false positive result (particularly in inferior nasal quadrants).
Inconspicuous akinetopsia is often described by seeing motion as a cinema reel or a multiple exposure photograph. This is the most common kind of akinetopsia and many patients consider the stroboscopic vision as a nuisance. The akinetopsia often occurs with visual trailing (palinopsia), with afterimages being left at each frame of the motion. It is caused by prescription drugs, hallucinogen persisting perception disorder (HPPD), and persistent aura without infarction. The pathophysiology of akinetopsia palinopsia is not known, but it has been hypothesized to be due to inappropriate activation of physiological motion suppression mechanisms which are normally used to maintain visual stability during eye movements (e.g. saccadic suppression).
When the pathology involves both eyes, it is either homonymous or Heteronymous.
Hallucinatory palinopsia consists of the following four symptom categories. A person often reports symptoms from multiple categories.
Streff syndrome is a vision condition primarily exhibited by children under periods of visual or emotional stress.
Frequently patients will have reduced stereopsis, large accommodative lag on dynamic retinoscopy, and a reduced visual field (tubular or spiral field). Streff Syndrome was first described in 1962 by an optometrist, Dr. John Streff as Non-malingering syndrome. In 1962, Dr. Streff and Dr. Richard Apell expanded the concept to add early adaptive syndrome as a precursor to Streff syndrome. Dr. Streff believed the visual changes were induced by stress from reading. There is dispute on the taxonomy of functional vision defects. Some research indicates that Streff syndrome may be caused by a dysfunction in the magnocellular pathway of the retinal ganglion cells. These cells are only 10% of the retinal nerve cells and register motion detection.
Early Adaptive Syndrome
A homonymous hemianopsia is the loss of half of the visual field on the same side in both eyes. The visual images that we see to the right side travel from both eyes to the left side of the brain, while the visual images we see to the left side in each eye travel to the right side of the brain. Therefore, damage to the right side of the posterior portion of the brain or right optic tract can cause a loss of the left field of view in both eyes. Likewise, damage to the left posterior brain or left optic radiation can cause a loss of the right field of vision.
Symptoms of CVI usually include several (but not necessarily all) of the following:
- The person with CVI exhibits variable vision. Visual ability can change from one day to the next but it can also fluctuate from minute to minute, especially when the person is tired. When undertaking critical activities, people with CVI should be prepared for their vision to fluctuate, by taking precautions such as always carrying a white cane even if they don't always use it to the full, or always having very large print available, just in case it's needed. (For example, consider the consequences of losing vision while giving a public speech). Managing fatigue can reduce fluctuations but does not eliminate them. Changes in environment, even minor, are mostly responsible for what appears to be variable vision.
- One eye may perform significantly worse than the other, and depth perception can be very limited (although not necessarily zero).
- The field of view may be severely limited. The best vision might be in the centre (like tunnel vision) but more often it is at some other point, and it is difficult to tell what the person is really looking at. Note that if the person also has a common ocular visual impairment such as nystagmus then this can also affect which part(s) of the visual field are best. (Sometimes there exists a certain gaze direction which minimises the nystagmus, called a "null point.")
- Even though the field of view may be very narrow indeed, it is often possible for the person to detect and track movement. Movement is handled by the 'V5' part of the visual cortex, which may have escaped the damage. Sometimes a moving object can be seen better than a stationary one; at other times the person can sense movement but cannot identify what is moving. (This can be annoying if the movement is prolonged, and to escape the annoyance the person may have to either gaze right at the movement or else obscure it.) Sometimes it is possible for a person with CVI to see things while moving their gaze around that they didn't detect when stationary. However, movement that is too fast can be hard to track; some people find that fast-moving objects "disappear." Materials with reflective properties, which can simulate movement, may be easier for a person with CVI to see. However, too many reflections can be confusing (see cognitive overload).
- Some objects may be easier to see than others. For example, the person may have difficulty recognising faces or facial expressions but have fewer problems with written materials. This is presumably due to the different way that the brain processes different things.
- Colour and contrast are important. The brain's colour processing is distributed in such a way that it is more difficult to damage, so people with CVI usually retain full perception of colour. This can be used to advantage by colour-coding objects that might be hard to identify otherwise. Sometimes yellow and red objects are easier to see, as long as this does not result in poor contrast between the object and the background.
- People with CVI strongly prefer a simplified view. When dealing with text, for example, the person might prefer to see only a small amount of it at once. People with CVI frequently hold text close to their eyes, both to make the text appear larger and to minimise the amount they must look at. This also ensures that important things such as letters are not completely hidden behind any scotomas (small defects in parts of the functioning visual field), and reduces the chances of getting lost in the text. However, the simplification of the view should not be done in such a way that it requires too rapid a movement to navigate around a large document, since too much motion can cause other problems (see above).
- In viewing an array of objects, people with CVI can more easily see them if they only have to look at one or two at a time. People with CVI also see familiar objects more easily than new ones. Placing objects against a plain background also makes them easier for the person with CVI to see.
- For the same reason (simplified view), the person may also dislike crowded rooms and other situations where their functioning is dependent on making sense of a lot of visual 'clutter'.
- Visual processing can take a lot of effort. Often the person has to make a conscious choice about how to divide mental effort between making sense of visual data and performing other tasks. For some people, maintaining eye contact is difficult, which can create problems in Western culture (for example, bonding can be difficult for some parents who have an infant with CVI, and lack of contact in an older child can cause others to regard him or her with suspicion).
- It can also be difficult for some people with CVI to look at an object and reach for it at the same time. Looking and reaching are sometimes accomplished as two separate acts: look, then look away and reach.
- People with CVI can sometimes benefit from a form of blindsight, which manifests itself as a kind of awareness of one's surroundings that cannot consciously be explained (for example, the person correctly guesses what they should do in order to avoid an obstacle but does not actually see that obstacle). However, this cannot be relied on to work all the time. In contrast, some people with CVI exhibit spatial difficulties and may have trouble moving about in their environment.
- Approximately one third of people with CVI have some photophobia. It can take longer than usual to adjust to large changes in light level, and flash photography can be painful. On the other hand, CVI can also in some cases cause a desire to gaze compulsively at light sources, including such things as candle flames and fluorescent overhead lights. The use of good task lighting (especially low-temperature lamps which can be placed at very close range) is often beneficial.
- Although people (with or without CVI) generally assume that they see things as they really are, in reality the brain may be doing a certain amount of guessing and "filling in", which is why people sometimes think they see things that turn out on closer inspection not to be what they seemed. This can occur more frequently when a person has CVI. Hence, a person with CVI can look at an optical illusion or abstract picture and perceive something that is significantly different from what a person without CVI will perceive.
The presence of CVI does not necessarily mean that the person's brain is damaged in any other way, but it can often be accompanied by other neurological problems, the most common being epilepsy.