Neurocase (2001) Vol. 7, pp. 331–337

© Oxford University Press 2001

Localization of Hemiachromatopsia
R. A. Short and N. R. Graff-Radford
Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA

Abstract
Impaired color perception with relative preservation of form vision (achromatopsia) caused by cerebral lesions was
first described over a century ago. However, for many years some researchers questioned the existence of an area of
cerebral cortex apart from the primary visual cortex specialized in color processing. The development of sophisticated
structural and functional neuroimaging techniques has allowed verification of the cortical structures important in
color perception. We describe a case of a patient with impaired color perception in one hemifield of vision
(hemiachromatopsia) and compare the localization of the lesion with previous cases described in the literature. These
cases show that lesions of the ventromedial occipital cortex can impair color perception and leave form vision intact.

Introduction
Central achromatopsia is a disorder of impaired color perception caused by an acquired cerebral lesion (Tranel, 1998). In
1888, Verrey reported a case of hemiachromatopsia that on
post-mortem examination had a lesion located in the fusiform
and lingual gyri of the contralateral inferior occipital cortex
implicating these cerebral areas as specialized in color
processing (Zeki, 1990). Even though several cases of central
achromatopsia were reported over the ensuing decades, many
researchers did not accept that an area of cerebral cortex
specialized in color processing existed outside of the primary
visual (striate) cortex. The reasons for questioning the concept
of central achromatopsia included lack of standardized color
testing techniques and lack of pathological data in some cases.
Some authors also believed that tests of color perception were
simply a more sensitive method of detecting visual field
deficits (or impairments of form vision). Therefore, the
lesions responsible for the loss of color vision were likely to
be in the same areas associated with visual field defects, i.e.
the primary visual cortex or optic radiations. However, in
1974, Meadows resurrected the hypothesis that the inferior
occipital cortex is particularly specialized in color perception,
based on a review of reported cases of central achromatopsia.
All of the cases had visual field defects involving the upper
visual quadrants but never exclusively the lower visual
quadrants (Meadows, 1974). If the color vision center was
in close proximity to the inferior striate cortex or inferior
optic radiations then this could explain why lesions causing
achromatopsia also often cause upper visual quadrant defects.
A year previously, Zeki had demonstrated that certain cortical
areas of the monkey may be preferentially responsive to
color stimuli by showing pre-striate area V4 contained regions

that responded differentially to various wavelengths of light
(Zeki, 1973, 1977). However, area V4 seemed to be located
much more superiorly in the occipital cortex than the lingual
and fusiform gyri and thus, the homologous area in humans,
if it existed, still needed confirmation.
The advent of improved structural and functional neuroimaging techniques in the last two decades has allowed
further definition of the cortical areas involved in color
processing in humans. These techniques seem to confirm
that the lingual and fusiform gyri in humans contain the
homologue of area V4 in monkeys. We will review the
neuroimaging of the previously reported cases of achromatopsia and our own case to localize further the cortical areas
in humans most important in color perception.

Case
A 68-year-old right-handed woman was referred for evaluation of memory. She had been diagnosed several weeks prior
with a stroke presumed to be cardioembolic from atrial
fibrillation. Her only complaint was that she could not read
and had no other neurological symptoms except for occasional
mild headaches. She was a retired office worker with a high
school education and she had been an avid reader prior to
her stroke. She had a remote history of nephrectomy for
kidney cancer and hysterectomy and oopherectomy.
On neurological examination she scored 24/30 on the
Mini-Mental State Examination (MMSE) missing four
points on orientation and two on delayed recall. Her reading
was slow and she had to resort to spelling out many words,

Correspondence to: R. A. Short, 200 Hawkins Drive, Iowa City, IA 52242, USA. Tel: ⫹1 319 356 2551; Fax: ⫹1 319 356 4505;
e-mail: ShortR@mail.medicine.uiowa.edu

332 R. A. Short and N. R. Graff-Radford

Fig. 1. Humphrey visual fields.

letter by letter. There was no finger agnosia, dysgraphia or
acalculia. She had some mild difficulty in naming objects
within the room, but otherwise had no aphasia. There was
no visual field deficit to confrontation, nor did she have
simultanagnosia, optic ataxia or optic apraxia. However,
she described bright colors (red, green, blue, and yellow)
presented in the right hemifield of each eye to be gray and
could not identify the colors. She was surprised that the
colors appeared gray as she had not noticed it prior to
the examination. Thirty minutes later, when asked about
visual problems, she acknowledged something was wrong
with her vision but she did not know what it was. She could
name colors correctly in the left hemifield. Motor and sensory
examination, reflexes, coordination, gait and station were all
normal. The color perception deficits on examination were
reproduced 3 weeks later and 1 year later on follow-up visits,
indicating a stable deficit.
On neuro-ophthalmological examination she had a right
superior quadrantanopsia (Fig. 1). Visual acuity was 20/30
OD and 20/25 OS. She was able to identify nine of
10 Hardy–Rand–Rittler (HRR) color plates in all except
the right superior quadrant. No other ophthalmological
abnormalities were found except for mild bilateral nuclear
sclerotic cataracts. The neuropsychological testing results
are summarized in Table 1, which shows impairments in
general intellect, memory and language. On the Wide Range
Achievement Test Revision 3 (WRAT3) subtests of reading
and spelling, she scored in the 30th and 68th percentiles,
indicating more difficulty with reading than writing (i.e.
partial alexia). The responses on the Boston Naming Test
were circumlocutory without paraphasic errors and without
much benefit from phonemic cues. The responses indicated
intact knowledge about objects. Examples of her responses
include: whistle—‘something you blow into’, when given

phonemic cue—‘not a whistle’; scissors—gestures correctly
with hands and answers ‘not scissors but in the family’;
broom—gestures sweeping motion and answers ‘what you
would do to a floor’, answered correctly when given phonemic
cue. Generative naming showed above average scores on
letter fluency but impaired in category fluency. Her copy of
the Rey–Osterrieth figure was accurate in the parts completed
but she received a low score due to time limits. She did not
have neglect on a letter cancellation test.
Magnetic resonance imaging (MRI) with coronal cuts
parallel to the plane of the face showed an area of ischemic
infarction in the left medial occipital/temporal cortex (Fig. 2).
Also present were areas of subcortical ischemia and a small
area of infarction in the left sensory cortex. Routine laboratory
evaluation, transesophageal echocardiogram and magnetic
resonance angiography were unremarkable.
Mapping of the MRI lesion to coronal templates (90° angle
to orbitomeatal line) in a previously described technique
(Damasio and Damasio, 1989), revealed an area of infarction
in nearly all of the fusiform gyrus and the lingual gyrus and
smaller portions of the parahippocampus and hippocampus
(Fig. 3).This corresponded to Brodmann areas 18, 19, 28,
36, and 37. Spared areas included the supracalcarine cortex,
forceps major, and paraventricular area. The area of infarction
correlated to the distribution of the lateral branch of the left
posterior cerebral artery. Figure 4 is a photograph of the
ventral occipital and temporal cortex of a gross pathological
specimen with the lingual and fusiform gyri highlighted.

Discussion
Previous cases of achromatopsia reported with neuroimaging
findings are summarized in Table 2 (Green and Lessell, 1977;
Pearlman et al., 1979; Damasio et al., 1980; Kolmel, 1988;

Localization of hemiachromatopsia 333
Table 1. Neuropsychological scores
Parameter
Wechsler Adult Intelligence Scale – Reviseda
Verbal IQ
Performance IQ
Full-scale IQ
Wide Range Achievement Test (Revision 3)
Reading
Spelling
Arithmetic
Rey–Osterrieth Complex Figure, copy
Random Letter Cancellation Task
Right
Left
Boston Naming Test
Letter fluencyb (C, F, L)
Category fluencyb (animals, fruits, vegetables)
Trail Making Taskb, part A
Trail Making Taskb, part B
Wisconsin Card Sorting Test
Perseverative responses
Categories
Failure to maintain set
Wechsler Memory Scale–Revisedb
Logical memory I
Logical memory II
Auditory Verbal Learning Testb
Delayed recall
Brief Visual Memory–Revised
Delayed recall

Raw or standard score

Descriptor/percentile

78
78
76

7%
7%
5%

92
68
85
26

30%
25%
16%
⬍1%

29
30
8
41
22
67 s
⬎5 min

Normal
Normal
⬍1%
72–81%
3–5%
2%
⬍1%

43
3
0

6%
⬎16%
⬎16%

6
0

Severely impaired
Severely impaired

0

Severely impaired

2

Severely impaired

aAge-corrected standard scores (mean, 100 ⫾ 15) for factor scores of Wechsler Adult Intelligence Scale using Mayo’s Older Americans Normative Studies
standard scores (Ivnik et al., 1992a, b, c).
bAge-corrected scaled scores (mean, 10 ⫾ 3) using Mayo’s Older Americans Normative Studies scaled scores (Ivnik et al., 1996; Lucas et al., 1998).

Victor et al., 1989; Rizzo et al., 1992, 1993; Paulson et al.,
1994). Alexia and prosopagnosia are common additional
neurobehavioral abnormalities. All but two cases were caused
by an ischemic infarction. The cause of the infarction was
not reported in several of the studies, although both of
Paulson’s cases were presumed cardioembolic (Paulson et al.,
1994). Consistent with Meadows’ review, visual field
defects are common, and when present always involve the
superior visual fields. The visual defects usually do not
involve the entire quadrant or hemifield, but spare central
fixation. Kolmel’s two cases are particularly important as
they demonstrate that achromatopsia can occur without visual
field deficits (Kolmel, 1988).
Although different anatomical terms are used in the previous reports, some comparisons can be made. The lingual
gyrus and the posterior fusiform gyrus occupy the inferior
and medial aspects of the occipital lobe below the calcarine
fissure. The fusiform gyrus is sometimes referred to as the
medial occipital temporal gyrus. Brodmann areas 18 and 19
are visual association areas above and below the calcarine
cortex (area 17). Both the lingual and posterior parts of the
fusiform gyrus contain areas 18 and 19. The common area
of involvement in all of these cases is the ventromedial
aspect of the occipital lobe, which comprises the lingual and
posterior fusiform gyrus and areas 18 and 19. Although our

patient had a more extensive lesion in the fusiform gyrus
extending anteriorly into the temporal lobe (area 37), based
on previous cases, the area causing the hemiachromatopsia
was located more posteriorly. All of the cases had some
sparing of the primary visual cortex and the optic radiations,
indicating that these areas are not responsible for the impairment in color perception. Therefore, our review implicates
the lingual gyrus and occipital portion of the fusiform gyrus
as the cortical areas most likely involved when there is
impairment of color perception. This is consistent with the
findings of previous authors that the caudal portion of the
lingual gyrus is the area most commonly involved in central
achromatopsia (Damasio et al., 1991) .
Functional imaging with positron emission tomography
(PET) scanning provides further evidence that these areas
are important in color processing. Lueck et al. (1989) showed
the lingual and fusiform gyri in the occipital cortex to be
selectively activated when individuals were shown abstract
color (Mondrian) displays in comparison with black and
white displays. McKeefry and Zeki (1997) have localized
color-related activation even further using functional MRI
(fMRI) studies and Mondrian displays. Their data show
activation of the medial aspect of the posterior fusiform
gyrus, which they termed human area V4. This area lies in
the lateral bank of the collateral sulcus, which separates the

334 R. A. Short and N. R. Graff-Radford

Fig. 2. Fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) images, coronal cuts.

Fig. 3. Templates corresponding to images in Fig. 2.

Localization of hemiachromatopsia 335

lingual and fusiform gyri. However, Beauchamp et al. (1999)
have shown that more anterior areas in the fusiform gyrus
and collateral sulcus are also activated on fMRI using passive
viewing of the Farnsworth-Munsell 100-Hue test. Activation
of these color-selective areas increases more than 3-fold
when active sequencing of colors is required, suggesting an
attention component. Beauchamp et al. (2000) have also
reported similar testing on a patient with an incomplete
achromatopsia who was able to name viewed colors but was
impaired on the Farnsworth-Munswell 100-Hue test. On MRI
the patient had ischemic lesions in the bilateral ventral
occipitotemporal cortex with some preservation of more
posterior areas on the left. These posterior areas were still
activated in fMRI imaging, providing a nice correlate to
the fMRI studies on normals. The results of both studies
emphasize the importance of the posterior ventral occipito-

Fig. 4. Medial aspect of occipital and temporal cortex. The highlight shows
the lingual gyrus and the fusiform gyrus.

temporal cortex in simple color perception tasks and the
more anterior areas in more complex color perception tasks.
A few other characteristics of our case deserve mention.
Neither of Paulson’s two patients was aware of the color
deficits and both had left hemiachromatopsia and superior
quadrantanopia with lesions involving the right lingual and
fusiform gyri. The authors postulated that this could be a
form of anosoagnosia related to a right hemisphere lesion
(Paulson et al., 1994). Our patient also had lack of awareness
of the impaired color perception even after the deficit had
been demonstrated to her, suggesting that this is not specific
to right hemispheric lesions. This lack of awareness does not
seem to be caused by visual neglect because she performed
normally on the line cancellation test.
Our patient’s color deficit could not be demonstrated with
HRR color plates. Color plates are designed to test vision
within a central few degrees of fixation, which explains
the difficulty in demonstrating the hemiachromatopsia with
the standard color plates (Damasio et al., 1991). Therefore,
when testing for a partial central achromatopsia, using brightly
colored objects and moving them from one visual quadrant
to another is the preferred method (Damasio et al., 1991)
even though this relies on the patient’s subjective perception
of color.
Our patient also had a combination of an anomia, partial
alexia and amnesia. Although she was not tested for
prosopagnosia, her responses on the Boston Naming Test did
not indicate a visual object agnosia because she was able to
provide circumlocutory responses or gestures, indicating
intact knowledge of the picture objects. However, as shown
in the review table, visual agnosia, particularly prosopagnosia,
often accompanies central achromatopsia. Pure alexia also
often accompanies hemiachromatopsia and in fact in
Dejerine’s classic 1892 case of pure alexia there was mention
of impaired color perception in the right visual field (Damasio
and Damasio, 1983).

Table 2. Summary of cases with achromatopsia and neuroimaging
Author

Achromatopsia

Visual field deficit

Associated findings

Neuroimaging

Green and Lessell, 1977
Pearlman et al., 1979

Bilateral
Bilateral

Bilateral
LSQ

Damasio et al., 1980

Left

Left upper scotoma

Alexia, prosopagnosia
CT
Color anomia, prosopagnosia, CT
topographagnosia
None
CT

Damasio et al., 1980

Bilateral

Left upper scotoma

Prosopagnosia

CT

Victor et al., 1989

Left

Right hemianopia

Anomia, alexia, amnesia

MRI

Kolmel, 1988
Kolmel, 1988
Rizzo et al., 1992

Right
Left
Bilateral

None
None
Left hemianopia

None
None
Prosopagnosia

MRI
MRI
MRI

Rizzo et al., 1993

Bilateral

Left hemianopia

Alexia, prosopagnosia

MRI

Paulson et al., 1994
Paulson et al., 1994

Left
Left

RSQ
RSQ

None
None

MRI
MRI

Lesion location

Cause

B occipital
B lingual and fusiform

Tumor
Infarct

Right ventromedial occipital
(18, 19, ?37)
B ventromedial occipital white
matter (21, 36, 37, 19, 18)
Left extensive occipital, right
ventromedial occipital
Left medial OTG
Right lingual and medial OTG
Extensive right occipital, left
inferior occiptial (18, 19)
Extensive left occipital, right
fusiform and posterior lingual
Right fusiform, partial lingual
Right fusiform and lingual

Infarct
Infarct
Infarct
Infarct
Infarct
Trauma
Infarct
Infarct
Infarct

LSQ, left superior quadrant; RSQ, right superior quadrant; B, bilateral; OTG, occipitotemporal gyrus; MRI, magnetic resonance imaging; CT, computed
tomography.

336 R. A. Short and N. R. Graff-Radford

In summary, our case and review of the literature demonstrate that the ventromedial occipital cortex is crucial to color
processing and that lesions in this area can cause central
achromatopsia. Visual field deficits and various other neurobehavioral deficits commonly occur with the achromatopsia.
The most common cause of the lesion is probably embolic
ischemic infarction in the lateral branch of the posterior
cerebral artery. Patients can sometimes be unaware of the
color deficit. Therefore, the clinician must check for color
perception in patients with lesions in this area to identify the
impairment.

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Received on 6 December, 2000; resubmitted on 3 January, 2001;
accepted on 15 March, 2001

Localization of hemiachromatopsia 337

Localization of hemiachromatopsia
R. A. Short and N. R. Graff-Radford
Abstract
Impaired color perception with relative preservation of form vision
(achromatopsia) caused by cerebral lesions was first described over a century
ago. However, for many years some researchers questioned the existence of
an area of cerebral cortex apart from the primary visual cortex specialized in
color processing. The development of sophisticated structural and functional
neuroimaging techniques has allowed verification of the cortical structures
important in color perception. We describe a case of a patient with impaired
color perception in one hemifield of vision (hemiachromatopsia) and compare
the localization of the lesion with previous cases described in the literature.
These cases show that lesions of the ventromedial occipital cortex can impair
color perception and leave form vision intact.

Journal
Neurocase 2001; 7: 331–7

Neurocase Reference Number:
O227

Primary diagnosis of interest
Hemiachromatopsia

Author’s designation of case
A 68-year-old right-handed woman

Key theoretical issue
d Left ventral occipitotemporal lesions can cause hemiachromatopsia with

lack of awareness of the colour perception deficit
Key words: hemiachromatopsia; colour perception; ventromedial occipital
cortex

Scan, EEG and related measures
Magnetic resonance imaging

Standardized assessment
Neuropsychology, visual fields

Other assessment
Color perception

Lesion location
d Left ventral occipitotemporal

Lesion type
Ischaemic

Language
English