Achromatopsia
Achromatopsia
Achromatopsia, or dyschromatopsia, is an inability or impaired ability to perceive colors. This may be ophthalmological or neurological in origin, congenital or acquired; only in the latter case does the patient complain of impaired color vision.
Achromatopsia is most conveniently tested for clinically using pseudoisochromatic figures (e.g., Ishihara plates), although these were specifically designed for detecting congenital color blindness and test the red-green channel more than blue-yellow. Sorting colors according to hue, for example with the Farnsworth-Munsell 100 Hue test, is more quantitative, but more time consuming. Difficulty performing these tests does not always reflect achromatopsia (see Pseudoachromatopsia). Probably the most common cause of achromatopsia is inherited "color blindness", of which several types are recognized: in monochromats only one of the three cone photoreceptor classes is affected, in dichromats two; anomalous sensitivity to specific wavelengths of light may also occur (anomalous trichromat). These inherited dyschromatopsias are binocular and symmetrical and do not change with time.
Acquired achromatopsia may result from damage to the optic nerve or the cerebral cortex. Unlike inherited conditions, these deficits are noticeable (patients describe the world as looking "gray" or "washed out") and may be confined to only part of the visual field (e.g., hemiachromatopsia).
Optic neuritis typically impairs color vision (red-green > blue-yellow), and this defect may persist while other features of the acute inflammation (impaired visual acuity, central scotoma) remit.
Cerebral achromatopsia results from cortical damage (most usually infarction) to the inferior occipitotemporal area. Area V4 of the visual cortex, which is devoted to color processing, is in the occipitotemporal (fusiform) and lingual gyri. Unilateral lesions may produce a homonymous hemiachromatopsia. Lesions in this region may also produce prosopagnosia, alexia, and visual field defects, either a peripheral scotoma which is always in the upper visual field, or a superior quadrantanopia, reflecting damage to the inferior limb of the calcarine sulcus in addition to the adjacent fusiform gyrus. Transient achromatopsia in the context of vertebrobasilar ischemia has been reported.
The differential diagnosis of achromatopsia encompasses color agnosia, a loss of color knowledge despite intact perception; and color anomia, an inability to name colors despite intact perception.
References
Orrell RW, James-Galton M, Stevens JM, Rossor MN. Cerebral achromatopsia as a presentation of Trousseau’s syndrome. Postgraduate Medical Journal 1995; 71: 44-46
Zeki S. A century of cerebral achromatopsia. Brain 1990; 113: 1721-1777
Cross References
Agnosia; Alexia; Anomia; Prosopagnosia; Pseudoachromatopsia; Quadrantanopia; Scotoma; Xanthopsia
