REVIEW ARTICLE


Chiasmal and
C O N T I N UU M A UD I O
I NT E R V I E W A V AI L A B L E
ONLINE
Postchiasmal
Disease
Downloaded from http://journals.lww.com/continuum by 841TdHYulZ42jk40fHZkQEYvs+CyVqc/8DpU8TjBhmyAfvczVCaoWvgiQUASJzsAPIR/s+cCcR4354SamZEg0j0F7tiyJ3x8QXnRujp+OWSuw5+M4hTPac+JNAi6yX8UoSA42jBuh8MKeNlnWCZA9PQHpF3Pgn86wU1D2zVIR2I= on 07/08/2021

By Heather E. Moss, MD, PhD, FAAN

ABSTRACT
PURPOSE OF REVIEW: This article reviews the anatomy, symptoms, examination
findings, and causes of diseases affecting the optic chiasm, optic tracts,
optic radiations, and occipital lobes.

RECENT FINDINGS: Modern ophthalmic imaging can be used to monitor the

CITE AS:
CONTINUUM (MINNEAP MINN) 2019;
effects of diseases of the optic chiasm and tract on the retinal ganglion
25(5, NEURO-OPHTHALMOLOGY): cells. It can also be used to visualize transsynaptic degeneration of the
1310–1328. anterior visual pathway in the setting of acquired retrogeniculate lesions.
Visual prostheses that directly stimulate the occipital lobe are a potential
Address correspondence to
Dr Heather E. Moss, Spencer strategy for rehabilitation that is in active clinical trials.
Center for Vision Research;
2370 Watson Ct, Ste 200,
SUMMARY: Detecting and characterizing visual deficits due to optic chiasm
Palo Alto, CA 94303,
hemoss@stanford.edu. and retrochiasmal disease are important for the diagnosis, localization,
and monitoring of neurologic disease; identifying patient disability; and
RELATIONSHIP DISCLOSURE:
Dr Moss serves on the board of
guiding rehabilitation.
directors of the North American
Neuro-Ophthalmology Society
and as a review editor for Current
Eye Research, an associate INTRODUCTION

editor for Frontiers in Neurology,
a section editor for the Journal
of Neuro-ophthalmology, and a
special section editor for
Neuro-ophthalmology. Dr Moss
receives research/grant support
from the Myelin Repair Foundation,
the National Institutes of
Health/National Eye Institute
(K23 EY024345, P30 EY 026877),
and Research to Prevent
Blindness and publishing
royalties from Elsevier. Dr Moss
has served as a legal consultant
providing record review and
deposition on neuro-ophthalmic
diseases.
UNLABELED USE OF
PRODUCTS/INVESTIGATIONAL USE
DISCLOSURE:
Dr Moss reports no disclosure.
© 2019 American Academy
of Neurology.
A
number of ways to organize a discussion of the afferent visual
pathway are reasonable. A division point at the lateral geniculate
nucleus separates disorders affecting retinal ganglion cells
(pregeniculate) from those affecting cells after the first synapse in
the neurologic visual pathway (postgeniculate). A division point
behind the chiasm separates disorders characterized by heteronomous field loss
(impacting different fields in each eye, seen in prechiasmal and chiasmal lesions)
from those causing unilateral homonymous visual field loss (postchiasmal). A
division point in front of the chiasm separates optic nerve disorders, in which
a single lesion affects monocular vision, from chiasmal and retrochiasmal
disorders, in which a single lesion affects vision in both eyes. This issue of
Continuum divides discussion at the chiasm. Accordingly, this article discusses
the anatomy, symptoms, and common causes of vision loss related to visual
pathway lesions affecting anywhere from the chiasm to the primary visual cortex
in the occipital lobe, including the optic tracts and optic radiations (FIGURE 6-11).
EXAMINATION
Testing of central and peripheral vision in each eye separately is an essential step
in localizing vision loss due to retrobulbar (behind the eye) pathologies.

1310 OCTOBER 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 Central Vision KEY POINTS
Visual acuity represents spatial
● Central vision can be
resolution of vision at the point affected in chiasmal lesions
of visual fixation (eg, the but is spared in unilateral
smallest size of a character such retrochiasmal lesions.
as a letter, number, or shape a
● If a homonymous field
person can distinguish) and is
defect is complete,
typically measured by using localization beyond a
black letters on a white retrochiasmal location is not
background. For monitoring and possible based on
localizing neuro-ophthalmic peripheral vision testing
alone.
diseases, visual acuity should be
measured by using appropriate ● Confrontation visual field
glasses or a pinhole to exclude deficits are specific but not
impairment due to refraction or sensitive for peripheral
vision loss.
mild cataract. Visual acuity can
be affected in eyes that have both ● Optic nerve head pallor in
nasal and temporal field loss both eyes is diagnostic of
from chiasmal disorders but is chronic injury to the retinal
ganglion cells in both optic
FIGURE 6-1 not affected by unilateral
nerves, the chiasm, or one
Major afferent visual pathway structures retrochiasmal lesions. optic tract.
highlighted on prosected gross specimen (ventral
view). Optic chiasm (yellow arrow), optic tracts
(white arrows), lateral geniculate nuclei (red
Peripheral Vision
arrows), optic radiations (blue arrows), and Tests of peripheral vision guide
primary visual cortex (green arrows). localization of vision loss, with
Modified with permission from Sherbondy et al, J Vision.1 chiasmal disturbances classically
© 2008 Association for Research in Vision and
Ophthalmology. causing bitemporal vision loss in
both eyes and retrochiasmal
disturbances causing unilateral
homonymous visual field defects (ie, same side affected in both eyes). If a
homonymous field defect is complete, further localization is not possible based
on peripheral vision testing alone. In cases of incomplete homonymous visual
field loss, some visual field features have localizing value (FIGURE 6-2). These are
highlighted in the relevant anatomic sections below.
Peripheral vision is assessed at the bedside by various confrontation visual
field techniques that are specific for visual field loss but not sensitive when
compared with gold standard visual field testing such as Humphrey visual fields.2
It is particularly difficult to detect visual field loss that spares the far peripheral
fields (ie, central to midperipheral vision loss) or that only involves the far
peripheral fields (ie, spares central and midperipheral fields) on confrontation
testing. Visual field testing, like most sensory aspects of the neurologic
examination, requires active patient participation and is subject to conscious and
unconscious manipulation on this basis.3

Fundus Examination
Pallor of the optic nerve heads suggests chronic injury to the retinal ganglion
cells anywhere along their pathway from the retina to the lateral geniculate
nucleus. Optical coherence tomography (OCT), a near infrared laser–based
technique that is widely used in ophthalmologic clinical settings to obtain
micron-level–resolution images of the retina, can be used to measure the

CONTINUUMJOURNAL.COM 1311

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHIASMAL AND POSTCHIASMAL DISEASE

FIGURE 6-2
Visual field loss patterns due to neurologic disease.

thickness of the ganglion cell layer in the retina, thereby quantifying injury.4 It is
important to note that, although ganglion cell changes in the retina support a
diagnosis of retinal ganglion cell injury, absence of atrophy on OCT (and, in fact,
absence of pallor on funduscopy) does not exclude retrobulbar ganglion cell
injury because lesions that do not result in cell death will not manifest retinal
changes, and retinal changes for lesions that do result in cell death can take
2 weeks to be detected in the retina and 20 weeks to stabilize.5

Pupil Examination
Anisocoria is not caused by lesions involving the afferent visual pathway.
However, any lesion that affects the retinal ganglion cells coming from each eye
in an unequal manner can cause a relative afferent pupillary defect. Relevant
to this article are optic chiasm lesions that affect the eyes asymmetrically and
optic tract lesions that affect more fibers from the contralateral eye than
ipsilateral eye to cause a contralateral relative afferent pupillary defect.6

DISEASES OF THE CHIASM

The optic chiasm’s clinical importance is out of proportion to its size because of
the presence of all afferent fibers from both eyes within its small confines and its
common involvement by sellar and suprasellar tumors.

Anatomy
Visual input coming from the optic nerves of both eyes redistributes by field
at the optic chiasm. Axons supplying the temporal visual fields of each eye
decussate to join axons supplying the nasal visual fields of the contralateral eyes

1312 OCTOBER 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

such that the right visual field of both eyes feeds into the left optic tract and the KEY POINTS
left visual field of both eyes feeds into the right optic tract. The central temporal
● Lack of optic nerve head
fibers from both eyes decussate in the posterior chiasm. pallor does not exclude
injury to the ganglion cells in
Symptoms the optic nerves, chiasm, or
Visual symptoms of chiasmal disease are broad and depend on the anatomic optic tracts.
manner in which the chiasm is affected. Compressive lesions affecting the
● Relative afferent pupillary
chiasm affect the crossing fibers to cause vision loss in the temporal visual fields defects can occur in
in both eyes. Because of the anatomy of the crossing ganglion cell axons, lesions asymmetric chiasm and
affecting the posterior chiasm cause central temporal field loss in both eyes unilateral optic tract lesions.
and spare the periphery, whereas anterior chiasmal compression affects the
● Lesions affecting the
peripheral fields. Anterior lesions can concurrently affect the optic nerves, anterior chiasm affect the
causing superimposed nasal or central visual field defects and potentially visual peripheral temporal fields,
acuity loss in the affected eye(s). When an optic nerve visual field defect is whereas those affecting the
accompanied by a temporal defect in the other eye from concomitant chiasmal posterior chiasm affect the
central temporal fields with
involvement, it is termed a junctional scotoma. Posterior lesions can concurrently sparing of the periphery.
affect the optic tracts, causing superimposed contralateral homonymous visual
field loss. A complete chiasmal lesion causes complete vision loss in both eyes.
Lateral chiasmal compression can cause a nasal field deficit in the ipsilateral eye,
but this is rarely seen in practice.
In the case of complete bitemporal hemianopic visual field loss, the spared
visual fields in each eye are complementary, meaning that the missing field in
each eye is seen by the other eye, so that as long as both eyes are open, the whole
visual world is seen. However, the lack of overlapping vision between the
eyes can cause difficulties with depth perception. Rarer symptoms include
postfixation blindness, in which a blind spot exists behind the object of focus
where the seeing fields diverge,7 and hemifield slide, in which difficulty fusing
the remaining fields from separate eyes results in a vertical or horizontal offset,
the latter manifesting as split or central overlap of images from the two eyes
(CASE 6-1).8 Photophobia has been reported in cases of acute chiasmopathy.9
Ocular motility abnormalities associated with chiasmal pathology include
spasmus nutans in young children10 and seesaw nystagmus, which is a vertical,
pendular dissociated nystagmus during which one eye moves up and intorts
while the other moves down and extorts; this is thought to reflect dysfunction of
the visuovestibular control system, perhaps related to injury to the interstitial
nucleus of Cajal.11 Because of the proximity of the chiasm to the hypothalamus,
pituitary, and cavernous sinuses, lesions in this area can be associated with
endocrine disturbances, ocular motor nerve palsies, and facial numbness.

Examination and Evaluation

Vision loss in chiasmal diseases occurs because of retinal ganglion cell
dysfunction. Accordingly, examination findings can include optic nerve head
pallor in the case of chronic injury, and relative afferent pupillary defect in the
case of asymmetric vision loss. Because the crossing fibers are affected in patients
with a bitemporal hemianopia, the typical optic disc appearance is bowtie
atrophy (in one or both eyes), in which the temporal and nasal sides of the disc
are paler than the superior and inferior poles. OCT can be helpful in detecting
subtle retinal ganglion cell injury that may not affect visual function and in
quantifying chronic visual pathway injury.12 This may have relevance for
prognosis after surgical intervention to remove lesions compressing the chiasm.13

CONTINUUMJOURNAL.COM 1313

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHIASMAL AND POSTCHIASMAL DISEASE

CASE 6-1 A 34-year-old woman presented to her optometrist because of gradual

blurring of vision in the right eye more than the left eye. Based on concern
for optic neuropathy, she was referred for urgent neuro-ophthalmic
consultation.
Visual acuity with correction was 20/100 in the right eye and 20/25 in
the left eye. Color vision was decreased in the right eye. There was a
relative afferent pupillary defect in the right eye. Confrontation visual
fields showed an inability to count fingers in the lower temporal fields of
both eyes. Funduscopic examination showed mild temporal pallor of
both optic nerve heads. Cranial nerves III to XII and the rest of the
neurologic examination were unremarkable.
Optical coherence tomography showed retinal ganglion cell injury in
both eyes, limited to the nasal retina in the left eye and diffuse in the right
(FIGURE 6-3A). MRI of the sella showed a prominent suprasellar lesion
displacing the chiasm upward (FIGURES 6-3B). Endocrine evaluation was
unremarkable.
The patient underwent transsphenoidal resection of the lesion with
decompression of the chiasm (FIGURES 6-3C). Pathology showed a pituitary
adenoma. The postoperative course was complicated by hyponatremia.
One month postoperatively, the patient reported that vision in her
right eye had improved and that she could read with it. She felt that
reading with the left eye was harder than it had been before surgery,
although it remained easier than with the right eye. When she read with
both eyes, she had trouble focusing and noted shifting distance between
the letters. She was coping by covering the right eye, although this made
her notice the visual field loss on the left. Visual acuity was 20/50 with
the right eye and 20/20 with the left eye. There was a relative afferent
pupillary defect in the right eye. She could not count fingers in the
upper or lower temporal field of the right eye or in the lower temporal
field of the left eye. Alternate cover testing revealed an esotropia
with distance or near fixation. Visual field testing showed bitemporal
hemianopia with involvement of the central nasal field of the right
eye (FIGURE 6-4A).
By 8 months after resection, the patient’s vision had improved in both
eyes and the variable letter-spacing symptoms had resolved. She had
stopped closing one eye to control this symptom. She was driving
without difficulty and had returned to work. Visual acuity was 20/40 in
the right eye and 20/20 in the left eye. There was a relative afferent
pupillary defect in the right eye. She had deficits to finger counting in the
upper and lower temporal fields of both eyes and temporal pallor of both
optic nerve heads. Visual field testing showed persistent bitemporal
hemianopia with improvement in the central field of the right eye and
superior fields of both eyes (FIGURE 6-4B).

1314 OCTOBER 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

FIGURE 6-3
Findings of the patient in CASE 6-1. Macular optical coherence tomography (A). Purple circle
indicates the fovea, which is the retinal region where central vision is sensed. Red shading
indicates regions of retinal ganglion cell thinning. Yellow shading indicates regions of
borderline retinal ganglion cell thinning. Images are oriented as viewed by the examiner.
Coronal T2-weighted MRIs show preoperative chiasmal compression (B) and reduced
compression postoperatively (C) (arrows indicate left side of chiasm).

CONTINUED ON
PAGE 1316

CONTINUUMJOURNAL.COM 1315

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHIASMAL AND POSTCHIASMAL DISEASE

CONTINUED FROM
PAGE 1315

FIGURE 6-4
Visual fields of the patient in CASE 6-1. A, Visual fields 1 month postoperatively show
bitemporal hemianopia with involvement of the central nasal field of the right eye. B,
Visual fields 8 months postoperatively show persistent bitemporal hemianopia with
improvement in the central field of the right eye and superior fields of both eyes. Images
are oriented as seen by the patient.

COMMENT This patient had a typical presentation of pituitary adenoma with vague
symptoms of vision loss. The patient did not notice the visual field deficits,
which were complementary (ie, temporal loss in each eye was made up for
by nasal field sparing in the other eye) and, therefore, did not affect
binocular vision. Central vision was decreased in both eyes, and optical
coherence tomography results indicated injury of the retinal ganglion cells
at the time of diagnosis, with nasal ganglion cell injury in the left eye
suggesting chiasmal pathology. After chiasmal decompression, she
experienced a recovery of central vision in the left eye and incomplete
recovery in the right eye. She had persistent bitemporal visual field loss.
After struggling with hemifield slide symptoms in which her esotropia
resulted in a gap between the seeing visual fields of both eyes, her ocular
motility normalized and she was able to function well with both eyes open.

1316 OCTOBER 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

The classic pattern of ganglion cell layer thinning on OCT of the macula is KEY POINTS
binasal, such that identification of this finding in patients with cryptic optic
● The optic chiasm is best
atrophy should prompt imaging of the chiasm. viewed on coronal or sagittal
The chiasm is best visualized on coronal and sagittal neuroimaging obtained MRI or CT sequences
by using narrow spacing between slices. Coronal T2-weighted MRI sequences are obtained with narrow slice
particularly useful because the white CSF outlines the chiasm where it passes spacing.
through the suprasellar area. Pathology is more difficult to detect on axial slices
● Homonymous peripheral
because of the anatomic angle of the chiasm. Thus, specialized sequences beyond vision loss affects
a standard head CT are necessary to exclude chiasmal pathology. navigation, and
homonymous visual field
Common Pathologies loss that reaches central
vision affects reading.
In adults, the most common pathology affecting the chiasm is compression
due to pituitary adenoma,14 craniopharyngioma,15 and meningioma.16 Internal
carotid aneurysms can also compress the chiasm.17 Pituitary apoplexy, caused
by hemorrhage into a pituitary adenoma, is a cause of chiasmal vision loss that
necessitates emergent management. The chiasm can be affected by optic nerve
diseases, such as optic neuritis and optic pathway gliomas, as well as other
inflammation, other tumors, vascular malformations, and infections both
intrinsic and extrinsic to the chiasm. It is worth noting that optic neuritis due to
neuromyelitis optica (NMO) is more likely to affect the optic chiasm than optic
neuritis due to multiple sclerosis or idiopathic optic neuritis.18

COMMON FEATURES OF DISEASES OF THE VISUAL PATHWAY BEHIND

THE CHIASM
Although the regions behind the chiasm contain multiple anatomically distinct
structures, they produce similar visual symptoms and signs since they are part of
the same conduit for visual information from the contralateral hemifield.

Symptoms
Visual symptoms of afferent visual pathway disease behind the chiasm are
related to loss of vision in the same field of both eyes. If the vision loss extends to
the far periphery, patients often have difficulty with awareness of objects that
approach them from the affected side and with navigation. If the vision loss
extends to fixation (ie, central vision), patients often have difficulty reading.
This is because vision used for reading extends to the right and left of fixation to
allow whole-word reading. Patients with a right homonymous hemianopia that
extends to fixation who read from left to right are unable to see entire words
at once and often adapt a letter-by-letter strategy of reading. They are also unable
to locate the end of a line of text. Patients with a left homonymous hemianopia
that extends to fixation who read from left to right usually are less impaired,
although they have difficulty locating the start of lines of text.
Although Charles Bonnet syndrome is classically described in association with
central vision loss from retinal disease, a similar phenomenon of release
hallucinations, which are spontaneous visual perceptions in a blind field, can
occur in unilateral homonymous visual field loss.19 They are typically formed and
nonthreatening, although this is not always the case. Patients may not volunteer
these symptoms for fear that they represent psychiatric disease.
Patients will often attribute homonymous visual field loss to a problem with
the eye ipsilateral to the field loss. Thus, particularly in cases of transient visual
loss, in which the examination will not confirm or refute this impression, it is

CONTINUUMJOURNAL.COM 1317

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHIASMAL AND POSTCHIASMAL DISEASE

important to maintain an open mind regarding reported unilateral vision loss

being due to either one eye or the contralateral brain.

Common Pathologies
In adults, stroke, trauma, and tumors are the most common cause of isolated
unilateral homonymous hemianopias.20 Because these deficits occur on an
anatomic basis, any disease affecting the optic tracts or brain parenchyma is
capable of causing them.

DISEASES OF THE OPTIC TRACT

The optic tract is the first segment in the conduit carrying visual information
from the contralateral hemifield defined by redistribution of the retinal ganglion
cell axons in the chiasm.

Symptoms
Lesions cause contralateral homonymous hemianopias that can be complete or
incomplete. Because of incongruity, this may affect the two eyes asymmetrically.
Optic tract lesions spare central visual acuity, although they can cause substantial
reading impairment if they reach fixation for the reasons discussed above.

Examination and Evaluation

Incomplete homonymous hemianopias from optic tract lesions can be highly
incongruous, meaning the area of visual field loss is different in each eye. Optic
tract diseases affect retinal ganglion cells. Therefore, examination may detect
optic nerve head pallor in chronic lesions. Because of the pattern of ganglion cells
at the optic nerve head, this is characteristically seen as temporal pallor in the
ipsilateral eye and “bowtie” pallor affecting the temporal and nasal sectors but
sparing the inferior and superior sectors in the contralateral eye. The optic tract
contains more fibers from the contralateral eye than the ipsilateral eye, and this
can result in a contralateral relative afferent pupillary defect (CASE 6-2).
Neuroimaging of the optic tract is challenging, with clinical input often
necessary to identify subtle imaging findings.21 Typical OCT findings in chronic
tractopathies include ganglion cell loss in both eyes that is homonymous (ie, on
the same side of both eyes) and ipsilateral to the optic tract lesion.

Causes
Lesions of the optic tract are relatively rare. Many of the diseases that affect the
chiasm can affect the optic tract. This includes compressive lesions in the
suprasellar region and optic nerve diseases, such as optic neuritis and optic
pathway gliomas. The optic tract can become involved in temporal lobe tumors
and their treatment.

DISEASES OF THE LATERAL GENICULATE NUCLEUS

The lateral geniculate nucleus is the first relay in the conduit carrying
contralateral visual field information.

Anatomy
The unique vascular supply to this region results in two unique and highly
localizing visual field patterns. Damage to the region supplied by the lateral
choroidal artery causes a wedge-shaped visual field deficit straddling the

1318 OCTOBER 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

horizontal midline. The region supplied by the anterior choroidal artery is KEY POINTS
complementary to this, consisting of two wedge-shaped visual field regions
● Homonymous visual
immediately adjacent to the upper and lower vertical midlines.22 These patterns field loss with an afferent
of visual field loss are known as sectoranopia. pupillary defect on the same
side of the visual field
Symptoms loss suggests a contralateral
optic tract lesion.
Lesions of the lateral geniculate nucleus cause contralateral homonymous
hemianopias that can be complete or incomplete. Because of incongruity ● Visual symptoms due to
(differences in area of field loss between the eyes), symptoms may be perceived optic radiation disease are
predominantly in one eye compared with the other. usually accompanied by
other neurologic symptoms
localizing to the affected
Examination and Evaluation territory.
Like optic tract lesions, lateral geniculate nucleus lesions produce incongruous
homonymous hemianopias. Lateral geniculate nucleus damage can affect ● Congruous homonymous
presynaptic retinal ganglion cells and lead to optic nerve head pallor in a similar visual field loss is a hallmark
of occipital lobe disease.
pattern to optic tract lesions. However, afferent pupillary defects are not seen
because the retinal ganglion cells subserving this reflex do not reach the lateral
geniculate nucleus.

Causes
Lesions of the lateral geniculate nucleus are rare and can occur from any pathology
affecting the thalamus. Of particular note is localizing sectoral visual field deficits
(described above) caused by anterior and posterior circulation ischemic strokes
via disruption of the anterior and lateral choroidal arteries, respectively.

DISEASES OF THE OPTIC RADIATIONS

The optic radiations are the second leg of the contralateral visual field pathway
connecting the lateral geniculate nucleus to the primary visual cortex.

Symptoms
The optic radiations extend diffusely through the white matter of the temporal
and parietal lobes; temporal lobe lesions cause contralateral superior homonymous
visual field loss, and parietal lobe lesions cause contralateral inferior homonymous
visual field loss. The inferior bundle, also known as the Meyer loop, detours into
the anterior temporal lobe before coursing posteriorly to the inferior striate cortex.
Because of the diffuse spread of the optic radiations, the visual symptoms due
to field loss are often incongruous, with one eye affected more than the other,
and are usually accompanied by other neurologic symptoms localizing to the
affected territory. Relevant to this topic are higher-order visual symptoms
related to deficits in visual processing in the temporal (ventral stream) and
parietal (dorsal stream) pathways. For more information on higher-order visual
disturbances, refer to the article “Higher Cortical Visual Disorders” by Sashank
Prasad, MD, and Marc Dinkin, MD, in this issue of Continuum.23

Examination and Evaluation

Visual field testing is typically incongruous (ie, different in both eyes) and does
not necessarily respect the horizontal meridian because this is not an anatomic
boundary. The retinal ganglion cells are not directly affected, and, therefore,
pupillary changes and optic nerve head pallor are not observed unless the patient
has postpapilledema atrophy related to high intracranial pressure caused by the

CONTINUUMJOURNAL.COM 1319

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHIASMAL AND POSTCHIASMAL DISEASE

CASE 6-2 A 35-year-old woman was referred for evaluation of optic nerve head
pallor that had been noted by her optometrist on routine examination for
an updated glasses prescription. She had no visual symptoms.
Visual acuity with correction was 20/20 in each eye. She could not
count fingers in the left upper or lower fields with either eye. Right visual
fields were intact to confrontation in both eyes. She had a left afferent
pupillary defect. There was temporal pallor of the right optic nerve head
and bowtie pallor of the left optic nerve head.
Formal visual field testing showed an incongruous left homonymous
hemianopia (FIGURE 6-5A). Optical coherence tomography showed
temporal retinal thinning in the right eye and nasal retinal thinning in
the left eye (FIGURE 6-5B). MRI showed bilateral polymicrogyria and
schizencephaly in the right temporal lobe with right optic tract atrophy
(FIGURES 6-5C through 6-5E). These findings were felt to be developmental
in origin. On further questioning, the patient reported having seizures as
a child.

COMMENT This case illustrates the features of an optic tract lesion, including
contralateral hemianopia, optic nerve head pallor in both eyes, and
contralateral afferent pupillary defect. The patient likely had minimal
symptoms related to her visual field loss because she had had it her whole
life and had developed adaptive strategies.

1320 OCTOBER 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

FIGURE 6-5
Findings of the patient in CASE 6-2. Visual field testing shows an incongruous left
homonymous hemianopia (A). Images are oriented as seen by the patient. Optical coherence
tomography of the macula (central retina) (B). Purple circle indicates the retinal region
where central vision is sensed (fovea). Red shading indicates regions of retinal ganglion
cell thinning. Yellow shading indicates regions of borderline retinal ganglion cell thinning.
Images are oriented as viewed by the examiner. Axial (C) and coronal (D, E) postcontrast
T1-weighted MRIs show a schizencephalic cleft in the right temporal lobe (C, D, large arrows)
and atrophic right optic tract corresponding to the ganglion cell layer atrophy seen on the
right side of both maculae (E, small arrow).

CONTINUUMJOURNAL.COM 1321

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHIASMAL AND POSTCHIASMAL DISEASE

lesion affecting the optic radiations. Interestingly, OCT testing has highlighted
the potential for retinal ganglion cell thinning from retrogeniculate lesions due to
transsynaptic degeneration.24,25 However, this is not typically severe enough to
manifest as optic nerve head pallor.

Causes
The causes of visual field loss from optic radiation injury are broad, with middle
cerebral artery ischemic stroke, parietal or temporal lobe hemorrhage, and
tumors as common causes. Because of the anterior course of the Meyer loop,
anterior temporal lobectomies performed for epilepsy treatment may be
complicated by a superior quadrantanopia.

DISEASES OF THE OCCIPITAL LOBE

The primary visual cortex in the occipital lobe is the second relay in the conduit
carrying information from the contralateral visual hemifield connecting
elementary visual input to the circuits that process it.

Anatomy
The visual field in the primary visual cortex is arranged with the contralateral
central visual field at the occipital pole and contralateral far peripheral visual
field anteriorly (FIGURE 6-626). Because the temporal visual field in each eye is
larger than the nasal field, the deep occipital cortex represents far temporal
peripheral vision in the contralateral eye without corresponding representation
in the nasal field of the ipsilateral eye. This monocular region of vision is known
as the temporal crescent. Because of secondary contribution to the occipital pole
by branches of the middle cerebral artery, posterior cerebral artery infarcts often
spare the pole with associated sparing of the contralateral central vision (macula).

FIGURE 6-6
Primary visual cortex organization around the calcarine sulcus in the contralateral occipital
lobe. Visual field as seen by a patient (A), represented in the primary visual cortex (B), and
location on MRI (C). The contralateral visual field is distorted, folded on the horizontal
meridian, and inserted into the calcarine sulcus, with resulting representation of central
vision at the occipital pole and peripheral vision anteriorly.
Panels A and B reprinted with permission from Wurtz RH, Kandel ER.26 © 2000 McGraw-Hill Education.

1322 OCTOBER 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

The deep occipital cortex may also be spared by posterior cerebral artery infarcts,
leading to preservation of the temporal crescent. The contralateral visual field is
folded around the calcarine sulcus with the horizontal meridian in the trough and
the vertical meridians along the adjacent superior and inferior gyri.

Symptoms
Congruous (same in both eyes) homonymous hemianopic or quadrantanopic
visual field loss is the norm, and symptoms are often isolated to the visual system.
Interestingly, some patients have no symptoms and are diagnosed on screening
examination. This is more commonly seen in superior quadrant visual loss that
spares the central visual field because vision in that region is used less frequently
in common activities. Some patients with hemianopic loss also fail to notice
their deficit, which may be due to macular and temporal crescent sparing that
mitigates reading and navigation difficulties that might otherwise be caused by
complete homonymous hemianopic deficits. Patients may report a sensation of
movement in the blind field (the Riddoch phenomenon).
If occipital disease is limited to an anterior lesion, patients may only have loss
of the temporal crescent in the contralateral eye without a deficit in the
ipsilateral eye.
Bilateral visual field constriction, sometimes referred to as a keyhole deficit,
can occur in lesions affecting both occipital lobes and sparing the occipital poles.
Loss of the entire lower or upper visual field in both sides of both eyes can occur
with midline lesions that affect both upper or lower banks of the occipital cortex.
In cases of complete vision loss due to disease affecting both occipital lobes,
patients are often agnostic to their visual deficit and confabulate (Anton
syndrome). In these cases, vision loss only becomes apparent based on
observation of function or direct questioning about visual stimuli.
Superior quadrant visual field deficits may be associated with color vision loss in
the ipsilateral inferior field due to involvement of the central color processing
pathways in the inferior occipital lobe or temporal lobe. Alexia without agraphia
can accompany visual field loss due to occipital disease in the dominant
hemisphere that extends to the splenium of the corpus collosum. This results from
an inability to transfer visual information from the seeing field, perceived in the
nondominant hemisphere, to the language centers in the dominant hemisphere.
Positive visual phenomena occur in homonymous visual fields in the case of
occipital lobe seizures, migraine, and release hallucinations. Occipital lobe
seizures typically cause simple hallucinations in the contralateral field but have
also been reported to cause transient hemianopia.27 When associated with a
lesion, there may be interictal visual field loss, but this is not found in
cryptogenic cases.

Examination and Evaluation

Visual field testing shows highly congruous homonymous visual field defects.
Because of the anatomic layout of the visual field in primary visual cortex,
characteristic visual field patterns are seen from lesions in this area, including
quadrantic visual field loss that sharply respects the horizontal meridian, macular
sparing, peripheral temporal field sparing, and its converse, monocular peripheral
temporal field loss. Pupil examination is not affected. Although retinal changes
can occur due to transsynaptic degeneration, this is typically not apparent on the
funduscopic examination and requires OCT for visualization (CASE 6-3).

CONTINUUMJOURNAL.COM 1323

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHIASMAL AND POSTCHIASMAL DISEASE

CASE 6-3 A 57-year-old woman presented for evaluation of visual deficits

associated with a lung adenocarcinoma metastasis to the right occipital
lobe. She reported some fuzziness in the left field but no difficulties with
navigation or reading. Her cancer had been diagnosed 2 years previously
on workup for back pain. Multiple brain metastases were identified on
initial staging workup, including one in the right inferior occipital pole.
She had received stereotactic radiation therapy to the brain lesions as
well as systemic chemotherapy. The right occipital lesion was smaller
than at initial presentation (FIGURE 6-7).
Visual acuity was 20/20 with each eye, and the patient correctly
counted fingers in all peripheral fields with each eye. Funduscopic
evaluation was normal. Formal visual field testing showed a left superior
quadrantanopia, respecting the horizontal and nasal meridians in both
eyes (FIGURE 6-8A). Optical coherence tomography showed inferior
temporal ganglion cell thinning in the right eye and inferior nasal ganglion
cell thinning in the left eye (FIGURE 6-8B).

FIGURE 6-7
Imaging of the patient in CASE 6-3. Coronal
postcontrast T1-weighted MRI 2 years after initial
diagnosis and treatment with stereotactic
radiation and chemotherapy shows an enhancing
right occipital pole lesion inferior to the calcarine
sulcus (arrow).

1324 OCTOBER 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

 FIGURE 6-8
Findings of the patient in CASE 6-3. A, Visual field testing 2 years after diagnosis of a right
occipital pole lesion. Images are oriented as seen by the patient. B, Optical coherence
tomography of the macula 2 years after diagnosis of a right occipital pole lesion. Purple circle
indicates retinal region where central vision is sensed (fovea). Red shading indicates regions
of retinal ganglion cell thinning. Yellow shading indicates regions of borderline retinal
ganglion cell thinning. Images are oriented as viewed by the examiner.

This case illustrates the functional-structural correlation of primary visual COMMENT

cortex lesions. The right occipital pole lesion caused homonymous visual
field loss affecting the horizontal and vertical meridians. Although this was
evident on formal visual field testing, the patient was able to count fingers
in all peripheral fields and had minimal disability from her vision loss
because of sparing of the deeper occipital cortex and corresponding
peripheral visual fields. This reinforces the limited sensitivity of
confrontation visual field testing, particularly when far peripheral vision is
not affected. The optical coherence tomography result is an example of
transsynaptic degeneration of retinal ganglion cells from an acquired
occipital lesion with inferior temporal retinal thinning in the right eye
corresponding to superior nasal visual field loss and inferior nasal retinal
thinning in the left eye corresponding to superior temporal visual field loss.

CONTINUUMJOURNAL.COM 1325

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHIASMAL AND POSTCHIASMAL DISEASE

Causes
Posterior cerebral artery ischemic stroke is a common cause of occipital lobe
visual field loss in the adult population with vascular risk factors. As such, acute
presentations with visual field loss are a medical emergency. As discussed earlier
in this article, these often spare the occipital pole and anterior occipital lobe to
spare central vision and the temporal crescent contralateral to the infarct. These
features limit disability in reading and navigation. Any pathology that involves
the occipital lobes, including hemorrhage, tumors, demyelinating disease, and
posterior reversible encephalopathy syndrome (PRES), can also cause visual field
loss. All of these have characteristic neuroimaging findings that will guide the
differential diagnosis.
Rare causes of occipital cortex damage include posterior cortical atrophy,28,29 a
progressive neurodegenerative disease with multiple different pathologic bases,
and Creutzfeldt-Jakob disease,30 both of which may be accompanied by more
subtle neuroimaging findings (parietooccipital atrophy in the case of posterior
cortical atrophy and cortical ribboning on diffusion-weighted imaging in the case
of Creutzfeldt-Jakob disease).

REHABILITATION
Although the focus on visual field loss is often on diagnosis, characterization of
deficits and inquiring of symptoms in patients with lesions involving the visual
pathway are critically important to assess disability and guide rehabilitation.
Prominent nonvisual symptoms may lead to a delay in diagnosis of vision-
related disability.20
Therapies for visual field loss purport to restitute damaged tissue, teach
compensation, or substitute the intact visual field. Although a 2011 Cochrane
Review found that evidence for efficacy was limited and insufficient for many
interventions,31 given the low cost and minimal potential harm of many of these,
consideration remains important. Low-vision specialists, institutes for the blind,
and state departments of rehabilitation often have extensive resources that are
available to patients with visual disability due to neurologic disease. The reader is
referred to the excellent review by Agarwal and Kedar32 for treatment options,
some of which are briefly discussed below.

Orientation Support
Many states have a visual field restriction for legal driving.33 For patients who do
meet visual criteria for driving in their state, assessment by a driver rehabilitation
specialist or specialized occupational therapist is helpful for assessing safety and
providing training.
Wayfinding training offered by low-vision optometrists can guide
compensatory strategies with regard to navigation. Peli prism lenses, consisting
of small prisms placed on glasses in the seeing field, are an optical method to
redirect a portion of the blind field into the seeing field. This is a substitution
technique that should be considered for the motivated patient.34 Compensatory
saccadic training may also be helpful.35

Reading Support
For reading difficulties, using a bright marker to indicate the margin on the side
of the visual field loss and using a straight edge to help follow lines are relatively
simple strategies that can be helpful. Saccadic training, in which the patient

1326 OCTOBER 2019

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

learns to make predictive saccades in the direction of the text, can mitigate KEY POINTS
letter-by-letter reading in patients with hemianopic dyslexia.36 Scrolling text,
● Posterior cerebral artery
which moves text into the seeing field, may also be helpful. Alternative strategies infarcts often spare central
for gaining visual information (eg, books on tape, text recognition) should be vision and far peripheral
explored. vision in the affected field,
which can limit disability
from vision loss.
Cortical Visual Prostheses
For visual field loss due to injury anterior to the occipital lobe, electrical ● Posterior cortical atrophy
stimulation of the occipital lobe based on the visual scene in front of the and Creutzfeldt-Jakob
patient is a potential solution to restore vision in the blind field.37 Active clinical disease can cause
trials of implanted cortical visual prostheses are ongoing in the United States homonymous visual field
loss with only subtle
and abroad. neuroimaging findings.

CONCLUSION
Visual field testing guides localization of diseases affecting the chiasmal and
retrochiasmal visual pathways. Beyond their diagnostic role, visual symptoms are
important as a cause of disability in affected individuals. Various strategies may
help to rehabilitate navigational and reading impairments due to homonymous
visual field loss.

REFERENCES

1 Sherbondy AJ, Dougherty RF, Napel S, Wandell
BA. Identifying the human optic radiation using
diffusion imaging and fiber tractography. J Vision
2008;8(10):12. doi:10.1167/8.10.12.
2 Kerr NM, Chew SS, Eady EK, et al. Diagnostic
accuracy of confrontation visual field tests.
Neurology 2010;74(15):1184–1190. doi:10.1212/
WNL.0b013e3181d90017.
3 Stewart JF. Automated perimetry and
malingerers. Can the Humphrey be outwitted?
Ophthalmology 1995;102(1):27–32. doi:10.1016/
S0161-6420(95)31059-7.
4 Maldonado RS, Mettu P, El-Dairi M, Bhatti MT.
The application of optical coherence
tomography in neurologic diseases. Neurol
Clin Pract 2015;5(5):460–469. doi:10.1212/
CPJ.0000000000000187.
5 Kanamori A, Nakamura M, Yamada Y, Negi A.
Longitudinal study of retinal nerve fiber layer
thickness and ganglion cell complex in traumatic
optic neuropathy. Arch Ophthalmol 2012;130(8):
1067–1069. doi:10.1001/archophthalmol.2012.470.
6 Kardon R, Kawasaki A, Miller NR. Origin of the
relative afferent pupillary defect in optic tract
lesions. Ophthalmology 2006;113(8):1345–1353.
doi:10.1016/j.ophtha.2006.02.055.
7 Kirkham TH. The ocular symptomatology of
pituitary tumours. Proc R Soc Med 1972;65(6):
517–518.
8 Peli E, Satgunam P. Bitemporal hemianopia; its
unique binocular complexities and a novel
remedy. Ophthalmic Physiol Opt 2014;34(2):
233–242. doi:10.1111/opo.12118.
9 Kawasaki A, Purvin VA. Photophobia as the
presenting visual symptom of chiasmal
compression. J Neuroophthalmol 2002;22(1):3–8.
doi:10.1097/00041327-200203000-00002.
10 Kiblinger GD, Wallace BS, Hines M, Siatkowski
RM. Spasmus nutans-like nystagmus is often
associated with underlying ocular, intracranial, or
systemic abnormalities. J Neuroophthalmol
2007;27(2):118–122. doi:10.1097/WNO.
0b013e318067b59f.
11 Daroff RB. See-saw nystagmus. Neurology 1965;
15:874–877. doi:10.1212/WNL.15.9.874.
12 Phal PM, Steward C, Nichols AD, et al. Assessment
of optic pathway structure and function in
patients with compression of the optic chiasm: a
correlation with optical coherence tomography.
Invest Ophthalmol Vis Sci 2016;57(8):3884–3890.
doi:10.1167/iovs.15-18734.
13 Moon CH, Hwang SC, Kim BT, et al. Visual
prognostic value of optical coherence
tomography and photopic negative response in
chiasmal compression. Invest Ophthalmol Vis Sci
2011;52(11):8527–8533. doi:10.1167/iovs.11-8034.
14 Molitch ME. Diagnosis and treatment of pituitary
adenomas: a review. JAMA 2017;317(5):516–524.
doi:10.1001/jama.2016.19699.

CONTINUUMJOURNAL.COM 1327

Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.

CHIASMAL AND POSTCHIASMAL DISEASE
15 Fernandez-Miranda JC, Gardner PA, Snyderman
CH, et al. Craniopharyngioma: a pathologic,
clinical, and surgical review. Head Neck 2012;
34(7):1036–1044. doi:10.1002/hed.21771.
16 Mendenhall WM, Friedman WA, Amdur RJ, Foote
KD. Management of benign skull base
meningiomas: a review. Skull Base 2004;14(1):
53–60; discussion 61. doi:10.1055/s-2004-821364.
17 Purvin VA. Neuro-ophthalmic aspects of
aneurysms. Int Ophthalmol Clin 2009;49(3):
119–132. doi:10.1097/IIO.0b013e3181a8d586.
18 Storoni M, Davagnanam I, Radon M, et al.
Distinguishing optic neuritis in neuromyelitis
optica spectrum disease from multiple sclerosis:
a novel magnetic resonance imaging scoring
system. J Neuroophthalmol 2013;33(2):123–127.
doi:10.1097/WNO.0b013e318283c3ed.
19 Vaphiades MS, Celesia GG, Brigell MG. Positive
spontaneous visual phenomena limited to the
hemianopic field in lesions of central visual
pathways. Neurology 1996;47(2):408–417.
doi:10.1212/WNL.47.2.408.
20 Zhang X, Kedar S, Lynn MJ, et al. Homonymous
hemianopias: clinical-anatomic correlations in
904 cases. Neurology 2006;66(6):906–910.
doi:10.1212/01.wnl.0000203913.12088.93.
21 Kowal KM, Rivas Rodriguez FF, Srinivasan A,
Trobe JD. Spectrum of magnetic resonance
imaging features in unilateral optic tract
dysfunction. J Neuroophthalmol 2017;37(1):17–23.
doi:10.1097/WNO.0000000000000411.
22 Luco C, Hoppe A, Schweitzer M, et al. Visual field
defects in vascular lesions of the lateral
geniculate body. J Neurol Neurosurg Psychiatry
1992;55(1):12–15. doi:10.1136/jnnp.55.1.12.
23 Prasad S, Dinkin M. Higher cortical visual
disorders. Continuum (Minneap Minn) 2019;
25(5, Neuro-ophthalmology):1329–1361.
24 Dinkin M. Trans-synaptic retrograde degeneration
in the human visual system: slow, silent, and
real. Curr Neurol Neurosci Rep 2017;17(2):16.
doi:10.1007/s11910-017-0725-2.
25 Jindahra P, Petrie A, Plant GT. The time course of
retrograde trans-synaptic degeneration
following occipital lobe damage in humans. Brain
2012;135(pt 2):534–541. doi:10.1093/brain/awr324.
26 Wurtz RH, Kandel ER. Central visual pathways. In:
Kandel ER, Schwartz JH, Jessell TM, editors.
Principles of neural science. 4th ed. New York,
NY: McGraw-Hill Education, 2000:523–547.
1328
Copyright © American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
27 Adcock JE, Panayiotopoulos CP. Occipital lobe
seizures and epilepsies. J Clin Neurophysiol
2012;29(5):397–407. doi:10.1097/WNP.
0b013e31826c98fe.
28 Pelak VS, Smyth SF, Boyer PJ, Filley CM.
Computerized visual field defects in posterior
cortical atrophy. Neurology 2011;77(24):2119–2122.
doi:10.1212/WNL.0b013e31823e9f2a.
29 Crutch SJ, Schott JM, Rabinovici GD, et al.
Consensus classification of posterior cortical
atrophy. Alzheimers Dement 2017;13(8):870–884.
doi:10.1016/j.jalz.2017.01.014.
30 Kropp S, Schulz-Schaeffer WJ, Finkenstaedt M,
et al. The Heidenhain variant of Creutzfeldt-
Jakob disease. Arch Neurol 1999;56:55–61.
doi:10.1001/archneur.56.1.55.
31 Pollock A, Hazelton C, Henderson CA, et al.
Interventions for visual field defects in patients
with stroke. Cochrane Database Syst Rev
2011;(10):CD008388. doi:10.1002/14651858.
CD008388.pub2.
32 Agarwal A, Kedar S. Prognosis and treatment of
visual field defects. Semin Neurol 2015;35(5):
549–556. doi:10.1055/s-0035-1563573.
33 Prevent Blindness. State vision screening and
standards for license to drive. Prevent Blindness
web site. lowvision.preventblindness.org/2003/
06/06/state-vision-screening-and-standards-
for-license-to-drive/. Accessed August 12, 2019.
34 Bowers AR, Keeney K, Peli E. Community-based
trial of a peripheral prism visual field expansion
device for hemianopia. Arch Ophthalmol 2008;
126(5):657–664. doi:10.1001/archopht.126.5.657.
35 Roth T, Sokolov AN, Messias A, et al. Comparing
explorative saccade and flicker training in
hemianopia: a randomized controlled study.
Neurology 2009;72(4):324–331. doi:10.1212/01.
wnl.0000341276.65721.f2.
36 Schuett S. The rehabilitation of hemianopic
dyslexia. Nat Rev Neurol 2009;5(8):427–437.
doi:10.1038/nrneurol.2009.97.
37 Niketeghad S, Pouratian N. Brain machine
interfaces for vision restoration: the current
state of cortical visual prosthetics.
Neurotherapeutics 2018;16(1):134–143.
doi:10.1007/s13311-018-0660-1.
OCTOBER 2019