Int. Ophthal.

1,1: 9-18, 1978

Ischemic optic neuropathy*

SOHAN SINGH HAYREH

Iowa City, USA

Keywords: Optic nerve, Blood supply, Ischemic optic neuropathy (ION), Anterior ischemic optic neuropathy
(AION), Posterior ischemic ootic neuropathy (PION)

Abstract

Ischemic optic neuropathy (ION), based on vascular anatomy of the optic nerve, pathogenesis and clinical pic-
ture, consists of two distinct entities: anterior (AION) and posterior (PION) ischemic optic neuropathies. AION is
due to interference with posterior ciliary artery supply to the optic nerve head and retrolaminar part of the optic
nerve; it initially presents with visual loss and optic disc edema which progresses to optic atrophy in a month
or two. PION is due to occlusion of nutrient arteries to the posterior part of the optic nerve; in this condition
during the initial stages the optic disc is normal in spite of marked visual loss, but the atrophy develops later on.
Their pathogeneses, causes, clinical pictures, diagnosis and management are discussed briefly.

Ischemic optic neuropathy (ION) is a severe blinding
disease, with sudden onset of blindness at first in one
eye and then after a variable interval usually in the
second eye. The erroneous impression that it is a rare
disease is essentially based on frequent misdiagnosis
and lack of adequate knowledge on the subject. This
paper is primarily designed to create an awareness
of this visually crippling disease, and its early diagnosis
and adequate management.
Blood supply of the optic nerve
To understand the pathogenesis and clinical picture
of ION, it is essential to know the exact arterial blood
supply of the optic nerve. Following is a very brief
account of this. F o r this purpose the optic nerve
can be divided into two parts:
The posterior ciliary arteries (PCAs) (directly or via
the peripapillary choroid) are the only source of
supply to the lamina cribrosa and prelaminar regions
and the main (if not the only) source to the retro-
laminar region, and they may also supply the tempo-
ral part of the surface nerve fiber layer (10, 11, 12,
16) (Fig. 1). The PCAs are therefore extremely im-
portant in the blood supply to the anterior part of
the optic nerve; this is the crucial factor to be recog-
nized in the pathogenesis of acute ischemia of this
part of the nerve. There are usually two or three
PCAs (designated medial and lateral PCAs) arising from
the ophthalmic artery (9). The blood supply to this
region is segmental, producing segmental ischemia
in the event of occlusion of the corresponding PCA
or one of its subdivisions.

Anterior part o f the optic nerve
This consists of the optic nerve head and the retro-
laminar region (i.e., part behind the lamina cribrosa),
* Some of the figures have been reproduced by courtesy of
the British Journal of Ophthalmology (Figs. 2, 5, 7), American
Academy of Ophthalmology and Otolarngology (Fig. t) and
Springer-Verslag (Fig. 8). This investigation was supported by
Public Health Service Grant EY-01151.
10

Fig. 1. Diagrammatic representation of blood supply of the

optic nerve.

Posterior part of the optic nerve

This consists of the rest of the orbital part and

intracanalicular and intracranial parts (Fig. 2). The
blood supply to this region is by the multiple pial
branches derived from various sources, e.g., in the
orbit by the collateral branches of the ophthalmic
artery or its various intraorbital branches (9) (Fig. 1,
2), in the optic canal by branches of the ophthalmic
artery and sometimes from the orbital arteries, and
intracranially by branches of the ophthalmic, anterior
superior hypophysial and other surrounding intracra-
nial arteries (Fig. 2). In addition, in 75% of the
Fig. 2. Schematic representation of peripheral arterial supply
nerves the intraneural part of the central retinal ar- of the optic nerve, as seen from above.
tery gives out 1-8 centrifugal branches within the Abbreviations used in Figs. 1 and 2: A= Araehnoid; Ant.
Sup. Hyp. Art. = Anterior superior hypophyseal artery;
optic nerve (8, 21) (Fig. 1).
C = Choroid; CAR = Central retinal artery; Col. Br. =Collat-
eral branches of the ophthalmic artery and orbital arteries;
CRV = Central retinal vein; CZ = Circle of Zinn and Hailer
Pathogenesis or its substitute; D = Dura; ICA = Internal carotid artery;
LC = Lamina cribrosa; LPCA = Lateral posterior ciliary
artery; Med. Mus. = Medial muscular artery; MPCA = Medial
From this very brief account of the arterial supply posterior ciliary artery; OA = Ophthalmic artery; OD = Optic
of the optic nerve, it is evident that acute ischemia disc; ON = Optic nerve; P = Pia; PCA = Posterior ciliary artery;
PR = Preqaminar region; R = Retina; Rec. Br. C.Z. = Recur-
of the anterior part of the optic nerve develops in rent pial branches of the circle of Zinn and Hailer or its sub-
occlusive disorders of the PCAs or their subdivisions stitute or peripapillary choroid; S = Sclera.
which supply that part of the nerve. This has been
well established by various clinical, histopathological
and experimental studies (13, 16). The blood supply
in the optic nerve head depends upon the perfusion sure or a fall of blood pressure can reduce the perfu-
pressure (mean blood pressure minus intraocular sion pressure below a critical level and induce ION
pressure) in the feeding vessels: thus a temporary, without complete occlusion of the PCAs. Since the
but sufficiently prolonged, rise of intraocular pres- supply by the PCas and their subdivisions is strictly
Ischemic optic neuropathy
Fig. 3. Fluorescein fundus angiogtam during retinal arterio-
venous phase of a 63 year old woman with temporal arteri-
tis, AION, ESR 21 mm/hour and no perception of light,
one day after onset of loss of vision which was preceded
by transient amaurosis for 8 days. It shows normal filling
of the temporal choroid up to the fovea (supplied by the
lateral PCA) but no filling of the optic disc and choroid
nasal to the fovea (supplied by the medial PCA). Superficial
disc capillaries fill from the retinal circulation. Retinal
circulation normal except for a small area of non-filling
temporal to the disc due to non-filling of a small cilio-retinal
artery supplying that area (arrow).
segmental in the optic nerve, the commonest type of
ION involving the anterior part of the optic nerve is
segmental. One of the PCAs (usually the medial PCA)
may supply the entire anterior part of the optic nerve
(Fig. 3), and occlusion of such an artery would pro-
duce complete loss of vision in that eye. The optic
nerve head may be supplied by both medial and lateral
PCAs so that occlusion of one of them produces a
segmental involvement of the optic disc and corre-
sponding visual field defect (Fig. 4, 5).
If the occlusive disorder in~/olves one or the other
of the arteries supplying the posterior part of the
optic nerve, it would produce acute ischemia of the
area of the nerve supplied by the occluded artery
(Fig. 1, 2). This also produces a segmental involve-
ment of the optic nerve.
11
Terminology
A correct terminology to denote a disease is extreme-
ly important for understanding and communication.
Unfortunately acute ischemia of the optic nerve
has been called by all sorts of eponyms (16). The
most popular term in the European literature has
been 'vascular pseudo-papillitis' (6), and in English
'ischemic optic neuropathy'. Based on the pattern
of arterial supply of the optic nerve, it is evident
that acute ischemic disorder of the optic nerve con-
sists of two distinct entities, i.e. anterior and poste-
rior IONs, involving the anterior and posterior parts
of the optic nerve respectively. The anterior ION
(AION) is an occlusive disorder of the PCA, while
the posterior ION (PION) is due to occlusion of one
or more arteries supplying the posterior part of the
nerve. The clinical pictures of the two types of ION
Fig. 4. Fluorescein fundus angiogram during retinal arterio-
venous phase of an 82 year old man with temporal arteritis,
AION, ESR 34 ram/hour, 6/7.5 visual acuity and a tiny
central island visual field 6 days after onset. It shows normal
filling of the lateral PCA distribution which included temporal
half of the choroid up to the temporal edge of the optic
disc, including a small zone of the papillo-macular bundle
in the optic disc (responsible for 6/7.5 visual acuity and
the central island of visual field). Medial PCA did not flU
and involved major part of optic disc. Retinal circulation
normal.
12
Fig. 5. Fluorescein fundus angiorgram during retinal arterial
phase of left eye of a 78 year old man with visual acuity
6/12 in that eye, a negative temporal artery biopsy, ESR
69 mm/hour, bilateral AION and 5 weeks after onset in the
left eye. It shows filling of. the nasal choroid and nasal part
of the optic disc (supplied by the medial PCA) while the
temporal part of the disc and choroid (supplied by the
lateral PCA) did not fill (some fluorescence of the temporal
part of the disc due to previous injection of fluorescein a
few minutes earlier).
are also distinct (see below). Thus, it is important
to recognize the two types of I O N - a fact not fully
appreciated in the past.
Causes
The commonest cause of ION is atherosclerosis and
arteriosclerosis, which explains its most frequent
occurrence in elderly individuals. However, the most
important cause, particularly for the PCA occlusion,
is temporal (or giant cell) arteritis, which occasional-
ly may involve one of the arteries supplying the
posterior part of the nerve. In my experience, dia-
betes mellitus is the next common and important
Sohan Singh Hayreh
cause and may involve not only the elderly and the
middle-aged but even the juvenile d i a b e t i c - a fact not
adequately stressed in the past. Among the less fre-
quent causes are systemic arterial hypertension or
hypotension, collagen vascular diseases, and other
systemic or local diseases associated with microvascu-
lar occlusive disorders. AION may also be due to
ocular causes, e.g., raised intraocular pressure or
other factors producing precipitous fall of perfusion
pressure in the anterior part of the optic nerve,
and lesions compressing the blood vessels to this
part of the nerve. In a number of cases it may not
be possible to be definite about the cause of ION
because of the limitations in the diagnostic tech-
niques.
Clinical picture
ION is most commonly a disease of persons past
middle-age but sometimes does occur in much youn-
ger individuals (occasionally in second or third de-
cades). In the young persons it is usually due to
diabetes mellitus, arterial hypertension, collagen
vascular diseases or other vascular or hematological
disorders. The disease usually starts as a unilateral
condition but after days, months or years frequently
becomes bilateral-the longer the follow-up, the
higher the frequency of bilaterality, so that for prac-
tical purposes it may be considered ultimately a bi-
lateral disease.
The loss of vision in the eye is of a sudden onset
and mostly sectoral although in temporal arteritis
it is frequently total. If this loss of vision is preceded
by attacks of transient blurring or loss of vision, it
is highly suggestive of temporal arteritis as the cause.
The visual acuity may vary from no perception of
light to perfectly normal central vision, being much
worse in temporal arteritis than other type. The most
important and essential method of evaluating the loss
o f vision and diagnosis of ION is meticulous perimetry
because in the vast majority of these eyes optic nerve
related visual field defects are seen irrespective of
the fact whether the central vision is normal or ab-
normal. The commonest visual field defects are infe-
rior altitudinal, inferior nasal and central scotoma;
the other types include segmental defect, nerve fiber
bundle defects, vertical defects and peripheral con-
striction.
Ischemic optic neuropathy 13

On ophthalmoscopy, the appearance of the optic

disc differs in AION and PION. (i) In AION: the optic
disc shows edema during the initial stages of the dis-
ease. In the vast majority the edema is associated
with visual field defect; however, occasionally in very
early and mild cases the visual acuity and fields may
be apparently normal in spite of the presence of op-
tic disc edema (Fig. 6a). In temporal arteritis, about
half the eyes may show chalky-white swelling of the
optic disc with a rare hemorrhage (Fig. 7a). In the
other cases the disc usually shows pink or pale-pink
edema with frequent flame-shaped hemorrhages
at the disc margin and/or adjacent retina (Fig. 6b,
8a). In juvenile diabetics and some of the middle-
aged diabetics, the swollen disc may be covered
with a network of prominent fine vessels, mimicking
optic disc neovascularization (Fig. 9); the latter may
mislead the physician. The optic disc edema usually
starts to subside in about a week or two, completely
disappearing in a month or two when a variable
degree of pallor of the optic disc is seen, depending
upon the severity of AION. The vast majority of
optic discs with AION due to temporal arteritis
develop cupping of the optic disc (Fig. 7b), and this
is u n c o m m o n when AION is due to other causes.
(ii) In PION: the optic disc shows no abnormality
in spite of marked visual field of loss with or without
deterioration of visual a c u i t y - a state of affairs simi-
lar to that of optic neuritis in the posterior part of

Fig. 7. Right eye of a 72 old woman with temporal artefitis,

AION, ESR 52 mm/hour and no perception of light in that
eye preceded by transient amaurosis for one week. (a)
Three days after onset of blindness, showing chalky-white
swelling of the disc, with no hemorrhages. (b) 131/4months
later, showing cupping of the discs and atrophy.

Fig. 6. Fundus photographs of (a) left and (b) right eyes of

a 67 years old woman, with bilateral AION, negative temporal
artery biopsy, ESR 30 turn/hour, sudden deterioration of
vision in the right eye for 2 weeks, visual acuity only percep-
tion light and 6/6 in the right and left eye respectively,
visual fields with Goldmann Perimeter were normal in the left
eye except for an enlarged blind spot. Both optic discs
showed edema and flame-shaped hemorrhages-right disc
somewhat pale, and more swollen than the left disc. On
treatment with systemic corticosteroids the left disc recovered
to normal with normal visual acuity and fields, and the right
became atrophic with a visual acuity 6/90 and normal peri-
pheral fields with a dense central scotoma with V4.
14 Sohan Singh Hayreh

Fig. 8. Right eye of a 61 year old man with right inferior

altitudinal hemianopia of sudden onset 10 days previous-
ly, preceded by attacks of transient amaurosis in that part
for one week, AION involving upper half of the disc, visual
acuity 6/12, no evidence of temporal arteritis and ESR
7 mm/hour. (a) Fundus photograph showing swelling of the
optic disc with hyperemia of the superficial layer and some
flame-shaped hemorrhages below. (b, c) are fluoreseein
angiograms during the retinal arteriovenous and late phases
respectively- (b) shows non-i'filing of superior temporal
peripapillary ehoroid and patchy filling of the upper half
of the choroid; (c) shows diffuse fluorescein staining of the
optic disc with blurred margins.

the nerve. The fundus is normal on ophthalmoscopy.

After a month or two the optic disc develops pallor
which is the only ophthalmoscopic abnormality.
Fluorescein fundus angiographic changes are seen
only during the acute stages of AION and depend
upon the time since onset (14, 16). In complete
AION, the optic disc shows no filling of vessels of
PCA origin during the first, and maybe even second,
week after onset (Fig. 3, 4, 5); some filling is seen
after this. The filling of vessels in the optic disc also
depends upon whether the AION was the result of
transient or permanent i s c h e m i a - t h e former type
being the commonest. The edematous disc stains
with fluorescein during the late stages. In segmental
Ischemic optic neuropathy
Fig. 9. Fundus photographs of left eye of a 20 year old man
with diabetes mellitus since the age of 3 years and on insulin,
was seen after onset of blurred vision in both eyes, with
visual acuity 6/18 and 6/7.5 in the right and left eye respec-
tively and optic disc related visual fields in the left eye in
lower half and a small central scotoma to I_ of Goldmann
perimeter in the fight eye. (a) Left optic ~ s c one month
after onset, showing swelling, abnormally prominent fine
vessels on the disc (like neovascularization), some pefi-
papillary retinal hemorrhages and engorged veins but no
other evidence of diabetic retinopathy in the rest of the
fundus. He was treated with systemic cortico-steroids which
produced resolution of both fundi to normal (b) and normal
visual acuity and visual fields. Now followed for 4 years
with normal optic discs, fundi and vision, and stable diabe-
tes.
15
A I O N , t h e w h o l e disc s h o w s late staining (Fig. 8c),
initially m a r k e d in t h e n o r m a l p a r t o f t h e o p t i c disc.
T h e a t r o p h i c p a r t o f t h e disc s h o w s p o o r or n o fluo-
rescence. In t e m p o r a l arteritis, d u r i n g t h e very early
stages o f A I O N , t h e area o f t h e c h o r o i d a n d o p t i c
disc supplied b y t h e o c c l u d e d P C A does n o t fill
till late (Fig. 3 , 4 ) . In o t h e r cases o f A I O N , d u r i n g t h e
early stages t h e peripapillary c h o r o i d ( w i t h or w i t h -
o u t c h o r o i d a l w a t e r s h e d zones) shows d e l a y e d a n d
p o o r filling (Fig. 8b, 10). A f t e r a few days or weeks,
t h e c h o r o i d a l c i r c u l a t i o n is r e s t o r e d to n o r m a l . T h e
r e t i n a l c i r c u l a t i o n is n o r m a l unless it is o f a cilioretinal
t y p e w h i c h m a y show e v i d e n c e o f o c c l u s i o n d u r i n g
t h e early stages (Fig. 3). In P I O N t h e r e are n o anglo-
graphic a b n o r m a l i t i e s .
T h e rest o f t h e o c u l a r e x a m i n a t i o n usually reveals
n o a b n o r m a l i t y . I n t r a o c u l a r pressure m u s t b e record-
ed in all p a t i e n t s and m a y b e elevated in s o m e eyes.
Fig. 10. Fluorescein fundus angiogram of left eye of a 64
year old man with bare counting finger vision in a small
temporal island, which was plotted with V 4 only of the
Goldmann perimeter, AION of 12 days duration, ESR 5
mm/hour, no evidence of temporal arteritis, swollen optic
disc, and showing f'flling defect in the pefipapillary choroid
and the optic disc. He was treated with systemic corticoster-
oids-the visual acuity recovered to 6/12 - 6/15 and normal
peripheral visual fields with 12 of Goldmann perimeter
and small central scotoma.
16
Apart from the ocular findings there may be systemic
abnormalities indicative of the causative systemic
disease. Thus it is essential to do systemic evaluation
o f the patient to find out the cause o r l O N .
Diagnosis
In a patient with ION, the first important step is
to rule out the presence o f temporal arteritis because
of the potential danger of bilateral total b l i n d n e s s -
observance o f this fact can prevent many tragic blind-
nesses. This can be done by doing an emergency
estimation of the erythrocyte sedimantation rate
(ESR) in these patients. The question of normal
ESR in elderly persons is highly controversial. In m y
series of patients with AION and confirmed temporal
arteritis (on biopsy of the superficial temporal artery)
the ESR has varied from 21 to 135 (mean 85) m m /
hour by the Westergren method although in extremely
rare instances some authors have reported an ESR as
low as 13, 12 and even 6 m m / h o u r (2, 3, 4, 17, 20).
In m y patients with AION and a negative temporal
artery biopsy for temporal arteritis, the ESR varied
from 1 to 115 (mean 22) mm/hour. This indicates
that while elevated ESR is highly suggestive of tem-
poral arteritis, a normal ESR in the elderly does
not rule out temporal arteritis. Small and Gavrilles-
cu (23) showed the presence of serum C-reactive
the ESR has varied from 21 to 135 (mean 85) m m /
hour by the Westergren m e t h o d although in extremely
rare instances some authors have reported an ESR as
low as 13, 12 and even 6 ram/hour (2, 3, 4, 17, 20).
and in assessing the activity of the disease. This
requires further exploration.
Biopsy o f the superficial temporal artery should be
performed in all cases suspected of temporal arteritis
to establish the diagnosis; since temporal arteritis
is in fact a systemic disease, general symptoms
(e.g., malaise, weakness, muscular and joint pains,
anorexia, fever, etc.) and headache, particularly in
the temporal region, may be helpful in suggesting
the disease. The presence of euphoria in these pa-
tients or of the occult variety of the disease may mis-
lead the physician. Recently some investigators
have indicated the presence of normal areas in a
superficial temporal artery otherwise involved by
giant cell arteritis, so that a section through the nor-
Sohan Singh Hayreh
mal segment would give a false negative result (1,
18); in m y series of about a hundred patients with
AION so far I have had no reasons to believe that
there has been a false negative temporal artery
biopsy. As a result of this, I am of the opinion that
in patients with temporal arteritis and AION, the
chances of having a false negative temporal artery
biopsy are extremely small.
It has been suggested by some observers that carotid
angiography should be performed in these patients
(21). I feel there is no need to subject the vast
majority of patients with AION to such a procedure,
because the chances of finding any helpful infor-
mation from it are so small that it does not justify
the hazards involved; it should only be done if there
is an evident carotid artery abnormality clinically.
O p h t h a l m o d y n a m o m e t r y routinely has also been
suggested in these patients (21). It is not only an
unreliable and unreproducible test but also does
not give any useful information in the vast majority
about the state of circulation in the PCAs, because
AION is not due to occlusion of the central retinal
artery but to involvement of the P C A s - a normal
ophthalmic artery pressure does not rule out PCA
and small optic nerve vessel disease and vice versa.
As mentioned earlier, a systemic evaluation of a
patient with ION is extremely important, for both
diagnosis and management of these patients.
Differential diagnosis
It depends upon whether the patient is seen soon
after onset of AION (i.e., with optic disc edema)
or late (i.e., with optic atrophy). If an elderly indivi-
dual is seen with a history of sudden loss of vision
and optic disc related visual field defects with (i)
unilateral optic disc edema, or (ii) optic disc edema
in one eye and optic atrophy in the fellow eye, or
(ii) bilateral optic atrophy, the diagnosis is AION
unless proved otherwise. A lack of appreciation of
these facts has resulted in not only wrong diagnosis
but also unnecessary, hazardous, time consuming
and expensive neuro-surgical investigations. In diabe-
tics of any age, if the disc is swollen and there are
disc related visual field defects, AION must be con-
sidered as a most probable cause; diabetics get a par-
ticularly rough deal from ophthalmologists because
Ischemic optic neuropathy
any visual loss in them tends to be attributed to dia-
betic retinopathy and the patient tragically may be
treated with panretinal photocoagulation under the
erroneous impression that the prominent vessels
on the optic disc represent neovascularization. In
collagen vascular diseases, a sudden loss of vision
with a normal fundus during the early stages must
be considered due to PION unless proved otherwise.
It is beyond the scope of this paper to go into any
more details on the differential diagnosis of ION.
Treatment
The management of ION can be divided into the fol-
lowing categories:
1. 1ON due to temporal arteritis: This is an ocular
emergency. If in doubt, AION in persons over the age
of 60 years should be regarded as due to temporal
arteritis, particularly if a patient suddenly develops
attacks of transient altitudinal hemianopic amaurosis
or sudden complete blindness in an eye preceded b y
attacks of transient amaurosis. The treatment is to
institue therapy immediately with systemic corticos-
teroids in adequately high doses, e.g., prednisone
80-100 mg or even more daily. The object of the
treatment is to prevent the loss of vision in the
second eye. Occasionally the treatment may produce
some visual recovery which tends to be insignificant.
Dosage and duration of therapy is guided by the ESR
level. These patients require prolonged steroid ther-
apy, usually for years, and suddenly reducing or
stopping the drug prematurely may produce visual
symptoms in the normal eye and a rise of ESR.
2. I O N due to collagen vascular disease: These pa-
tients should be treated by high doses of systemic
corticosteroids during the initial stages of the disease.
In m y experience there is a significant recovery of
visual function in some of these patients after an
early and adequate corticosteroid therapy.
3. A I O N with diabetes mellitus: There is no weU-
established treatment available for this category. In
m y experience so far there has been a significant
recovery of visual function on treatment with high
doses of systemic corticosteroids during the initial
stages of the disease when the disc is edematous.
The steroids aggravate the diabetes and these patients
require very stringent inpatient control o f their dia-
17
betes by a diabetes specialist-without such strict
control this therapy should not be given.
4. A I O N due to causes other than vasculitis: Most of
the patients with AION fall in this category, where,
apart from arteriosclerosis and atherosclerosis, there
is no evident abnormality. Their management is
highly controversial. Most physicians do not treat
them, and dismiss them with a philosophical advice to
accept blindness as an 'act of God'. However, there
are some reports suggesting that systemic corticoster-
oids in high doses during the initial stages of the dis-
ease (i.e., with optic disc edema) have a beneficial
effect in a significant number of these patients
(5, 7, 15, 16).
I would like to emphasize very strongly that if sys-
temic corticosteroid therapy is to show beneficial
effect in categories 2, 3 and 4 above, the treatment
must be instituted as early as possible, while the optic
disc still shows a fair amount o f edema, starting
with at least 80 mg prednisone daily and maintained
on adequate doses (not less than 40 mg. daily) so
long as the disc shows edema (which lasts at the maxi-
mum for 4-8 weeks from its onset). Once the disc
has become atrophic, no treatment is worthwhile.
Since the treatment is given for not more than 4-6
weeks at the maximum, none of the serious side-
effects associated with long-term steroid therapy are
seen in the vast majority except those with diabetes
meUitus (see above). Since the whole of this treat-
ment is somewhat controversial, we are at present
conducting a double masked randomized therapeutic
trial with systemic corticosteroids versus placebo
to evaluate further the role of this therapy in AION.
In addition to this, I feel every attempt should be
made to reduce the intraoccular pressure to as low
a level as possible in patients with AION, with a view
improve perfusion pressure in the optic disc vessels
(Perfusion pressure = Mean Blood pressure minus
intraocular pressure).
Prophylactic measures against ION
Since ION, particularly in temporal arteritis, is a
blinding disease with poor prognosis for recovery
o f vision, and with a high risk of the second eye
being involved after the first, it is worthwhile to con-
sider the possible preventive measures that could
18 Sohan Singh Hayreh

be taken to lower the incidence of blindness due to 19. Lancet. Editorial on 'C-reactive protein or E.S.R.?'
ION. These are discussed at length elsewhere (16). Lancet 2:1166 (1977).
20. Permin, H., F. Juhl, A. Wiik & J. Balslov. Immunoglo-
Briefly these consist of early diagnosis of temporal bniin deposits in the dermo-epidermal junction zones
arteritis, i.e., before ocular symptoms develop, and, in patients with systemic lupus erythematosus.
Rheumatoid arthritis and temporal arteritis compared
if a patient has ION in one eye, therapy should be
by serological testing including a2 -macroglobniin.
started immediately even if the diagnosis of temporal Scand. J. Rheum 6:105-110 (1977).
arteritis is not conclusive. 21. Sanders, M.D.s Neurol. Sci. 31:308 (1977).
22. Singh, S. & R. Dass. The central artery of the retina.
II. A study of its distribution and anastomoses. Br. d.
OphthaL 44: 280-229, (1960).
23. Small, J.M. & K. Gavrilescu. The serum protein changes
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