Hemîachromatopsia of Unilateral Occipitotemporal
Infarcts
Henry L. Paulson, M.D., Steven L. Galetta, M.D., Murray Grossman, M.D.,
and Abass A l a v i , M . D .
Two patients developed unilateral occipito­
temporal infarcts that produced inferior qua­
d r a t i c achromatopsia and an accompanying
superior quadrantanopia. Magnetic resonance
imaging and single-photon emission comput­
ed tomographic studies of both patients sup­
ported the current view that color vision is
encoded in the lingual and fusiform gyri.
Although the quadrantic defect in color proc­
essing was profound, neither patient was
aware of it. Simple bedside testing of patients
with superior quadrantanopia may disclose
an unrecognized quadrantic achromatopsia.
M O R E THAN A CENTURY AGO, Verrey1 described

hemîachromatopsia, the loss of color vision in
one hemifield, and claimed that a functionally
separate cortex existed for color processing.
However, it is only in the pasiL 20 years that the
notion of a neuroanatomically distinct region
for color vision has been accepted. 2 Primate and
human studies have designated a region of the
visual association cortex known as V4 to be
critical for color processing. 39 In humans the V4
homolog resides in the ventromedial occipito­
temporal cortex. The close proximity of this
region to the inferior striate cortex suggests that
hemîachromatopsia might commonly occur in
patients with superior quadrant field defects.
Using both single-photon emission computed
tomography and magnetic resonance imaging,
we studied the neuro-ophthalmologic and neuroanatomic findings in two patients in whom
unilateral occipitotemporal infarcts produced
inferior quadrantic achromatopsia and a superi­
or quadrantanopia.

Accepted for publication March 18, 1994.
From the Departments of Neurology (Drs. Paulson,
Galetta, and Grossman) and Nuclear Medicine (Dr.
Alavi), University of Pennsylvania School of Medicine,
Philadelphia, Pennsylvania.
Reprint requests to Steven L. Galetta, M.D., Depart­
ment of Neurology, Hospital of the University of Penn­
sylvania, 3400 Spruce St., Philadelphia, PA 19104.

518

Subjects and Methods
Single-photon emission computed tomogra­
phy provides a measure of regional cerebral
blood flow, corresponding to the uptake of an
injected radiopharmaceutical. With both pa­
tients described in this study, the single-pho­
ton emission computed tomographic scan was
initiated 20 to 30 minutes after the injection of
20 mCi of 99M Tc-hexamethyl propylene amine
oxime and was performed with a three-headed
rotating single-photon emission computed to­
mography camera (Picker Prism; Picker Inter­
national, Cleveland, Ohio) for 40 minutes by
using high-resolution fanbeam collimators.
Projection images were obtained at 3-degree
angles on a 128 x 128 matrix over 360 degrees,
and reconstructed in the transaxial, coronal,
and sagittal planes (slice thickness, 10.7 mm).
The images were generated by normalizing the
maximal pixel counts to the maximal intensity
of a signal density calibration bar, and thus
represent a measurement of relative blood flow.

Case Reports
Case 1
In June 1992, a 56-year-old, right-handed
man was hospitalized because of the onset of
atrial fibrillation and sudden left visual field
loss. He denied other neurologic symptoms,
including weakness, sensory change, gait, or
speech disturbance. Neurologic examination
disclosed an isolated left homonymous superior
quadrantanopia. Computed tomography of the
brain and results of laboratory tests were unre­
markable except for mildly increased cholester­
ol levels. A transthoracic echocardiogram did
not disclose a thrombus. Because of the pre­
sumed diagnosis of a right occipital infarct that
was cardioembolic in origin, he was discharged
on warfarin therapy.

© A M E R I C A N JOURNAL OF OPHTHALMOLOGY 118:518-523, OCTOBER, 1994

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Fig. 1 (Paulson and associates). Goldmann perimetry in Patient 1, two months after his infarct, demonstrates a
left homonymous superior quadrantanopia with an intact inferior quadrant.

When first seen by us two months later, he
reported that his visual field loss had not
changed. Neurologic examination disclosed a
congruous left superior quadrantanopia, con­
firmed by Goldmann perimetry (Fig. 1). Results
of the rest of the neuro-ophthalmologic exami­
nation were normal.
When he was seen again seven and ten
months after the infarct, his superior quadran­
tanopia persisted and was confirmed by repeat
Goldmann perimetry. Light detection and form
vision were normal in the left inferior quadrant,
but color vision in this quadrant was profound­
ly disturbed. When vision was fixed at a central
point on a white background, the patient could
not sort colored threads or identify the color of
objects in the left inferior visual quadrant. In
this quadrant, objects and threads were most
frequently described as "washed out" or vari­
ous hues of gray. When colored threads or
objects crossed the midline, he immediately
identified their color. Only one of 25 colored
threads (orange) ranging from red to indigo was
correctly named, and only one of 15 attempts to
sort pairs of isoluminescent colored threads
was successful in the left inferior field. Even
when he was made aware of this marked distur­
bance in color processing, he could not retro­
spectively identify any problem with color vi­
sion. Results of the rest of the neurologic
examination were normal. Specifically, there

was no neglect, prosopagnosia, object agnosia,
or akinetopsia.
Magnetic resonance imaging six months after
the onset of his visual disturbance demonstrat­
ed an infarct in the inferomedial right temporal
lobe centered in the fusiform gyrus, partially
involving the lingual gyrus, and sparing the
occipital pole (Fig. 2). A lacunar infarct in the
right thalamus was also seen.
Seven months after the infarct, 99MTc-hexamethyl propylene amine oxime single-pho­
ton emission computed tomographic scanning
was obtained to determine the extent of the
perfusion defect corresponding to the infarct.
The single-photon emission computed tomo­
graphic scan showed a prominent perfusion
defect spanning the right ventromedial occipi­
tal and inferomedial temporal lobes (Fig. 2).
Case 2

In January 1993, a 76-year-old, right-handed
woman with a history of hypertension and
diabetes mellitus developed a right temporal
headache and a feeling that her vision was "not
right." Three days later she came to the hospital
because of dizziness, transient left-sided clum­
siness, and persistent visual disturbance. A
head computed tomography revealed a subacute right occipitotemporal infarct, and an elec­
trocardiogram showed atrial fibrillation.

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AMERICAN JOURNAL OF OPHTHALMOLOGY

October, 1994

Fig. 2 (Paulson and associates). Magnetic reso­
nance imaging (top, left and right) demonstrates the
infarct in patient 1 involving the right occipitotemporal gyri. An axial single-photon emission computed
tomographic image (bottom, left) indicates a perfu­
sion defect corresponding to the infarct.

Although the patient recognized her son, she
thought his face looked "foreign" to her. She
had some difficulty recognizing and describing
complex visual scenes, but named individual
objects and people correctly. There was a con­
gruous left superior quadrantanopia, confirmed
by Goldmann perimetry. Light detection and
form vision were normal in the left inferior
quadrant, but color vision was markedly abnor­
mal in this quadrant. She could not sort colors
or name the color of objects in the left inferior

quadrant but immediately recognized colors
once they crossed the midline. Our clinical
testing made her aware of this color distur­
bance; however, she, like the first patient, could
not retrospectively identify any noticeable
change in color vision since the infarct. Ocular
pursuit movements to the right were saccadic.
There was a mild left sensory drift without
neglect along with a moderate stocking-andglove peripheral neuropathy.
Magnetic resonance imaging (Fig. 3) obtained

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Hemiachromatopsia

521

Fig. 3 (Paulson and associates). Magnetic reso­
nance imaging (top, left and right) demonstrates the
infarct in Patient 2, involving the right occipitotemporal gyri. The sagittal view demonstrates that the
infarct extends posteriorly to include the lingual
gyrus (arrow), but does not extend beyond the calca­
rine sulcus (arrowhead). An axial single-photon
emission computed tomographic image (bottom, left)
illustrates a perfusion defect corresponding to the
infarct.

four days after admission showed a subacute
hemorrhagic infarct involving the right inferomedial temporal and occipital lobes, extending
dorsally to the calcarine fissure. The infarct
involved the lingual and fusiform gyri as well
as adjacent white matter. There were also sev­
eral previously noted small areas of signal ab­
normality in the brain stem, left caudate nucle­
us, and periventricular and subcortical white
matter, all consistent with prior small-vessel
disease. 99M Tc-hexamethyl propylene amine

oxine single-photon emission computed tomo­
graphic scan of the brain (Fig. 3) several days
later disclosed a prominent perfusion defect in
the right ventromedial occipital and inferomedial temporal lobes.
Laboratory studies disclosed increased glu­
cose, the presence of sickle cell trait, and a
microcytic anemia. Carotid and transcranial
Doppler studies showed mild to moderate
plaque in both carotid systems, and normal
flow in the posterior circulation. A transesoph-

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AMERICAN JOURNAL OF OPHTHALMOLOGY

ageal echocardiogram disclosed a left atrial
thrombus, supporting the clinical impression
that her infarct was cardioembolic.

Discussion
As a result of unilateral ventromedial occipitotemporal infarcts, both of our patients
developed contralateral inferior quadrantic
achromatopsia and superior quadrantanopia.
Achromatic vision was preserved in the inferior
quadrant, indicating the defect in color process­
ing was not caused by a lesion in the primary
visual cortex, but rather by one in the prestriate
visual association cortex. Although in both pa­
tients the quadrantic achromatopsia was pro­
found, both patients were unaware of their
problem with color vision until bedside testing
revealed it to them. These two patients allow us
to make several points about the manifestation
and localization of color processing in the hu­
man central nervous system.
Although a designated area for color vision
processing has recently been challenged, 10 "
our patients confirm that a lesion inferior to the
calcarine fissure produces a color vision deficit
involving the entire hemifield. The location of
our patients' infarcts suggests that the human
homolog of V4 resides in the fusiform and
lingual gyri. Even in Verrey's original 1888
report of a patient with hemiachromatopsia,
the autopsy showed damage to these two gyri. 1
Most recently reported cases of central achro­
matopsia (both complete and hemiachromatop­
sia) have demonstrated bilateral cortical inju­
ry.3'4 There are surprisingly few high-resolution
radiographie images of unilateral lesions caus­
ing hemiachromatopsia. The most compelling
is Kolmel's description 1 ' of two patients with
pure hemiachromatopsia, in which magnetic
resonance imaging disclosed discrete lesions
within the fusiform and lingual gyri. Similarly,
in our first patient the magnetic resonance im­
aging showed an infarct that involved the fusi­
form gyrus and, to a lesser degree, the lingual
gyrus. Although our second patient's infarct
was larger, it still encompassed the entire fusi­
form and lingual gyri as well as adjacent white
matter. In both patients, brain single-photon
emission computed tomographic scans cor­
roborated the magnetic resonance imaging
findings. The single-photon emission comput­
ed tomographic studies convincingly docu­
mented that the quadrantic achromatopsia was

October, 1994

associated with a unilateral perfusion de­
fect confined to the ventral occipitotemporal
region.
The superior quadrantanopia present in our
two cases is common in patients with hemiach­
romatopsia or complete achromatopsia. For ex­
ample, in Meadows's review 3 of the literature
on central achromatopsia, all 14 patients had a
field defect in at least one superior quadrant,
and frequently in both upper quadrants. This
field defect associated with achromatopsia pre­
sumably occurs both because of the close ana­
tomic relationship of the fusiform and lingual
gyri to the inferior bank of striate cortex, and
because of their shared vascular supply (an
inferior branch of the posterior cerebral artery).
An occipitotemporal lesion may cause a superi­
or quadrantanopia by directly damaging the
inferior striate cortex (VI), as likely occurred in
our second patient. Alternatively, lesions that
spare VI may still result in a superior field
defect by damaging the adjacent prestriate cor­
tex, V2/V3, which contains a retinotopic map
that directly and reciprocally connects to VI. 12
Finally, a superior quadrantanopia may occur
from an isolated white matter lesion interrupt­
ing the optic radiations to VI. 13 In most cases,
an infarct producing both a superior quadrant
field defect and inferior quadrantic achroma­
topsia will not be limited to cortex or white
matter alone. This point is illustrated by our
patients. In both, the lesions likely involved
inferior optic radiations as well as prestriate
visual association cortex, including V4. The
involvement of both optic radiations and pre­
striate cortex may be clinically important, since
it has been proposed that involvement of the
adjacent white matter could contribute to the
severe color processing deficits seen in some
patients. 10
It is important to emphasize that neither of
our patients was aware of their dense quadran­
tic achromatopsia. They reliably experienced
colors in the world around them because color
vision was intact in their remaining hemifield.
Yet even when told of their quadrantic defect,
neither was able retrospectively to identify a
change in color vision. It was only when atten­
tion was drawn to their color deficit that either
patient "saw" colors as gray or dulled in the
affected quadrant. Possibly this represents a
form of anosognosia, or lack of awareness of the
deficit. Although both patients' infarcts were in
the nondominant right hemisphere, there were
no other signs of neglect in either patient. A
review of the literature suggests this lack of

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Vol. 118, No. 4

a w a r e n e s s of a q u a d r a n t i c a c h r o m a t o p s i a m i g h t
not b e rare. For e x a m p l e , a l t h o u g h all five of
G r e e n a n d Lessell's 4 p a t i e n t s w i t h a c h r o m a t o p ­
sia h a d bilateral l e s i o n s , t w o of the p a t i e n t s
initially h a d a s u p e r i o r q u a d r a n t a n o p i a w i t h o u t
o b v i o u s defects in color v i s i o n . In b o t h cases a
s e c o n d c o n t r a l a t e r a l lesion led to a c h r o m a t o p ­
sia involving n o t o n e b u t b o t h hemifields,
w h i c h s u g g e s t s t h a t a u n i l a t e r a l defect in color
p r o c e s s i n g w e n t u n d e t e c t e d after the first le­
sion. The s a m e may a p p l y to P e a r l m a n , Birch,
a n d M e a d o w s ' s 1 4 p a t i e n t , w h o also initially h a d
a s u p e r i o r q u a d r a n t a n o p i a from a u n i l a t e r a l
infarct, b u t d e v e l o p e d a c h r o m a t o p s i a in b o t h
hemifields only after a s e c o n d c o n t r a l a t e r a l
lesion. W h e t h e r a lack of a w a r e n e s s of q u a d r a n ­
tic a c h r o m a t o p s i a occurs preferentially w i t h
right h e m i s p h e r i c lesions is u n k n o w n , a n d will
require directly c o m p a r i n g the clinical findings
in p a t i e n t s w i t h left vs right h e m i s p h e r i c le­
sions.
There are m a n y e l a b o r a t e tests t h a t m a y b e
u s e d to define a deficit in color p r o c e s s i n g ,
i n c l u d i n g color p e r i m e t r y a n d t h e F a r n s w o r t h M u n s e l l 1 0 0 - H u e Test. H o w e v e r , the s i m p l e
b e d s i d e tests we h a v e u s e d s e r v e well to i d e n t i ­
fy a c h r o m a t o p s i a . A n initial quick screen w i t h
colored objects will u n c o v e r a defect in color
vision. A s e c o n d test i n v o l v i n g the s o r t i n g of
i s o l u m i n e s c e n t colored t h r e a d s or disks can
identify a t r u e a c h r o m a t o p s i a . As l o n g as t h e
e x a m i n e r d o c u m e n t s t h a t a c h r o m a t i c vision is
well p r e s e r v e d in the affected q u a d r a n t a n d
carefully m o n i t o r s visual fixation w h e n colors
are p r e s e n t e d to this q u a d r a n t , o n e can r a p i d l y
a n d easily detect a q u a d r a n t i c a c h r o m a t o p s i a .

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523

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