Signs and symptoms
Patients with cerebral achromatopsia deny having any experience of color when asked and fail standard clinical assessments like the Farnsworth-Munsell 100-hue test (a test of color ordering with no naming requirements). Patients may often not notice their loss of color vision and merely describe the world they see as being "drab". Most describe seeing the world in "shades of gray". This observation notes a key difference between cerebral and congenital achromatopsia, as those born with achromatopsia have never had an experience of color or gray.Pathophysiology
Cerebral achromatopsia differs from other forms of color blindness in subtle but important ways. It is a consequence of cortical damage that arises throughClassification
A 2005 study examined 92 case studies since 1970 in which cerebral lesions affected color vision. The severity and size of the visual field affected in cerebral achromatopsiacs vary from patient to patient.Bilateral and hemifield
The majority of cases in the 2005 study were the result of bilateral lesions in the ventral occipital cortex. It is unknown whether this was the result of bilateral lesions being more likely to produce color-loss symptoms, or if it was a sampling effect of patients with more severe brain trauma more often being admitted for treatment. In many of the cases examined, patients reported only partial loss of color vision. The locations of color vision loss can be restricted to one hemisphere or one quarter of the visual field. The term "hemiachromatopsia" has been used to denote patients who experience loss of color in only one hemisphere of the visual field. However, as applied to achromatopia resulting from brain trauma, the term is incomplete in characterizing the often-complex nature of the vision loss.Transient
In still rarer cases, temporary ischemia of the associated ventral occipital cortex can result in transient achromatopsia. The condition has thus far been characterized only in stroke patients and provides further support for a color processing area. In one case, a 78-year-old stroke victim had lost the ability to identify color, but was unaware of his deficit until doctors performed color discretion tests. Even when presented with this information, the patient believed he had retained his ability to perceive color even though the world around him appeared grey. He attributed this achromatism to "poor lighting" and it took several weeks for the patient to fully appreciate the extent of his disability. In addition, the characteristic comorbidity of prosopagnosia was present. After two months and frequent sessions with doctors, tests indicated his color had fully returned. The ischemia caused by lesions on the posterior cerebral arteries had subsided and follow up MRI scans indicated that blood flow had once again returned to the ventral occipital cortex.Co-occurrence with other deficits
The most common disorder seen alongside cerebral achromatopsia isDiagnosis
Cerebral achromatopsia can be diagnosed easily withDifference from congenital achromatopsia
The most apparent distinguishing characteristic between congenital achromatopsia and cerebral achromatopsia is the sudden onset of color vision loss following a severe head injury or damage to the occipital lobe following a stroke or similar ischemic event. Non-invasive imaging techniques can be the most helpful in determining whether the area of damage following a traumatic event is an correlated with color-vision processing. Simple diagnostic tools can also be used to determine whether a patient is a likely candidate for further testing, as advancing imaging procedures can often prove expensive and unnecessary. Co-morbid factors can be valuable indicators of the likelihood of cerebral achromatopsia. One disorder often seen alongside cerebral achromatopsia is prosopagnosia, the inability to recall or recognize faces. The correlation is still the subject of ongoing research, but the most telltale clue in this association is the close proximity of brain lesions seen in prosopagnosics and cerebral achromatopsiacs without prosopagnosia. Figure 1 illustrates overlap of brain lesions compiled from numerous case reports of both disorders. A common area of damage associated with both disorders can be seen in the right occipital lobe.Treatment
No current treatment is known for the disorder.History
The number of reported cases of cerebral achromatopsia are relatively few compared with other forms of color-vision loss. In addition, the severity of the color perception deficits along with other psychological effects vary between patients. A case of cerebral achromatopsia, acquired after a cortical lesion, was described by Dr. Verrey in 1888. but the evidence was dismissed by both Gordon Holmes and Salomon Henschen, two eminent neurologists who had identified the position and limits of the primary visual cortex. Verrey's description all but disappeared until a color center was identified in macaque monkey. and until a review of the literature showed the common location of the rare cases of cerebral acrhomatopsiaCurrent research
Based on the knowledge gained from cerebral achromatopsia case studies, current research is focusing on learning more about the cortical area involved in color processing. A recent study provided some of the first direct evidence of color-specific processing in the human ventral occipital cortex. A subject with a history of seizure activity was examined using fMRI and electrode implantation. Using the fMRI, researchers examined the areas of blood oxygenation in the occipital lobe as the subject viewed various color-specific stimuli. The result of the experiment was the identification of an area in the subject, slightly anterior to the lesioned area in cerebral achromatic patients, that responded to variance in color stimulation. The resolution of the MRI was a limiting factor in identifying areas corresponding to specific colors. The next portion of the study used an electrode implanted in the right hemisphere in the location identified by the fMRI scan as pertaining to color processing. It was found the electrical activity of the area increased when the subject was presented with blue stimuli. The next, and most significant finding of the study, was that when the electrode was used to present an electrical stimulus in the subject’s brain, the subject reported the perception of the color blue. Such a result is consistent with other reports of electrical stimulation in visual field maps eliciting perception of phosphines in subjects’ visual field. The color stimulus presented is not the only factor in determining the involvement of the ventral occipital cortex in color processing. The amount of attention and the type of object also affect the activation of the ventral occipital cortex. It has been noted that this area of the occipital lobe may not be a processing center but rather a pathway that is a critical intersection of several cortical areas involved in color perception.Society and culture
The disorder is often presented as evidence of our incomplete knowledge of color processing. Color vision research is a well-studied field of modern neuroscience and the underlying anatomical processing in the retina have been well categorized. The presence of another factor in the perception of color by humans illustrates the need for more research.The case of the colorblind painter
The most famous instance of cerebral achromatopsia is that of "Jonathan I." immortalized in a case study bySee also
*References
{{Medicine Color vision Visual disturbances and blindness Neuropsychology Agnosia Stroke