19

CHAPTER
Neuro-ophthalmology 815

hypothalamus, passing to the pituitary through the

hypothalamo-hypophyseal portal system:
○ Human growth hormone (HGH or GH) or
somatotropin.
○ Follicle-stimulating hormone (FSH).
○ Luteinizing hormone (LH).
○ Prolactin (PRL).
○ Thyroid-stimulating hormone (TSH). 80%
○ Adrenocorticotrophic hormone (ACTH), which regulates
Prefixed Postfixed
blood cortisol levels.
○ Beta-endorphin.
• The intermediate lobe secretes melanocyte-stimulating
hormone (MSH).
• The posterior pituitary releases antidiuretic hormone
(ADH) and oxytocin.

10% 10%
Pituitary adenomas
Fig. 19.45  Anatomical variations in the position of the
Tumour classification is based on the type of hormone secreted; chiasm
about 25% of primary pituitary tumours do not secrete any hor-
mones and may be asymptomatic, cause hypopituitarism and/or
ophthalmic features. An older classification system divided pitui- of the disc will be involved, resulting in a band or ‘bow
tary tumours into acidophilic, basophilic and chromophobic types tie’-shaped atrophy.
based on their histological staining characteristics, but is now not • Papilloedema is rare.
commonly used. ○ Visual field defects depend on the location and direction
of enlargement of a compressive lesion, as well as the
Ophthalmic features of large adenomas anatomical relationship between the pituitary and chiasm
(Fig. 19.45 and see below). Patients may not present until
Large pituitary lesions may first present to ophthalmologists, often
central vision is affected from pressure on macular fibres.
with vague visual symptoms, and a low threshold should be
○ Lower nasal optic nerve fibres traverse the chiasm
adopted for visual field assessment in chronic headache of any
inferiorly and anteriorly, hence the upper temporal
sort. It is also important to perform a careful visual field assess-
quadrants of both visual fields are affected first by most
ment on both eyes in patients with unexplained unilateral central
expanding pituitary lesions, giving a bitemporal superior
visual impairment.
quadrantanopia progressing to the classic chiasmal visual
• Symptoms field lesion, a bitemporal hemianopia (Fig. 19.46). Field
○ Headache may be prominent due to local effects but does
not have the usual features associated with raised ICP,
and diagnostic delay is therefore common.
○ Visual symptoms may be vague; they usually have a LE RE
gradual onset and may not be noticed by the patient until
Hand movement
well established.
• Colour desaturation across the vertical midline of the Counting fingers
uniocular visual field is an early sign of chiasmal
compression.
○ The patient is asked to compare the colour and intensity
of a red pin or pen top as it is moved from the nasal to
the temporal visual field in each eye.
○ Another technique is to simultaneously present red
targets in precisely symmetrical parts of the temporal and
nasal visual fields, and to ask if the colours appear the Decussating fibres
are most vulnerable
same.
• Optic atrophy is present in approximately 50% of cases with
field defects. When optic atrophy is present the prognosis
for visual recovery after treatment is guarded. When nerve
Fig. 19.46  Typical progression of bitemporal visual field
fibre loss is confined to fibres originating in the nasal retina defects caused by compression of the chiasm from below
(i.e. nasal to the fovea) only the nasal and temporal aspects by a pituitary adenoma. LE = left eye; RE = right eye
816 Chiasm
30
Fig. 19.47  Typically asymmetrical bitemporal hemianopia
loss is commonly asymmetrical between the two eyes
(Fig. 19.47).
○ Upper nasal fibres traverse the chiasm high and
posteriorly and therefore are involved first by a lesion
such as a craniopharyngioma that arises above the chiasm
(Fig. 19.48). If the lower temporal quadrants of the visual
field are affected more profoundly than the upper, a
pituitary adenoma is unlikely.
○ A lesion compressing the anterior chiasm, such as a
pituitary adenoma in conjunction with a postfixed
chiasm, may give rise to a ‘junctional scotoma’ – the
syndrome of a central or paracentral defect on the side of
optic nerve involvement together with a contralateral
superotemporal defect (see Fig. 19.53). The precise
mechanism is disputed, but is commonly attributed to
simultaneous involvement of the fibres of one of the
optic nerves together with contralateral inferonasal fibres
looping forward into the nerve in the putative knee of
Wilbrand.
LE RE
Counting fingers
Hand movements
The posteriorly crossing
fibres are most vulnerable
Craniopharyngioma
Fig. 19.48  Progression of bitemporal visual field defects
caused by compression of the chiasm from above by a
craniopharyngioma. LE = left eye; RE = right eye
30
○ Macular fibres decussate throughout the chiasm, but are
concentrated posteriorly. A lesion that preferentially
compresses the posterior chiasm, such as a pituitary
adenoma in conjunction with a prefixed chiasm, may
therefore cause bitemporal hemianopic changes
predominantly affecting the central and paracentral fields.
Predominant involvement of the optic tracts by a
posterior lesion can give a homonymous hemianopia.
○ Extensive loss of the temporal visual field in both eyes
can disrupt sensory fusion, decompensating a phoria and
causing problems with near vision. ‘Postfixation
blindness’ refers to the presence of a non-seeing area
distal to the fixation point due to the overlap of two
blind hemifields (Fig. 19.49); despite the similar
terminology, it is not linked to a postfixed chiasm.
Patients may complain of difficulty with fine close-up
Overlapping
blind fields
Defective Defective
temporal temporal
field of field of
left eye T right eye
M M
Fig. 19.49  Mechanism of postfixation blindness. M = macula;
T = target
(Courtesy of G Liu, N Volpe and S Galetta, from Neuro-Ophthalmology
Diagnosis and Management, Saunders 2010)
 19
CHAPTER
Neuro-ophthalmology 817

tasks such as threading a needle and cutting fingernails,

and near visual acuity measured binocularly may be
worse than when measured with each eye individually.
Double vision may result from slippage of the two fields
with fusional failure (‘hemifield slide’); diplopia may also
result from cranial nerve palsy (see next).
○ Differential diagnosis of bitemporal defects includes
dermatochalasis, tilted discs, optic nerve colobomas, nasal
retinoschisis, nasal retinitis pigmentosa and functional
(‘non-physiological’) visual loss.
• Extraocular muscle paresis due to disruption of the cranial
nerves traversing the cavernous sinus.
• See-saw nystagmus (see later) is a rare feature.

Investigation
• MR with gadolinium contrast (Fig. 19.50) utilizing multiple
planes and thin sections demonstrates the relationship
between a mass lesion and the chiasm, and is usually the
preferred imaging modality. Adenomas are typically
hypointense on T1 and hyperintense on T2 images.
• CT will demonstrate enlargement or erosion of the sella. Fig. 19.51  Facial features in acromegaly
• Endocrinological evaluation is complex, particularly as
combined hormonal over- and under-secretion may be
present, and is usually undertaken by an endocrinologist.
Prolactin-secreting (lactotrophic) adenoma
Lactotrophic adenoma or prolactinoma (formerly known as
chromophobe adenoma, though lesions can be weakly acido-
philic) is the most common pituitary adenoma. Patients are typi-
cally young to middle-aged adults. In women excessive prolactin
secretion leads to the infertility–amenorrhoea–galactorrhoea syn-
drome, and in men may cause hypogonadism, impotence, sterility,
decreased libido, and occasionally gynaecomastia and galactor-
rhoea. Up to 95% remain as microadenomas, though those that
do not may present initially with visual features.

Corticotrophic adenoma
A corticotrophic adenoma (a variant of basophil adenoma)
secretes ACTH and causes Cushing disease (Cushing syndrome
refers to the clinical picture of increased blood cortisol from
A any cause), which may include central obesity and moon face,
cutaneous striae, pigmentation and other features such as
hypertension.

B tongue, coarseness of features with prominent supraorbital ridges
Fig. 19.50  T1-weighted gadolinium-enhanced MR of a
pituitary adenoma. (A) Sagittal and (B) coronal images
(Courtesy of D Thomas)
Somatotrophic adenoma
Somatotrophic (acidophil) tumours secrete excessive GH, causing
acromegaly in adults (once bone elongation has been completed)
and gigantism in children. Presentation is in middle age with
features including enlargement of the head, hands, feet and
and nasiolabial folds (Fig. 19.51), enlargement of the jaw with
dental malocclusion, and hirsutism in females. There are many
complications, including diabetes mellitus, hypertension, cardio-
myopathy and carpal tunnel syndrome.
818 Chiasm

Treatment of pituitary adenomas

• Observation may be appropriate for incidentally discovered
and clinically silent tumours.
• Medical therapy is usually the initial step and consists of the
reduction in tumour size and secretion using agents such as
dopamine agonists (e.g. cabergoline and the older
bromocriptine) and somatostatin analogues such as
octreotide, with supplementary hormonal correction as
appropriate.
• Surgery consists of tumour debulking rather than complete
excision and is usually carried out endoscopically via a
trans-sphenoidal approach through a gum incision behind
the upper lip. Indications include the failure or intolerance
of medical management and sometimes decompression for
acute visual loss (see below). Visual field improvement is
fastest in the earliest weeks and months following surgery.
• Radiotherapy is rarely employed due to the risk of
complications, but is utilized in some circumstances. Newer
techniques include intensity-modulated radiation therapy Fig. 19.52  Sagittal T1-weighted MR image of a
craniopharyngioma with secondary hydrocephalus
and stereotactic radiosurgery.
(Courtesy of K Nischal)
• Monitoring. Long-term ophthalmological review is required,
with serial assessment of visual function.
Meningioma
Pituitary apoplexy Intracranial meningiomas typically affect middle-aged women.
Pituitary apoplexy (PA) is caused by acute haemorrhage into or Visual field defects and clinical signs depend on the location of the
infarction of the pituitary gland, and is usually associated with a tumour (Fig. 19.53).
previously undiagnosed adenoma; Sheehan syndrome is infarction • Tuberculum sellae meningiomas often produce a junctional
of the pituitary usually associated with childbirth and is generally scotoma (see above and Fig. 19.53) due to their location.
regarded as a form of PA. PA typically manifests with the sudden • Sphenoidal ridge tumours compress the optic nerve early if
onset of a severe headache, nausea and vomiting, sometimes with the tumour is located medially and late if the lateral aspect
meningism and occasionally reduced consciousness or stroke. of the sphenoid bone and middle cranial fossa are involved
There is often reduced visual acuity and/or a bitemporal hemiano- (Fig. 19.54A). A classic finding in the latter is fullness in the
pia depending on the anatomical effects of the lesion. Double temporal fossa due to hyperostosis (Fig. 19.54B).
vision due to compromise of the adjacent ocular motor nerves is
common. Acute hormonal insufficiency can lead to life-threatening
L.E. R.E.
complications such as an Addisonian crisis. Investigations include Junctional scotoma
MR, urgent visual field testing, and hormonal assessment. Acute
medical management, including hormone administration and
surgical decompression, may be necessary. Tuberculum sellae meningioma

Craniopharyngioma
Craniopharyngioma is a slow-growing tumour arising from ves-
tigial remnants of the Rathke pouch along the pituitary stalk.
Affected children frequently present with dwarfism, delayed sexual
development and obesity due to interference with hypothalamic
function. Adults usually present with visual impairment.
Visual field defects are complex and may be due to involvement
of the optic nerves, chiasm or tracts.
The initial defect frequently involves both inferotemporal fields Sphenoidal ridge meningioma
because the tumour compresses the chiasm from above and
behind, damaging the upper nasal fibres (see Fig. 19.48). MRI Olfactory groove meningioma
shows a solid tumour that appears isointense on T1 images (Fig. Fig. 19.53  Examples of optic nerve compression by
19.52). Cystic components appear hyperintense on T1 images. meningioma; a junctional scotoma resulting from a
Treatment is mainly surgical, but recurrences are common. tuberculum sellae lesion is also shown
 19
CHAPTER
Neuro-ophthalmology 819

homonymous, in contradistinction to the bitemporal hemianopia

seen in chiasmal compression, which produces heteronymous
loss, with opposite sides of the visual field affected in each eye.

Congruity
A homonymous hemianopia may be incomplete or complete. In
the context of incomplete hemianopia, congruity refers to how
closely the extent and pattern of field loss in one eye matches that
of the other. Almost identical field defects in either eye are
therefore highly congruous, while mismatching right and left
visual field defects are incongruous. Hemianopia secondary to
pathology in the anterior retrochiasmal visual pathways is charac-
teristically incongruous, while that due to pathology further back
A (e.g. the posterior optic radiations) manifests a higher degree of
congruity.

Clinical features
• Homonymous hemianopia
○ The optic tracts arise at the posterior aspect of the chiasm,
diverge and extend posteriorly around the cerebral
peduncles, to terminate in the lateral geniculate bodies.
○ Each optic tract contains crossed fibres from the
contralateral nasal hemiretina, and uncrossed fibres from
the ipsilateral temporal hemiretina.
○ Nerve fibres originating from corresponding retinal
elements are, however, not closely aligned.
○ Homonymous hemianopia caused by optic tract lesions is
therefore characteristically incongruous.
○ Lesions of the lateral geniculate body also produce
asymmetrical hemianopic defects.
○ The causes of optic tract disease are similar to those
affecting the chiasm but the tract is particularly
vulnerable when the chiasm is prefixed (see above).
• Wernicke hemianopic pupil
B ○ The optic tracts contain both visual and pupillomotor
fibres. The visual fibres terminate in the lateral geniculate
Fig. 19.54  Sphenoid ridge meningioma. (A) CT axial image; body but the pupillary fibres leave the optic tract anterior
(B) reactive hyperostosis to the lateral geniculate body, projecting through the
(Courtesy of A Pearson – fig. A)
brachium of the superior colliculus to terminate in the
pretectal nuclei.
• Olfactory groove meningioma may cause loss of the sense of ○ An optic tract lesion may therefore give rise to an afferent
smell, as well as optic nerve compression. pupillary conduction defect.
• Treatment usually consists of surgery, but radiotherapy is ○ Characteristically, the pupillary light reflex will be normal
sometimes required. Observation alone may be adequate. when the unaffected hemiretina is stimulated, and absent
when the involved hemiretina is stimulated (i.e. light is
shone from the hemianopic side).
RETROCHIASMAL PATHWAYS ○ In practice, this Wernicke hemianopic pupillary reaction
is difficult to elicit because of scatter of light within the
Optic tracts eye – a fine beam should be used.
• Optic atrophy may occur when the optic tracts are damaged
because their fibres are the axons of the retinal ganglion
Overview cells. The ipsilateral disc manifests atrophy of the superior
Retrochiasmal pathology results in partial or total binocular visual and inferior aspects of the neuroretinal rim (fibres from the
field defects involving contralateral visual space. This hemianopia temporal retina), while the contralateral disc manifests a
(hemianopsia) involving the same side of the field in both eyes is ‘bow tie’ pattern (nasal and nasal macular fibres).
820 Retrochiasmal Pathways
b. Anterior parietal
radiation lesion
Lateral ventricle
a. Temporal radiation lesion
a cortex
Temporal horn of
lateral ventricle
Lateral geniculate body
Fig. 19.55  Visual field defects caused by lesions of the optic radiations and visual cortex
• Contralateral pyramidal signs may occur when an optic
tract lesion also damages the ipsilateral cerebral peduncle.
Optic radiations
Anatomy
The optic radiations extend from the lateral geniculate body to
the striate cortex, which is located on the medial aspect of the
occipital lobe, above and below the calcarine fissure (Fig. 19.55).
As the radiations pass posteriorly, fibres from corresponding
retinal elements lie progressively closer together. For this reason,
incomplete hemianopia caused by posterior radiation lesions are
more congruous than those involving the anterior radiations.
Because these fibres are third-order neurones that originate in the
lateral geniculate body, lesions of the optic radiations do not
produce optic atrophy. The optic radiations and visual cortex have
a dual blood supply from the middle and posterior cerebral
arteries.
Temporal radiations
• Visual field defect consists of a contralateral superior
homonymous quadrantanopia (‘pie in the sky’), because the
inferior fibres of the optic radiations, which subserve the
upper visual fields, first sweep anteroinferiorly (Meyer loop)
into the temporal lobe around the anterior tip of the
temporal horn of the lateral ventricle (see ‘a’ in Fig. 19.55).
• Associated features are likely to include contralateral
hemisensory disturbance and hemiparesis, because the
temporal radiations pass very close to the sensory and motor
fibres of the internal capsule before passing posteriorly and
rejoining the superior fibres. Other features of temporal lobe
disease include paroxysmal olfactory and gustatory
c. Main radiation lesion
d. Anterior visual
cortex lesion
b
c
d
e. Macular
e lesion
Calcarine fissure
hallucinations (uncinate fits), formed visual hallucinations
and seizures, with receptive dysphasia if the dominant
hemisphere is involved.
Anterior parietal radiations
• Visual field defect consists of a contralateral inferior
homonymous quadrantanopia (‘pie on the floor’) because
the superior fibres of the radiations, which subserve the
inferior visual fields, proceed directly posteriorly through the
parietal lobe to the occipital cortex. However, a lesion
involving only the anterior parietal part of the radiations is
rare. Hemianopia resulting from a parietal lobe lesion tends
to be relatively congruous (see ‘b’ in Fig. 19.55).
• Associated features of dominant parietal lobe disease
include acalculia, agraphia, left–right disorientation and
finger agnosia. Non-dominant lobe lesions may cause
dressing and constitutional apraxia and spatial neglect.
Main radiations
Deep in the parietal lobe, the optic radiations lie just external to
the trigone and the occipital horn of the lateral ventricle. Lesions
in this area usually cause a complete homonymous hemianopia
(see ‘c’ in Fig. 19.55).
• Optokinetic nystagmus (OKN), elicited with a rotating
striped optokinetic drum, may be useful in localizing the
cause of an isolated homonymous hemianopia.
○ Physiological OKN involves smooth pursuit of a target,
followed momentarily by a saccade in the opposite
direction to fixate on the next target.
○ If a homonymous hemianopia is due to a lesion in the
parietal lobe, the smooth pursuit pathways towards the
side of the lesion are likely to be affected, making this
component of OKN defective. OKN will therefore be
asymmetrical: erratic when the drum is rotated towards
 19
CHAPTER
Neuro-ophthalmology 821

the side of the lesion, but regular when the drum is
rotated away from the side of the lesion.
○ If the lesion is in the occipital lobe, the smooth pursuit
pathways are intact and OKN will be symmetrical – this
is the Cogan dictum, which also states that the parietal
lobe lesion is more likely to be a tumour and the occipital
lesion an infarction.
field defects, visual agnosia, alexia and acalculia. The diagnosis is
commonly missed; other degenerative conditions often exhibiting
early occipital cortical involvement include Alzheimer and Creut-
zfeldt-Jakob disease; presentation in the latter can be with acute
visual symptoms only. Electroretinography may be abnormal. In
immunocompromised states, including drug-induced, progres-
sive multifocal leukoencephalopathy can present with isolated
occipital features.

Striate cortex Balint syndrome

Balint syndrome refers to the combination of simultanagnosia
Clinical features (inability to discern the overall impression of an image whilst
• Visual field defects distinguishing individual components), optic ataxia (defective
○ In the striate cortex the peripheral visual fields are visually-directed reaching) and ocular apraxia (impairment of vol-
represented anteriorly. This part of the occipital lobe is untary saccades). It is typically due to parieto-occipital disease,
supplied by a branch of the posterior cerebral artery. with a range of causes reported.
○ Central macular vision is represented posteriorly just
lateral to the tip of the calcarine cortex, an area supplied Posterior reversible encephalopathy syndrome
mainly by a branch of the middle cerebral artery. Posterior reversible encephalopathy syndrome (PRES) or revers-
Occlusion of the posterior cerebral artery will therefore ible posterior leukoencephalopathy syndrome refers to a clinical
tend to produce a macular-sparing congruous presentation thought to result from vascular endothelial dysfunc-
homonymous hemianopia (see ‘d’ in Fig. 19.55). tion seen in patients with malignant hypertension or eclampsia,
○ Damage to the tip of the occipital cortex, as might occur treatment with some drugs (e.g. tacrolimus, ciclosporin) and
from a head injury, tends to give rise to congruous, rarely other associations such as autoimmune disease. Cortical
homonymous, macular defects (see ‘e’ in Fig. 19.55), visual deficits can occasionally be the presenting feature, others
although macular sparing may sometimes occur with including headache and seizures.
vascular lesions of the occipital lobe.
○ The anteriormost part of the calcarine cortex subserves
the temporal extremity of the visual field of the OCULAR MOTOR NERVES
contralateral eye, the area of visual space that extends
beyond the field of binocular single vision and is
perceived monocularly. A lesion in this area may Third nerve
therefore give rise to a monocular temporal field defect in
the contralateral eye, known as a temporal crescent. Nuclear complex
• Associated features of visual cortex disease (cortical
The nuclear complex of the third (oculomotor) nerve is situated
blindness) include formed visual hallucinations, denial of
in the midbrain at the level of the superior colliculus, ventral to
blindness (Anton syndrome) and the Riddoch phenomenon
the Sylvian aqueduct (Fig. 19.56). It is composed of the following
(only moving visual targets perceived).
paired and unpaired subnuclei:
Causes • Levator subnucleus is an unpaired caudal midline structure
that innervates both levator muscles. Lesions confined to
• Stroke in the territory of the posterior cerebral artery is this area will therefore give rise to bilateral ptosis.
responsible for over 90% of homonymous hemianopia with
no other neurological deficit. 8 Orbital
• Other causes include trauma, tumours and rarely migraine;
a range of uncommon inflammatory (including autoimmune 7 Supr. orbital fissure
Internal carotid artery
and infective), degenerative and toxic disorders can also be
causative, but will usually manifest with more widespread Postr. communicating artery
6 Cavernous
Oculomotor nerve (III)
neurological features; imaging and lumbar puncture are Trochlear nerve (IV) 5 Basilar
typically key to diagnosis. Mimicking by retinal disease such Postr. cerebral artery
Supr. cerebellar artery
as acute zonal occult outer retinopathy (AZOOR) may occur. Basilar artery
4 Root
3 Ventral fascicular
Abducent nerve (VI) (Weber)
Benson syndrome 2 Dorsal fascicular
(Benedikt)
1 Nuclear
Benson syndrome (posterior cortical atrophy) is a rare condition
often referred to as a visual variant of Alzheimer disease. Patients
may have normal visual acuity, anterior and posterior segment
examination, but commonly abnormal colour vision, homonymous Fig. 19.56  Dorsal view of the course of the third nerve