OPHTHALMOLOGY - pmj.bmj.com · July 1950 LINDSAY-REA: Neuro-Ophthalmology 385 'i OPAQUE NERVE FIBRES FIG. 4.-Thispeculiarappearance, whichinmanycases stronglysuggests apapilloedema,

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PAPILLOEDEMAFIG. I.-Papilloedema or 'choked disc' due to raised intracranial tension. In this

instance the papilloedema was due to a frontal lobe tumour.

NEURO - OPHTHALMOLOGYBy ROBERT LINDSAY-REA, M.D., M.CH., F.R.C.S.

Neuro-ophthalmology is essentially a post-graduate study, which not only presupposes aknowledge of neurology, neuro-surgery and oph-thalmology, but also of many other branches ofmedicine. For instance, consider a patient suffer-ing from severe headaches, who has developed asquint and changes in the optic disc, followed byblindness of one or both eyes with the Wasser-mann reaction of the blood positive. The neuro-ophthalmologist, aided by his opthalmoscope, hasnot much difficulty in giving his opinion that the

diagnosis is a syphilitic condition at the base of thebrain.The ophthalmoscope may be looked upon as the

special instrument of the neuro-ophthalmologist.In this article some of the common conditions of

the fundus which will meet the eye of the neuro-ophthalmologist are described.

Plate i is that of papilloedema of the optic nerve.It is a photograph which has been coloured. Suchan appearance should be easily familiar to medicalpractitioners and neurological students, but the

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July 1950 LINDSAY-REA: Neuro-Ophthalmology 383

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FIG. 2.-On the right is the optic disc with some choroidal pigment around its edge.The surface of the optic papilla is paler in colour than the surrounding retina.In the centre of the papilla is the normal physiological pit which is much paler incolour than the surrounding surface and, at the bottom of the papilla, the laminacribrosa is faintly seen. The arteries are lighter in colour and are seen emergingfrom the depths of the physiological pit. The veins are darker in colour. To theleft is the normally pigmented macula. Note that where the arteries cross theveins there is no kinking.

beginner, at first glance, is confounded by thechanged aspect of the disc. If a vessel is followedto its source, it will be noticed how it disappearsand then briefly appears again at its termination.The inflammatory oedema around the disc is re-sponsible for this disappearance of the vessels.Now compare Plates i and 2. The latter is theophthalmoscopic appearance of the normal fundusoculi. The edge of the disc is well defined, notalways showing the black choroidal pigment. Thedisc surface is not so deeply pink in colour as thesurrounding retina. The veins are of larger calibrethan the arteries, and the physiological pitting atthe centre of the disc may be quite marked, oftenshowing fine markings at its depth, which are the

fibres of the sclera called the lamina cribrosa. Thevessels do not stand away prominently from thesurface of the disc. Refer back to Plate I. Herethe disc edge is indistinct; the veins are engorgedand stand away from the disc surface and thephysiological pit has disappeared. The surface ofthe disc is a deeper colour.To measure the height of these swollen vessels

the observer must relax his accommodation andturn up the convex lenses of the ophthalmoscopeuntil the highest number is found with which thehighest or most protruding vessels are distinctlyseen. Then turn the light on to another part ofthe fundus. The vessels will be found to appearindistinct. Rotate the lenses back to the one with

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384 POSTGRADUATE MEDICAL JOURNAL July 1950

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CONSECUTIVE OPTIC ATROPHY

FIG. 3.-The fundus of a blind woman who suffered from anintracranial tumour (probably a tuberculoma) in childhood,showing clearly the white lines accompanying the vesselsperipheralwards. These-are not seen in primary optic atrophy.

which such a vessel is distinctly seen. Thedifference between these two numbers indicatesthe height of the papilloedema; e.g. if the lightestvessel on the disc is seen sharply with a --6 andthe fundus vessel with a +3, then the height ofthe papilloedema is 3 diopters.The following changes take place in the de-

velopment of papilloedema:--(a) First there is an increase in the calibre of the.

veins and increased redness of the disc with ablurring of the upper and lower nasal margins.

(b) Odema, on and around the nerve head be-gins to appear and the nasal and temporal edges ofthe disc are lost, so that the entire sharp edge ofthe disc has disappeared.

(c) Later there is a decided increase in theelevation of the surface of the disc and the veinscontinue enlarging. Oedema and minute ha.emor-rhages form and the physiological pit is obliterated.

(d) In the most acute stage (Plate I) the pictureis a most puzzling one; portions of the vessels onthis area are seen lying on a whitish backgroundtogether with minute haemorrhages. However bychanging the lenses in the ophthalmoscope from

the highest to the lowest the levels of the variousstructures can be perceived.

Often in the normal eye the veins appear largeand tortuous, but when these are associated withany symptoms of disease (not only of the nervoussystem but also of the body generally), theyshould be carefully kept under observation. Thecondition known as pseudo-neuritis where thereis no swelling of the disc surface, seen mostcommonly in young people, should be kept underobservation for months since some such cases haveturned out to be real papilloedema.

Papilloedema occurs in 8o per cent. of intra-cranial tumours. The depth of the papilloedemavaries from a slight swelling to one of five to sixdiopters. Up to eight diopters has been observed.Subtentorial tumours produce a severe form ofpapilloedema owing to the anatomical structurefound in the posterior portion of the cranium. Inthe lesser degrees of swelling it is difficult todetermine by means of the ophthalmoscopewhether the papilloedema is a passive oedema dueto raised intracranial pressure (pleurocephalicoedema) -or due to inflammatory oedema of the

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July 1950 LINDSAY-REA: Neuro-Ophthalmology 385

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OPAQUE NERVE FIBRESFIG. 4.-This peculiar appearance, which in many cases strongly suggests a papilloedema, is due

to the presence of the myelin sheath of the nerves in the retina which normally ends behindthe lamina cribrosa. Rarely these white patches are seen separated by some distance fromthe optic disc, but when, as in this picture, the white area more or less surrounds the opticedge the retinal vessels are seen to disappear and appear again at the edge of this area,giving the impression that the vessels are obscured by oedematous exudates. The medullarynerve sheath areas are always characterized by brushlike extremities.

nerve head (optic neuritis). In the former theremay be no disturbance of vision; in the latterthere is some loss of function. However, in theformer if the intracranial pressure is not released,the pressure on the nerve head will cause great lossof vision, even complete blindness. Continued pres-sure on the optic nerves brings a changed appear-ance of the disc. There is a decided subsidence ofthe vascularity of the papilloedema and increasingpallor of the disc; the vessels become more normalin calibre or even show a shrunken appearance.

The retinal arteries may show accompanying whitelines due to thickening of the perivascular sheaths.The following statistics are from Marchesani as tothe occurrence of papilloedema:-

Intracranial tumour .. .. 186Lues cerebri .. .. .. 7Tuberculosis cerebri .. .. 2Cerebral abscess .. .. 6Meningitis ...... 3Encephalitis epidemica .. .. ITower skull .... .. 7

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386 POSTGRADUATE MEDICAL JOURNAL July 1950

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FIG. 5.-The optic disc is greyish white with a sharp edge and anormal choroidal ring. Frequently the colour of the disc inprimary optic atrophy is of a marble whiteness. The physio-logical pit is present and the size of the vessels is normal orslightly reduced. The remainder of the fundus appearsnormal. When pigment is present in the retina surroundingthe disc due to inflammatory changes it is never so black ordefinitely outlined as in the normal choroidal pigment.

Hydrocephalus ......Cranial injury ......Aneurysm ...... IHyperpiesis or nephritis 39Polycythaemia 2Leukaemia ILymphogranulomatosis .. Ivon Recklinghausen's disease .. 2Syringomyelia ...... ISinus or orbital disease .. ..Venous thrombosis 2Ocular causes (' ex vacuo ') .. 2Unexplained 1......

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Such an appearance as in Plate 3 is known asconsecutive optic atrophy; this is in contradistinc-tion to secondary optic atrophy, a condition whichfollows optic neuritis or papillitis and is the result

of inflammation of the nerve and retina (neuro-retinitis).

Plate 4 is inserted here to teach the student notto confound the appearance of opaque nervefibres with that of papilloedema-a common mis-take. It should be noted that the opaque nervefibres are most commonly restricted to one portionof the edge of the disc but, as in Plate 4, there maybe greater involvement of the edge of the disc.The vessels dip in and come out of these bundlesand simulate the obscuring of the vessels byoedema; the surface of the optic disc, however, isnot raised. The medullated nerve sheath areas arealways characterized by their brushlike extremities.

Primary optic atrophy (Plate 5) is the termapplied to the condition of the disc when the opticnerve is degenerated. Compare Plate 5 with Plate2. In the latter note all the minute vessels on thesurface of the disc; the former does not show anyvessels on the surface of the optic nerve, The

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Yuly 19510 LINDSAY-REA: Neuro-Ophthalmology 337

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P'ARTIAL PRIMARY OPTIC ATROPIHY TN DISSEMIINATrED SCEI,IROSISFIG. 6.--Compare the pink colour of the inner band

of nerve substance with the pale appearance ofthe outer half. The pallor extends to the edgeof the disc.

normal disc has a soft pink tint which contrastswith the central physiological pit, whereas inprimary atrophy the surface is equally white allover. Bear in mind the following contrasts:-

Primary Optic Atrophy(a) Sharp edges.(b) Physiological pit present.(c) Normal choroidal pigment as in Plate 2.(d) Sheaths of vessels invisible.

Consecutive Optic Atrophy(a) Blurred edges.(b) Physiological pit filled in.(c) Finely scattered irregular pigment.(d) Sheaths of vessels visible as white lines

accompanying the vessels as far as theequator of the eyeball (see Plate 4).

Primary optic atrophy may be produced bylesions of the retina due to disease or poisons; bylesions of the optic disc (as in glaucoma), or of thenerve itself (as in tabes dorsalis); by inflammation,growths or injuries. Disseminated sclerosis isresponsible for more cases of pallor than sinusdisease. However, the pallor of disseminatedsclerosis is practically always on the temporal sideof the disc, involving the papillo-macular bundle.Fractures of the base of the brain often pass

through the optic canal and such a lesion isgenerally followed by primary optic atrophy.

Plate 6 illustrates what is known as partialprimary optic atrophy. The temporal portion(papillo-macular bundle) of the disc has becomepale, whilst the nasal portion is still pink. Housephysicians are often dubious about this conditionbut examination of every case of disseminatedsclerosis will soon convince the observer of itsgenuineness. The writer has seen several cases ofpapillitis or optic neuritis in patients with dis-seminated sclerosis but these form a very smallpercentage of cases seen.The nerve head is affected in the disease known

as neuro-myelitis optica (apparently a close relativeof disseminated sclerosis). One sees a bilateralpapillitis, accompanied by blindness, followed byresolution and leaving behind only a slight pallorof the entire disc surface. The visual function inthis last-mentioned condition may eventually notbe affected or only so for minute perception ofcolours.

AcknowledgmentWe are indebted to Messrs. Wm. Heinemann

(Medical Books) Ltd., for the loan of the platesfor the coloured illustrations taken from theauthor's ' Neuro-Ophthalmology.'

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