Anomalies of Color Vision Flashcards
What is the prealence of color vision defect in males and females in the population?v
- Large random population surveys show prevalence of deficiency of:
- European Caucasians is about 8% in men, 0.4% in women
- Between 4% and 6.5% in men of Chinese and Japanese ethnicity
- The male: female prevalence ratio is markedly different in Europeans and Asians
- Inherited – majority
- Usually non-progressive
- Acquired – less prevalent
- Secondary to ocular disease
- Inherited – majority
Name the 3 basis on which color vision defect can be classified.
- Classification based on:
- Origin
- Type of color defects – clinical testing
- Classification based on Color Matching Test
How is color vision defect classified based on origin?
- 2 Principal Types of Color Defect:
- Inherited – congenital
- Acquired
How is color vision defect classified based in Clinical testing?
- Classification of color vision status based on the minimum number of primary colors used to match perceived colors
- Trichromatism – require 3 primaries to make a match
- Dichromatism – require 2 primaries to make a match
- Monochromatism – require 1 primary to make a match
What is protan dichromacy known as?
- Protanomalous (defective pigment) / Protanopia (missing completely) trichromacy
- Aka protanomaly
- Red/green color defective
What is Deutan anomalous trichromatic known as?
- Aka. Deuteranomaly
- Deuteranopia (missing pigment) / deuteranomalous (defective pigment) trichromacy
If the S cone is missing in an individual, what is the condition known as?
- Tritanopia – completely defective S cone
- Tritanomalous trichromacy – tritanomaly
- Defective but present
How is color vision defect classified based on Color Matching Test?
- Classification of color vision status is based on the minimum number of primary colors used to match perceived colors
- Trichromatism –
- Normal trichromacy
- Anomalous trichromacy – 3 photopigments are present but the absorption spectrum of ONE of these photopigments is displayed
- Protanomaly
- Deuteranomaly
- Tritanomaly
- Dichromatism
- Protanopia
- Deuteranopia
- Tritanopia
- Monochromatism
- Rod monochromacy
- Cone monochromacy
- Trichromatism –
In Deutranomaly, the M cone pigment are shifted toward which wavelength?
- Deuteranomaly – displacement right towards longer wavelengths
- Prontanomaly – displaces left towards shorter wavelengths
- The displacement of cone photopigments result in deficient color discrimination
If L cone pigments are missing completely, what is it called? What replaces the L cone pigment?
- Protanopia – the L cone is completely missing
- The missing photopigment is presumably replaced by a remaining photopigment
What are the characteristics that distinguish anomalous color vision defectives from an individual with normal color vision?
- In Anomalous trichromacy 3 photopigments are present
- But the absorption spectrum of ONE of these photopigments is displayed to an abnormal position
- Among the characteristics that distinguish Anomalous color vision from COLOR NORMALS are:
- Spectral sensitivity – chromatic & Luminance
- Wavelength discrimination
- Color confusion lines
- The perception of saturation
- The Chromatic System can be identified in human subjects by:
- Determining spectral sensitivity for large stimuli
- Flashed for long duration
- On a photopic white background
What is the peak spectral sensitivity of anomalous trichromatic? How is it different than normal? Why are the peaks displaced?
- Anomalous trichromacy: spectral sensitivity function has 3 peaks – 440, 520, 620
- Normal Trichromacy: 426, 530, and 557
- Anomalous trichromacy peaks are displaced due to interaction between the L and M cones
- Dichromacy – only 2 peaks
Why is there a greater displacement of peak luminosity function in Protanopia than deutranopia?
- Greater displacement in protanopia (than deuteranopia) suggests that L-cones play more of a role in generating normal V(l) function than do M-cones
How is the luminosity function dislocation manifest in Anaomalous trichromacy when compared to Dichromacy?
- Anomalous trichromacy – the luminosity functions in anomalous trichromacy manifest the same general dislocation as dichromatic functions, but less pronounced
Describe the wavelength discrimination ability of Protanopes, Deuteranopes & Tritanopes (compared to normals).
- For both protanopia and deuteranopia, there is relatively well-developed wavelength discrimination in the region of 490 nm (shorter wavelength)
- BUT at longer wavelengths – beyond approximately 545 nm – there is no ability do discriminate between stimuli on the basis of wavelength differences alone
- In tritanopia there is well-developed wavelength discrimination at longer wavelengths, but poor wavelength discrimination in the region of 495 nm
-
WAVELENGTH DISCRIMINATION =
- Color normal (normal trichromats) – have down to 1 nm discrimination capability near 490 and 590 nm
- Protanopes and deuteranopes only discriminate well between 450 and 540
- Tritanopes have a gap with no discrimination between 460 & 480
- A normal observer can distinguish approximately 150-200 variations in color
- The dichromatic (protanopic and deuteranopic) observers are able to distinguish perhaps 20-30 colors
What is color confusion lines? What is copunctal points?
- Color confusion lines – the limited ability of people with dichromacy to distinguish among colors can be illustrated by plotting their COLOR CONFUSION LINES on the CIE diagram
- Confusion lines originate from copunctal points
- All colors falling along a confusion line are indistinguishable
Describe the color confusion lines for Protanopes, Deuteranopes & Tritanopes.
- Deuteranopia and protanopia share a color confusion line that is tangential to the spectral locus from approximately 545 to 700 nm.
- the colors associated with these wavelengths (green, yellow, orange, and red) are confused with each other, hence the term red-green color anomaly
- in tritanopia, blue-violet and yellow are confused with each other, thus the term blue-yellow anomaly
Name the colors where Protanopes and Deuteranopes share color confusion.
- Green, yellow, orange, and red
Where does saturation of colors (white.neutral point) occur for normals, Protanopes, Deuteranopes, and Tritanopes?
- The perception of saturation –
- In normal trichromacy, 570 nm appears less saturated (more WHITISH) than do other wavelengths
- So for NORMALS it is hard to distinguish yellow from white
- For deuteranopia & protanopia –
- Its 498 nm (deuteranopia) and 492 nm (deuteranopia)
- These particular wavelengths appear WHITE – they are totally desaturated and are referred to as neutral points
- Wavelength discrimination is best in the region of the neutral point
- These particular wavelengths appear WHITE – they are totally desaturated and are referred to as neutral points
- Its 498 nm (deuteranopia) and 492 nm (deuteranopia)
- For tritanopia – the neutral point is approximately 569 nm
- In normal trichromacy, 570 nm appears less saturated (more WHITISH) than do other wavelengths
- Individuals with normal color vision and anomalous trichromacy DO NOT manifest neutral points
- Upon questioning, a person with dichromacy may report that certain green traffic lights appear WHITE (or whitish)
How are individuals with color vision defects able to assign accurate color labels?
- Color Labeling –
- Although individuals with red-green dichromacy are essentially monochromatic for wavelengths beyond approximately 545 nm, they can do surprisingly well at labelling colors, especially when other cues are present
- A person with protanopia or deuteranopia may, for instance, have no difficulty labeling an apple as red and a banana as yellow (color memory)
- This is an easy task because he has learned that others label apples as red and bananas as yellow
- This individual is likely to have more difficulty assigning labels to the colors forming a pattern on a shirt because he may not have witnessed other people naming the colors
- Although a person with dichromacy does not have a wavelength-based discrimination for long-wavelength stimuli, we should be careful when drawing conclusions regarding his perceptions of these stimuli (whether he uses brightness etc.) as we cannot be certain of what a person with dichromacy perceives
- In deuteranopia & protanopia, the spectrum is divided into blue and yellow regions separated by the neutral point wavelength, which is perceived as WHITE
- Wavelength discrimination is best in the region of the neutral point
- Yellow – the region of the spectrum where individuals with red-green anomalies have no wavelength-based discrimination
- For instance, a person with deuteranopia might say the entire region is yellow, while a person with protanopia could interpret the dimming at longer wavelengths as a reddening
- Note: the decrease in brightness at longer wavelengths that is experienced in protanopia
- In tritanopia – the neutral point separates green and red regions
- In this case, the shorter and longer wavelength regions of the spectrum are separated by the neutral point at approximately 569 nm
What is the prevalence of Protans, Deutans, and Tritans in a population?
- Inherited color defects are congenital, genetically inherited, and without other associated abnormality
- The majority of red-green anomalies are inherited, transmitted in an X-linked recessive fashion
- Consequentially, they are considerably more common in men than women, with prevalences of approximately 8.0% and 0.4% respectively
- Inherited tritan anomalies are extremely rare and transmitted in an autosomal dominant fashion
- Prevalence in males:
- Protanopia:5%
- Protanomaly 1%
- Deuteranopia: 1%
- Deuteranomaly: 1%
- Tritan defects 0.005%
What is the most and least prevalent type of color vision defect?
- Most: protanopia
- Least: tritan defects (tritanopia and tritanomaly)
What are the societal implication of color vision defects?
- These defects are not physically debilitating, but they can have a major impact on one’s life
- Some may not even be aware of their deficiency until a peer makes fun of their choice of color in art class, which they must learn to cope
- Many who have a color vision deficiency learn of it only after they FAIL a color vision test
- Some who are informed that they are so afflicted deny it
- Some color-defective individuals are defensive and understandably insolent when forced to deal with the consequences of their deficiency, which can deny them employment in certain occupations
- Certain occupations related to public safety may have color vision standards that exclude individuals with anomalous color vision
- Airline pilot, firefighter, US custom & border protection, USDA meat inspector (ishihara)
- For other professions, NORMAL COLOR DISCRIMINATION can be an advantage:
- Chemical or electrical engineering, pharmacy, optometry, ophthalmology, dentistry, surgery, videographer
- Individuals with red-green color anomalies may have difficulty distinguishing among colors that are dark (e.g., socks) or desaturated (pastels) if these colors fall along the red-green confusion lines
- Their ability to discriminate and name colors is improved by increasing the level of illumination
- Unfortunate that many individuals learn of their color deficiency when they take a color vision test as part of a physical examination for employment and then are disqualified for the position after years of planning
- Eye care practitioners should ensure that color testing is done at a yong age for the purpose of providing good baseline data
- Parents and teachers should be informed
- Parents counselled about child’s career choices
Do sunglasses interfere with the ability of patients with inherited anomalies to quickly and correctly identify colored traffic signals?
- YES – certain non-neutral tints (i.e., colors other than GRAY) make it more difficult for these individuals to detect and recognize TRAFFIC LIGHTS
- This result suggests that colored sunglasses should NOT be recommended for patients who have anomalous color vision (especially protans who may have difficulty seeing Red traffic lights even under normal conditions)
Explain why Protans perceive REDS as dim or dark.
- The photopic luminosity curve peaks at approximately 555 nm – it has this shape because there is a disproportionately large input (approximately 2 or 3 to 1) from LWS cones compared to MWS cones & little/no input from SWS cones
- Consequently, compared to color normal, one would predict that protanopes (who lack LWS photopigment) and protanomalous trichromats, (who have an LWS photopigment that is more like the normal MWS photopigment), would experience a severe reduction in the relative brightness of RED LIGHTS
- Examples proving:
- Protans have difficulty seeing automobile REAR TAIL & BRAKE LIGHTS under conditions in which it is easy for color normal to see them
- This can lead to delayed reaction times, which has been linked to a higher incidence of rear-end automobile accidents in protans
- Protans have difficulty seeing automobile REAR TAIL & BRAKE LIGHTS under conditions in which it is easy for color normal to see them
- Protans may also have difficulty seeing RED traffic lights even under normal conditions
- Deep-Red (long-wavelength) surface colors may look quite black to protanopes
- The red light- emitting dioees on consumer electronic devices can be difficult for protans to see
- Deep-red laser pointers can be invisible to protans
- Deutans have NO significant relative LOSS of luminosity compared to normal – this is also the case for deuteranomalous trichromats
Describe the inheritance pattern of Red-Green Color Vision Defect if father is normal, mother is anomalous
- Daughters: 100% carriers but color normal
- Sons: 100% color deficient (anomalous)
Describe the inheritance pattern of Red-Green Color Vision Defect if father is normal, mother is carrier
- Daughters: 50% carriers, 50% color normal
- Sons: 50% color deficient (anomalous), 50% normal
Describe the inheritance pattern of Red-Green Color Vision Defect if father is anomalous, mother is normal
- Daughters: 100% carriers but color normal
- Sons: 100% color normal
Describe the inheritance pattern of Red-Green Color Vision Defect if father is normal, mother is normal
- Daughters: 100% color normal
- Sons: 100% color normal
Describe the inheritance pattern of Red-Green Color Vision Defect if father is anomalous, mother is carrier
- Daughters: 50% color deficient (anomalous); 50% carriers but color normal
- Sons: 50% color deficient (anomalous); 50% color normal
Describe the molecular genetics of Red-Green Color Vision Anomalies
- The X-chromosome typically has one copy of the L-cone opsin gene an one or more copies of the M-cone opsin gene
- Intergenetic –
- Normal
- Dichromacy
- Intragenetic –
- Normal
- Dichromacy
- Anomalous trichromacy
- Hybrid gene – the basis for anomalous trichromacy
Describe the difference between acquired and inherited (congenital) color vision defect.
- Inherited Color vision anomalies –
- Heterozygous female (xX) is a carrier (x to take place of X with the bar over it)
- A male with the defect gene has anomalous color vision (xY)
- Because the gene is recessive, a female must be homozygous (xx) to express the color vision anomaly
- Note: a son (male) always receives the defective gene from the mother
- Patients sometimes incorrectly assume that color anomalies are transmitted from father to son
- Note: a son (male) always receives the defective gene from the mother
- Acquired color vision anomalies –
- Secondary to disease or toxicity
- May be either red-green or blue-yellow in nature
- Because blue-yellow anomalies are so rarely inherited, it must be assumed that such an anomaly is acquired until proven otherwise
- Acquired anomalies may be unilateral or asymmetric
Describe Laterality in Acquired Anomalies.
- Must be assumed any difference in the color vision of the two eyes, as demonstrated on a color vision test, is due to an acquired anomaly
- Because acquired anomalies may be asymmetric, important to perform color vision tests monocularly when screening for these conditions
- If a patient with a unilateral anomaly is tested binocularly, the results may be normal, reflecting the performance of the unaffected eye
- The test should be first administered monocularly to the eye most likely to harbor disease
Describe the features of Autosomal Recessive (AR) achromatopsia. What lenses would help such patients?
- Rare condition where the patient manifests monochromatic vision; fully expressed at birth
- Autosomal Recessive Achromatopsias – common
- Two types:
- Complete AR Achromatopsia – only rods are present
- Incomplete AR Achromatopsia – residual L/M cone function
- Signs & Symptoms:
- No or very poor color discrimination, nystagmus, photophobia and VA ~20/200
- Differential diagnosis is not always straight forward
- Two types:
- Dark red lenses
- Minimize rhodopsin bleaching
- Recommended for bright light conditions
Describe the features of X-linked (XL) achromatopsia. What lenses would help such patients?
- X-Linked (XL) Achromatopsia – recessive inheritance
- Referred to as Blue or S cone monochromacy
- Contain only rods & S cone (though sometimes other cone types can be present)
- Clinical signs & Symptoms:
- Similar to rod monochromacy
- Magenta colored lenses
- Minimizes rod saturation
- Provide light to enable S cones may provide relief to patients
What is the difference between Achromatopsia and cerebral achromatopsia?
- Cone Monochromacy –
- A rare disorder where VA is normal but patients exhibit monochromatic color matching
- Appears to involve a defect in postreceptoral processing of color information
- Achromatopsias – retina
- Cerebral Achromatopsia – Extrastriate cortex
What is chromatopsia? Give 2 examples two conditions where it can be present.
- Chromatopsias –
- Not true color vision anomalies
- They do not typically produce a decreased ability to discriminate colors
- Represent distortion of color vision
- Not true color vision anomalies
- 2 conditions where it can be present:
- (1) Cataract Extraction
- Perception of blueness (cyanopsia)
- (2) Can be secondary to drugs/medications
- Digitalis may produce – Xanthopsia (yellow vision)
- Fluorescein in fluorescein angiography – xanthopsia
- (1) Cataract Extraction
Define Synesthesia. Why does it occur?
- A neurological phenomenon in which stimulation of ONE sensory or cognitive pathway leads to automatic, involuntary experiences in a SECOND sensory or cognitive pathway
- Cortical modules appear to be abnormally linked to each other such that stimulation of one sense results in the activation of another
- Increased cross-talk between regions specialized for different functions may account for the many types of synesthesia
- In one form of synesthesia, the presentation of a LETTER or a NUMBER results in the perception of a COLOR
- For instance, the patient may report the perception of:
- Green whenever he/she is presented with the number 5
- Red when presented with the number 9
- Not only can the effect be elicited by physical presentation of a stimulus, there is evidence that the same perception can result without external presentation of the trigger stimulus
- The patient may, for example, experience GREEN when asked to perform a mental calculation whose answer is the trigger stimulus (e.g., 4 + 1)
- For instance, the patient may report the perception of:
What is chromesthesia?
- Another common form of synesthesia is the association of SOUNDS with colors
- For some, everyday sounds such as doors opening, cars honking, or people talking can trigger seeing colors
- For others, colors are triggered when musical notes and/or keys are being played
- People with synesthesia related to music may also have perfect pitch because their ability to see/hear colors aids them in identifying notes or keys
Define Kollner’s Rule with few examples.
- According to Kollner’s rule, outer retinal disease and media changes result in blue-yellow color vision anomalies, whereas disease of the inner retina, optic nerve, visual pathawys, and visual cortex results in red-green anomalies
- This general guideline is not correct in every instance, and important exceptions have been reported
- Post receptoral lesions are more likely to affect both the types of cones opponent neuron channels
- It is not uncommon for a blue-yellow anomaly to be presen tin the early stages of an eye disease, and change into a red-green anomaly as the disease progresses (the reverse also may happen)
- A pt may manifest a nonselective loss
What type of color vision defect may occur in AMD, Diabetic Retinopathy, and Nuclear Sclerosis, Cataract?
What are the Aims of Color Vision Testing?
- Based on the knowledge of confusion lines
- Color vision testing is the existence of highly predictable confusion lines that allows for clever diagnostic distinction between color defective types
- Goals:
- Screening – i.e., congenital vs. acquired
- Diagnosis – i.e., type and severity
- Vocation or occupational testing
- Determining protan vs. deutan congenital deficiencies: inability to detect red (protan) can have greater occupational consequences
- Focus also on differentiating red-green vs. blue-yellow acquired defects
- Red-green – cone and optic nerve disease
- Blue-yellow – retinal and choroidal disease
Provide few examples of pseudoiochromatic (PIC) plate tests
- Ishihara
- Sensitive
- Protan and deutan defects
- HRR
- Sensitive
- Protan, deutan, tritan, and severity
- CVTME – color vision testing made easy
*patterns of objects, letters, or numbers placed on isoluminant backgrounds
State few examples of Arrangement color vision tests
- Color cap tests
- Farnsworth-Munsell 100
- Panel D-15
- Lanthony Desaturated D15
State few examples of color matching tests
- Anomaloscopes
- C100
- CUT
State an example of naming & occupational color vision tests
- NAMING
- E.g., often PIC, but sometimes tests specifically designed for vocation/occupations (Lantern tests)
- OCCUPATIONAL COLOR TESTS
- Rabin Cone Contrast Test
What is the role of disappearing (vanishing) plates in Pseudoisochromatic plate tests?
- The target/stimulus is defined by a color difference from the background
- Color differences of the target/symbol straddle confusion lines
- If the color differences are aligned on or close to the dichromatic confusion lines, the object is invisible to those with certain color deficiencies
- It serves to detect both types but not to distinguish them
- CHROMATICITIES OF A “DISAPPEARING PLATE” FROM ISHIHARA’S TEST:
- The colors of the figure and the background are in the portion of color space, where there is little difference between protans and deutans
- The colors straddle both confusion lines and the figure will not be seen by either type of CVD
- Shortcomings:
- Slower administration for CVD