Brit. 7. Ophthal. (1975) 59, 332

Cortical blindness in cranial arteritis
I. H. CHISHOLM

From Moorfields Eye Hospital, City Road, London ECIV 2PD

Visual symptoms are reported in up to 65 per cent of
patients suffering from cranial arteritis (Crompton,
1959; Meadows, I966; Wagener and Hollenhorst,
1958). These include transient or permanent blindness, field defects, ophthalmoplegia (diplopia, ptosis)
(Whitfield, Bateman, and Cooke, I963), and formed
visual hallucinations (Hart, I967). Ischaemia is the
underlying mechnism and may affect any part of the
visual pathway although visual loss is usually caused
by disease in the territory of the ophthalmic artery
affecting the retinal or choroidal circulations.
Wilkinson and Russell (1972) found that the ophthalmic artery was involved in 76 per cent (I 3 out of
17) of the arteries examined in I2 necropsy studies.
From this study it was also apparent that it was the
ophthalmic and posterior ciliary arteries that were
chiefly involved rather than the central retinal artery.
The proximal part of this artery was more severely
involved than the distal where it was buried in the
substance of the optic nerve, and this appeared to
correlate with the amount of elastic tissue in the
vessel wall. This further supports the concept of the
disease being an autoimmune reaction involving
arterial elastic tissue.
In a case reported by Symonds and Mackenzie
(I957) bilateral blindness was associated with normal
pupil responses, a fact which allowed the mistaken
diagnosis of hysteria to be made initially. The present
case, the full pathology of which was presented by
Wilkinson and Russell (1972), emphasizes the possibility of cortical blindness in this disease and its
clinical recognition.
Case report
A previously active 79-year-old man presented in Casualty
at Moorfields Eye Hospital with recent onset of visual and
mental deterioration. He had been unsteady on his feet
and generally unwell for about 2 weeks. His vision was
perception of light in each eye but his pupil responses were
normal with no afferent defect. The right fundus was
normal but detailed examination of the left fundus was
Address for reprints: as above c/o P. Cleary, FRCS

impossible because of lens opacities. His erythrocyte sedimentation rate was 58 mm/hr. A visually evoked response
using a computer of average transients (CAT Mnemetron)
(Fig. i) was performed at that time showing a flat response,
and a presumptive diagnosis of cortical blindness caused
by cranial arteritis was made and later confirmed by a positive temporal artery biopsy. The patient was admitted to
the National Hospital, Queen Square, where, despite treatment, he died five days later.

Right
15 pv

Left

Normal

250 msec

FIG. I Tracing of visually evoked responses and a normal
tracing for comparison (CAT Mnemetron)
The necropsy findings were of thrombosis and arteritic
involvement of both vertebral arteries with recent blood
clot extending to the origin of the basilar artery. The terminal parts of the carotid arteries were healthy. Examination
of both the right and left ophthalmic arteries showed some
areas of arteriosclerosis but no evidence of arteritis in either
the ophthalmic or ciliary arteries. Both cerebellar hemispheres showed infarction and softening. The right occipital lobe showed an old infarct involving the optic
radiation while on the left there was more recent and extensive softening involving the upper and lower lips of the
calcarine fissure extending to the posterior pole (Fig. 2).
These necropsy findings failed to show any evidence of
cranial arteritis in the territory of the ophthalmic artery
and fully supported the clinical diagnosis of cortical blindness.

Cortical blindness in cranial arteritis 333

FIG. 2 Brain section to show old infarct in right optic radiation and more extensive and recent softening in region of left

calcarine fissure (section looking posteriorly)

Summary

nizing this presentation of cranial arteritis and initiating immediate treatment.

A case is presented of cortical blindness in cranial
arteritis. A clinical diagnosis at the time of presentation was based on the integrity of the pupillary light
reflex and the absence of visually evoked responses.
Pathological examination confirmed bilateral occipital infarction. This report stresses the need for recog-

I should like to thank Mr M. D. Sanders for his help in
preparing this paper, and Mr P. Fells and Dr K. W. Ross
Russell for permission to report on this patient. I am
grateful to Professor Blackwood of the Department of
Neuropathology, the National Hospital for Nervous Diseases, for the necropsy report and photograph.

References
CROMPTON, M. R. (1959) Brain, 82, 377
HART, C. T. (I967) Brit. med. J., 3, 643

MEADOWS, S. P. (i966) Proc. roy. Soc. Med., 59, 329
SYMONDS, C., and MACKENZIE, I. (1957) Brain, 80 415
WAGENER, H. P., and HOLLENHORST, R. W. (I958) Amer. J. Ophthal., 45, 6I7
WHITFIELD, A. G. W., BATEMAN, M., and cooKE, W. T. (I963) Brit. j. Ophthal., 47, 555
WILKINSON, I. M. S., and RUSSELL, R. W. ROSS (1972) Arch. Neurol. (Chicago), 27, 378