syphilitic optic neuritis · syphilitic optic neuritis out-patient treatment at his local clinic...

J. Neurol. Neurosurg. Psychiat., 1950, 13, 216.

SYPHILITIC OPTIC NEURITISBY

G. S. GRAVESON

From the Department of Neurology, Manchester Royal Infirmary

The broad division of syphilitic disease of thecentral nervous system into parenchymatous andmeningo-vascular types has proved of considerablevalue, both in understanding the pathologicalprocesses involved in the clinical patterns of diseaseand in estimating their prognosis. Its applicationto syphilitic disease of the optic nerve has beenneglected; for whereas parenchymatous degener-ation manifesting itself as a so-called primary opticatrophy is well known, yet its counterpart-aninflammatory neuritis of the optic nerve-is illdefined in the literature and indeed seldom men-tioned. Partly, no doubt, this is because it is ararer disease and partly, perhaps, because its effectsare less serious and, therefore, have not created asmuch interest. Nevertheless, it is a condition ofsome importance, and its clinical features merit moreattention than has hitherto been given to them. Inthis paper an attempt is made to define the clinicalpicture of syphilitic optic neuritis more sharply onthe basis of a study of five cases seen in the lasttwo years.

On examination his visual acuity was 6/24: 6/6.Both fundi showed identical changes. The optic discwas hyperxmic and its margin was obliterated, the pinkcolour of the disc blending with that of the retina. Theretinal veins were engorged but not obscured by the discchanges. There was no measurable swelling of theoptic nerve head. The appearances were those of apapillitis. The fields of vision showed a central scotomato a 3 mm. white object in both eyes, a complete ringscotoma to a 10 mm. white object in the right eye, and apartial ring scotoma in the left eye. Coloured targetswere not seen at all and further testing revealed that hehad become, in fact, completely colour-blind. Botheye-balls were tender on pressure over their superiorpoles.

Neurological examination was otherwise normal andthere were no cardiovascular changes. The bloodWassermann reaction was positive 1 in 8. The cerebro-spinal fluid was normal in composition (1 cell per c.mm.;35 mg. protein per 100 ml.; globulin, no increase;Lange curve 0000000000); the Wassermann reactionwas negative.A diagnosis of syphilitic retrobulbar neuritis was

made and the patient was discharged from hospital and

Case ReportsCase No. 1.- A. R., a policeman, aged

37, was admitted to hospital in January,1948, complaiaing of defective vision.He had first noticed a loss of acuity inthe right eye in October, 1947. Whilstplaying snooker one day he discoveredthat he was unable to see clearly theball he was aiming to hit. Gradually,from this time onward, his distancevision became impaired and within amonth near vision was also involved, sothat he had difficulty in reading hiswriting with this eye. Between Novem-ber, 1947, and January, 1948, nofurther deterioration occurred. Visionin the left eye was affected about threeweeks after the onset in the right eye.There was a very slight deterioration indistance vision which had not pro-gressed after the first month. He hadhad no ocular pain or headache.

FIG. 1.-Case 1. (May, 1948) V.A. 6/18, 6/6. White targets. R. eye:enlarged blind spot, two nerve bundle scotomata and a smallparacentral scotoma to 2/2000. 1 mm. target not seen. L. eye:enlarged blind spot; constriction of 1/2000 isopter.

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SYPHILITIC OPTIC NEURITIS

out-patient treatment at his local clinic arranged. Aftertwo weeks preliminary treatment with bismuth (total6 ml. " stabismol ") he was given 4 mega units ofpenicillin, followed by a two months' course of intra-venous arsenic (5.05 g.) and intramuscular bismuth(15 ml. " stabismol ").

FIG. 2.-Ci se 1. (August, 1949) V.A. 6/6, 6/6. WR. eye: reduction in size of blind spot and nerve bund]1/2000 isopter present but constricted. L. eye:smaller; expansion of 1/2000 isopter.

In May, 1948, his visual acuity had improved to 6/18:6/5 and the bilateral papillitis had subsided. There wasa slight pallor of the discs and their edges were a littleindistinct. In the right fundus there was a small patchof choroiditis just below the macula which was respon-sible for a distortion of vision noticed just above thefixation point; a vertical straight line appeared wavy atthis point. The fields of vision had also improved(Fig. 1). The blind spot was enlarged in each eye.The right field showed a partial ring scotoma due to twonerve bundle defects, and also a paracentral scotomacorresponding to the patch of choroid-itis. The 2/2000 isopter was constricted U" s 6/12and the 1 mm. target not seen at all. Rkwm 0S -Colour vision had returned in the V5central part of the lower nasal quad-rant, a 20 mm. red target being visible /here. The left field showed only aconstriction of the 1/2000 and 2/2000isopters. The blood Wassermann '-'v1

reactionwa spositiv eI in 4. A furthercourse of bismuth and arsenic was given(30 ml. " acetylarsan " + 10 ml."stabismol").

In September, 1948, visual acuity was6/9: 6/6. The disc margins were stillindistinct, due to connective tissueovergrowth, but no cedema was present.The patch of choroiditis in the rightmacular area had become more pig- FIG. 3.-Camented. The right field of vision had enlargeimproved. The partial ring scotoma scotomwas smaller and the 1 mm. target was concen

now seen for 20 around the fixation point. The leftfield was unchanged.One further course of arsenic (30 ml. " acetylarsan ")

and one of bismuth (20 ml. " stabismol ") were givenand the patient was seen again in May, 1949. He wasthen very well, his only symptom being the distortion of

vision in the right eye. Visual acuity_="*"\QJ/h was 6/6: 6/6. The discs were normalR=ac°o- in colour, but the edges were blurredComc VOD-

and the physiological cup was filled.K i'. The patch of choroiditis in the right

fundus was densely pigmented andappeared to be healed. The fields ofvision (Fig. 2) showed an expansion ofthe 1/2000 isopter to about 10° in eacheye. The scotomata on the right sidewere unchanged. The red fields had

/ ; / ! j expanded though they were still con-" !}/,siderably constricted. The blood

/,5* Wassermann reaction was now negative./</// It was impossible to be sure of the

date of this patient's primary infection.He had been married ten years andhis wife was found to have a positive

hite targets. blood Wassermann reaction. He deniedle scotomata. extramarital exposure to infection afterblind spot his marriage. It seems most likely that

he acquired his disease before then andtransmitted it later to his wife. His

optic neuritis would, therefore, be a manifestationof the tertiary stage.

Case No. 2.-James H., aged 33 years, was first seenin March, 1948. A month previously he had awakenedone day to find that vision in his left eye was blurred.There was no accompanying headache or ocular pain.The blurring remained unchanged for two weeks andthen improved a little.On examination his corrected acuity was 6/6: 6/12.

Fundal examination revealed a bilateral papillitis. The

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se 2. (March, 1948) V.A. 6/6, 6/12. White targets. R. eye:d blind spot. L. eye: enlarged blind spot; broad ringia to 1/2000, paracentral scotoma to 2/2000, slightitric constriction of 1-5/2000 isopters.

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G. S. GRAVESONdiscs were hyperamic and the edges blurred thoughtheir position could be identified. The retinal veinswere slightly engorged but there were no hemorrhagesor exudate. The right field of vision (Fig. 3) showed anenlarged blind spot and a slight contrac-tion of the 1/2000 white isopter. The u.6weVQS%

R Q*S. -left field revealed a broad ring scotoma Q vos-to 1/2000 and a small paracentralscotoma to 2/2000. The blind spot onthis side was also enlarged. The5/2000 and 10/2000 red isopters wereconsiderably contracted, whilst a 5 mm.green target was not seen with eithereye. The 10 mm. green target was notseen on the left and only within 5°on the iight. Physical examinationwas otherwise normal.The Wassermann reaction was

positive in the blood (1 in 8) andcerebrospinal fluid, and the latter cor.-tained 47 lymphocytes per c.mm.;55 mg. protein per 100 ml.; a slightexcess of-globulin (Pandy) and a Lange FIG. 4.-Gascurve 01121100000. now shcHe was given 4-2 mega units of of the se

penicillin (200,000 units daily for three 1-2/200Cweeks). Six weeks later his acuitywas 6/6 right and left, though there was still a subjectivehaziness of vision in the left eye. Perimetry now showedonly a small paracentral scotoma in the left field and aconstriction of the 1/2000 isopter.

Re-examination of the fields of vision in May, 1948,showed that the scotoma in the left field of vision haddisappeared. The blind spots were smaller and the1/2000 isopters had expanded, though they were stillconstricted a little. The disc appearances, however,were unchanged. He was then given a course of eightweekly injections of 03 g. " kharsulphan " together withpotassium iodide gr. 10 t.d.s. and pil hydrarg c. creta gr. 1t.d.s. by mouth, and this was followed by ten weeklyinjections of 0-07 g. " bivatol ".

In August, 1948, the visual acuity was W, vQs -still 6/6: 6/6; the discs showed < VQS>blurred margins without swelling, dueto overgrowth of connective tissue.The fields of vision were almost normal. /The blood Wassermann reaction waspositive 1 in 4; the cerebrospinal fluidwas normal in composition and thecerebrospinal fluid Wassermann reactionnegative. After a rest period he wasgiven a second course of penicillin, in \January, 1949 (600,000 units daily forsix days) and a third and similar coursein April, 1949.

In May, 1949, the disc appearances -.and the fields of vision were unchanged,except for a slight expansion of the1/2000 isopter in the left eye (Fig. 4); FIG. 5.-eCthe blood Wassermann reaction was R e2ye2Csilpositive 1 in 2. nto 2/2(Cstil No. 3.-Ellen H., aged 44,the not seeCase No. 3.-Ellen H., aged 44, the 1-2/200

wife of James H. (Case 2), was first seen in February,1948, complaining ofblurring of vision in the right eye ofsix weeks' duration. The mistiness of vision, initially ofsudden onset, increased for two weeks and then remained

se 2. (May, 1949) V.A. 6/6, 6/6. Fields for white targetsDw reduction in size of blind spots, with a disappearancecotomata and expansion of isopters in L. eye. In R. eye0 isopters are a little smaller than in Fig. 3.

stationary. For the first three weeks she sufferedfrom headaches over the right eye and in the righttemporal region. Four months previously she haddeveloped a red macular rash over the arms, neck andtrunk. It had lasted six weeks and left a brownishdiscoloration over the skin. At the same time shenoticed that her hair was beginning to fall out.Examination showed widespread patchy areas of

brownish pigmentation of the skin of the neck, trunkand arms and a diffuse alopfcia of the scalp. The discsshowed a bilateral papillitis. In the right eye theswelling of the disc head extended for a distance of2-3 disc diameters into the surrounding retina,

ase 3. (February, 1948) V.A. 3/60, 6/9. White targets.: greatly enlarged blind spot; dense pericentral scotomaD00. Constriction of 2-5/2000 isopters. 1 mm. targeten. L. eye: enlarged blind spot with constriction of)O isopters.

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obscuring the disc margin and the vessels crossing it.The retinal veins were distended, but there were nohemorrhages or exudate. In the left eye there weresimilar but less pronounced changes. Visual acuity was3/60: 6/9. The right field of vision(Fig. 5) showed a small, dense, UedV Sporicentral scotoma and an enlarge- Rdra, °os-ment of the blind spot. The peripheral ->field was full (3/330), but there was aconstriction of the 5/2000 and 2/2000 /white isopters, whilst the 1 mm. whitetarget could not be seen. In the left jeye the blind spot was enlarged, but ,there was no central scotoma. The1, 2, and 5 mm. white isopters wereconstricted and also the 2-10 mm. \red isopters. Neurological examinationwas otherwise normal.The blood Wassermann reaction was

positive 1 in 8. The cerebrospinalfluid was normal in composition (1 cellper c.mm.; 25 mg. protein per 100 ml.;globulin, no increase; Lange curve,0000000000), and the Wassermann FIG. 6.-Casireaction negative. normalThe patient was given, first, four pericent]

weekly injections of bismuth (I ml." bismostab ") and then 4-2 mega units of penicillin(100,000 units b.d. for three weeks). This wasfollowed by alternating courses of arsenic (03 g."sulpharasine " for eight weeks) and bismuth oxychloride(0 75 ml. " chlorostol" for eight weeks), three of eachin the next twelve months.Two months after the beginning of treatment in

April, 1948, there had been a considerable improvementin her condition. Her hair had begun to grow againand the rash had almost completely disappeared. Hervisual acuity was now 6/18 6/6. The right disc wasstill hyperxemic and its margin was blurred, but the leftdisc was normal. The fields of vision showed enlarge-ment of the blind spots and constriction of the smallerisopters. The 1 mm. target was still invisible on theright side. In June, 1948, her acuity was 6/9: 6/5.The 1 mm. target was seen up to 10° in the right eye,except for a minute area at the fixation point (<10).By August this small scotoma had disappeared, theblind spots were smaller in size, and the 1-2/2000 whiteand 3-10/2000 red isopters had expanded considerably.

She was not seen again until July, 1949, by which timeher hair had regrown completely and there was no traceof the old skin eruption. Her visual acuity (6/9 : 6/6)and the fields of vision (Fig. 6) had not changed in thepreceding twelve months. The discs were quite normalin appearance, except that no physiological cup wasvisible in either eye. The blood Wassermann reactionwas now normal.The occurrence of this rare condition at the same time

in man and wife must be unique. Unfortunately, itwas not possible to be certain how each acquiredinfection. The husband gave a history of urethraldischarge at the age of 18 and again at 31, that is 18months before the onset of the optic neuritis. Because

of this relapse marital intercourse ceased. Yet his wifehad a secondary stage rash with alopecia at the time heroptic neuritis occurred. Both denied extramaritalintercourse at any time. It is felt, however, that each of

e 3. (July, 1949) V.A. 6/9, 6/6. Fields now show almostblind spots and expansion of 1-2/2000 isopters. Notral scotoma in the R. eye.

them acquired infection in this way at different timesand that the simultaneous development of optic neuritiswas a coincidence. In the husband's case the neuritiswas, therefore, a feature of the tertiary stage.

Case No. 4.-J. C., a man of 61, was first seen inOctober, 1949. Five weeks previously he had awakenedto find that vision in his right eye was misty. Thisslowly got worse for a week, by which time he could notsee to read with the eye and distance vision was reducedto about six yards. After a fortnight there was someimprovement, and when first seen he could read news-paper headlines. At no time did he have -ocular painor headache.

Examination revealed an acuity of 6/18: 6/9. Bothoptic discs showed the changes of papillitis: hyperxmia,blurring of the margins, disappearance of the physio-logical cup. The retina was normal. Ocular tendernesswas not increased. The only other neurologicalabnormalities were an absence of the ankle jerks, adiminution of vibration sense at the ankles, and apersistent jerking nystagmus to right and left. Theblood Wassermann reaction was found to be positive1 in 8.He was admitted to hospital on October 25. The

ocular signs were unchanged except for a further reduc-tion in vision to 6/36: 6/12. The fields of vision(Fig. 7) showed a concentric construction of whiteisopters with disappearance of the smaller ones-moremarked in the right eye than the left. The blind spotswere enlarged in both eyes. There were no scotomata.Colour fields were correspondingly affected. Examin-ation of the cerebrospinal fluid showed 110 lymphocytesper c.mm., proteins 50 mg. per 100 ml., globulinincreased, Lange curve 5543321000, and the Wassermannreaction positive.

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G. S. GRAVESONThe patient was treated with 600,000 units procaine

penicillin daily for three weeks. Improvement by theend of this period was striking. Visual acuity was now6/18: 6/9. The visual fields had expanded considerably

unt.rd VQ S-9R OsCo,d. VOS.- ------_---

FIG. 7.-Case 4. (November, 1949) V.A. 6/36, 6/9. WR. eye: 5-10/2000 isopters very constricted. 1targets not seen. The 10/2000 isopter partially outliienlarged blind spot. L. eye: gross enlargement owith constriction of 2 and 5 mm. isopters. 1 mm. tar

and the papillitis was subsiding. The cerebrospinalfluid had improved also; 29 lymphocytes per c.mm.,proteins 50 mg. per 100 ml., Lange curve 334332100.By January, 1950, his acuity was normal 6/6: 6/6.

The fields of vision (Fig. 8) showed further improvementand the papillitis had almost completely subsidedwithout atrophy developing. The blood Wassermannreaction was still strongly positive. Treatment is beingcontinued with arsenic and bismuth injections.

It was impossible to discover the date of primaryinfection in this patient. He denied symptoms of theprimary and secondary stage at any time. It seemsprobable that he acquired the disease from his wife,

FIG. 8.-Case 4. (January, 1950) V.A. 6/6, 6/6. Fieldsconsiderable reduction in size of blind spots and eisopters. 1 mm. targets now seen in both eyes.

who was found to have syphilis in 1946, sincewhen no intercourse had taken place. It would appear,therefore, that his optic neuritis was a tertiarymanifestation and his other neurological abnormalities

tend to support this conclusion.Re&a,tm OD - Case No. 5.-H. S., a man aged 37

years, was admitted to hospital onAugust 29, 1949. He gave the followinghistory. Sixteen years ago he had

\\\'s\f developed a right-sided ptosis, ap-\ 7 parently without diplopia, which cleared

up spontaneously in two to three weeks.A year later he awoke one morning tofind he was almost completely blind in

I'/'/ i the right eye. There was no accom-,/t''/ panying pain or headache. No change4'4'/h' in the vision of this eye had ever

occurred and he was only able to seey// movements with it. For at least ten

years he had known that he had losthis sense of smell. For eight years he

hite targets.had been aware of muscle spasms

and 2 mm. affecting the left side of his face. Threenes a greatly weeks before admission he noticedf blind spot one morning a blurring of vision in the'get not seen. left eye. This rapidly increased and

within a week he was unable tosee anything clearly.

Examination revealed bilateral anosmia; bilateralfacial paresis (L>R) with inability to whistle; inter-mittent shock-like spasms affecting, simultaneously, thewhole of the facial musculature on the left side. Pupilswere both regular, R, 3 mm.; L, 4 mm.: the left con-tracted briskly to light; the response of the right waspoor and ill-sustained. Vision was reduced to: right,hand movements; left, 2/60. The right optic discshowed the appearances of secondary atrophy. It wasdead white in colour, its margins were not clear cut, andthe physiological cup was filled. The retina and vesselswere normal. The left fundus showed an enormous

swelling of the disc head with a

VD- prolongation of the cedema alongRfc O.D the superior temporal vessels. TheCoWV,D,d veins were distended, especially the

superior temporal, and there wereheemorrhages around this vessel whichappeared to be the seat of a peri-

/hooos \ '\ phlebitis. In the macular area was acluster of refractile hard exudates.

+odo \ \ The vitreous was hazy and, in addition,there was an old vascularized keratitis

/ 1 /l, j near the inferior limbus. The fieldsof vision (Fig. 9) showed a persistingisland of peripheral vision on the rightside. Originally he must have had alarge central scotoma which brokeout into the periphery of the visualfield. In the left field the 5 and10 mm. white targets were seen vaguely

s now show but it was possible to detectxpansion of an enlargement of the blind spot.

There was no central scotoma.

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SYPHILITIC OPTIC NEURITISThe Wassermann reaction was strongly positive in

the blood but negative in the cerebrospinal fluid. Thelatter was under normal pressure (130 mm.) and wasnormal in composition (4 cells per c. mm., proteins

FIG. 9.-Case 5. (August, 1949) V.A. H.M. 2/60. WR. eye: peripheral island of vision in lower nasal

20 mm. target. L. eye: greatly enlarged blind sp(striction of isopters especially in the lower quadranmm. targets not seen. Vision very hazy in the pre

20 mg. p-r 100 ml., Lange curve 0000000000).The patient was given 600,000 units of procaine

penicillin daily for three weeks. Visual acuity in theleft eye had improved by then to 6/24, the swelling ofthe disc was subsiding, and a white sheathing of thesuperior temporal vessels had developed. The macularexudate remained.He was seen again in January, 1950, having had no

further treatment. The acuity was now 6/12 in the lefteye. He considered this to be his normal vision, for hehad had the keratitis for a long time. The visual fields

RPtw QS. -Comct VOS-

FIG. 10.-Case 5. (January, 1950) V.A. H.M. 6/12. FielcR. eye: enlargement of peripheral island of visioran original central scotoma breaking through to thin the upper temporal quadrant. L. eye: reductioblind spot ; nerve bundle scotoma in lower quadraniof isopters. 1 mm. target not seen.

(Fig. 10) showed much improvement. It was nowpossible to define a nerve bundle scotoma in the inferiorhalf field on the left. The 1 mm. white target was stillinvisible. The peripheral island of vision in the right eye

had also expanded. The left discRAQmOD was still hypermmic and its margins

Co.Ct VOD- were blurred, but the swellinghad almost subsided and allhemorrhages and exudates had dis-appeared. The sheathing along thesuperior temporal vessels was nowvery marked and there was a patch ofchoroiditis adjacent to the disc marginwhere these vessels crossed it. The

tolIjI/ 11 other neurological abnormalities p-r-,7K ' /1// sisted unchanged. The blood Wasser-7 /< X mann reaction was now negative.

The date of primary infection couldnot be discovered, but it seems clear thatit must have been at least 16 years beforethe present attack of optic neuritis. Themultiple cranial nerve involvement

'hite targets. suggests that the pathology is one ofquadrant to chronic syphilitic meningitis.ot with con-ts. I and 2-served field. The Clinical Course of Syphilitic

Optic NeuritisOptic neuritis may occur in syphilis in three forms;

(1) as an exacerbation of congenital infection,(2) in the secondary stage, and (3) as a feature ofthe tertiary meningo-vascular period. Of the fivecases described one falls into the second group andthe other four into the third. The ocular distur-bance itself, however, does not appear to differ inthese various stages.The onset is usually abrupt, blurring of vision

being noticed suddenly, and this may increase indensity for a period of days or

R OD - weeks and then remain stationary,CmctrdVOD or even sometimes improve a littlespontaneously. Headache, or ocularpain, was mentioned by one patientonly, a surprising feature in viewof its frequency in the commonacute retrobulbar neuritis.

Visual Acuity.-The outstandingfeature of these cases is the factthat visual defect has only beennoticed subjectively in one eye,whereas ophthalmoscopic changeshave been present in both. Case 5is an exception, but his oppositeeye has been amblyopic for many

is now show years. This seems to be of con-a suggesting siderable diagnostic importance,ie periphery since other forms of bilateral optictnin size of and retrobulbar neuritis producet, expansion

bilateral visual failure. The reduction

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G. S. GRAVESONin acuity was to less than 6/60 in two cases and to alevel above this in the other three.

Ophthalmoscopic Changes.-In four patients thefundus showed changes typical of papillitis. Thedisc was intensely hyperxmic, its margins wereblurred and the cup filled in, the retina and discbeing of a uniform redness. Swelling, however,was minimal, insufficient to submerge the bloodvessels, and there were no hmemorrhages or exudates.In Case 5, however, the changes were different.Here there was a gross cedema of the disc headand spread of inflammation, first to the superiortemporal vein, producing periphlebitis with haemor-rhages and later fibrous scarring and, secondly, tothe choroid producing local choroiditis. In thiscase there was also an accompanying vitreous haze.The disc changes have tended to subside more

slowly than the improvement in acuity resultingfrom treatment and have usually been incompletelyreversible. Even with restoration of normal visionfor long periods, the disc margins have remainedindefinite and the physiological cup has not returned,a result presumably of glial overgrowth. Only inCase 5 has post-neuritic atrophy been seen. InCase 1 a patch of choroiditis was present in themacular region, but developed after -the onset ofthe optic neuritis.

Changes in the Fields of Vision.-The earliestchanges are to be seen in subjectively unaffectedeyes. Here we may note as characteristic findings(1) an enlargement of the blind spot, and (2) aconcentric constriction of the smaller isopters bothwhite and coloured. Provided these changes arenot marked, they are compatible with normalvision. They indicate a very slight disturbance ofthe whole cross section of the nerve and an inter-ference with visual reception in the retina immed-iately surrounding the disc. When more severe,as in Cases 4 and 5, visual failure will occur in theabsence of any localized central defect in the field.Damage to particular groups of nerve fibres maythen occur, however, and result in correspondingfield changes. Thus involvement of the papillo-macular bundle will produce central or paracentralscotomata (Cases 1, 2, and 3), whilst damage toother segments will produce nerve bundle defectsradiating from the blind spot (Cases 1 and 5). Ina further case, only seen once and, therefore, notreported in full because of insufficient data, acomplete inferior altitudinal hemianopia togetherwith a central scotoma was seen.These changes have been more or less completely

reversed by treatment, particularly the centralscotomata, so that normal acuity has been restored.

Other Neurological Abnormalities.-In three cases(1, 2, and 3) neurological examination was otherwisenormal. In one of these the optic neuritis occurredin the secondary stage, in the other two in thetertiary stage. Case 4 showed evidence of minorcord changes: absent ankle jerks and diminishedvibration sense in the feet and ankles. Case 5showed evidence of multiple cranial nerve damage,the nerves being presumably involved in a chronicbasal meningitis.

Serology and Cerebrospinal Fluid Changes.-Theblood Wassermann reaction was positive in allcases and the cerebrospinal fluid Wassermannreaction only positive in those cases showingchanges in the fluid (Cases 2 and 4). These latterchanges consisted of increases in the cell count andprotein content and changes in the Lange curve,one paretic, one mildly luetic. It seems justifiableto conclude, in view of the normal findings in somecases, that syphilitic optic neuritis may occur in theabsence of diffuse meningeal changes.

Prognosis.-In marked contrast to syphilitic opticatrophy, the prognosis of syphilitic optic neuritis isgood with adequate anti-syphilitic treatment. Cases1, 2, and 3 were treated with penicillin followed bycourses of arsenic and bismuth and have regainednormal vision. In Cases 1 and 3 the blood Wasser-mann reaction has become negative, whilst inCase 2 the cerebrospinal fluid became normal andthe blood Wassermann reaction only weaklypositive after a period of 18 months. Cases 4 and5, treated with penicillin alone but in larger doses,have not been followed up so long; in Case 4normal vision has been achieved but the bloodWassermann reaction remains strongly positiveafter four months. In Case 5 there has been a veryconsiderable improvement in vision and a reversalof the blood Wassermann reaction after five months.When the condition is neglected, however, seriousloss of vision may occur, as exemplified in theearlier history of Case 5, who had an attack ofoptic neuritis in his right eye 16 years previously,with no recovery and the development of a severepost-neuritic atrophy. The condition was notdiagnosed at that time and no treatment was given.

Differential Diagnosis.-The condition has to bedistinguished from (a) acute retrobulbar neuritis ofnon-syphilitic origin, and (b) other syphiliticdisorders producing comparable ophthalmoscopicchanges.

In the common demyelinating acute retrobulbarneuritis the disturbance is unilateral and the patientusually experiences headache and ocular pain.The disc may be normal or show papillitis and there

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is marked tendency for atrophy to develop evenwhen vision has recovered. Syphilitic optic neuritis,on the other hand, tends to be bilateral from thestart, though vision may only be affected in one eye.Spontaneous remission is usual in the first andprobably uncommon in the second. Of the fieldchanges the existence of defective acuity in theabsence of a central scotoma is a point in favour ofa syphilitic aetiology. Differentiation from the muchrarer acute bilateral retrobulbar neuritis may bemore difficult. In either case, diagnosis mustdepend on the finding of a positive Wassermannreaction.

Syphilitic optic neuritis has to be distinguishedfrom (1) hyperemia of the disc occurring in thesecondary stage of infection, (2) syphilitic papill-cedema as a sequel to hydrocephalus due to agumma or acute or chronic meningitis and, possibly,(3) syphilitic opto-chiasmal arachnoiditis. Hyper-emia of the disc is rare and is noticed only

incidentally, since it produces no visual symptoms.Papilloedema may be difficult to differentiate fromthe early stages of optic neuritis as it, too, mayproduce little or no visual impairment. Its under-lying cause will usually give rise to distinguishingsymptoms and signs, however, and confirmationwill be obtained by finding a raised cerebrospinalfluid pressure. Opto-chiasmal arachnoiditis isimpossible to diagnose with certainty clinically, butit can cause ophthalmoscopic and field changessimilar to those of optic neuritis. It should,perhaps, be considered in cases of apparent neuritiswhich fail to improve with treatment.

DiscussionMany names have been attached to this and

similar syphilitic disorders of the optic nerveincluding neuroretinitis, perioptic neuritis, retro-bulbar neuritis, papillitis, gumma of the nerve head,opto-chiasmal arachnoiditis, choroiditis juxtapapillaris. This confusion of nomenclature isdoubtless due, in part at least, to the absence ofhistological study of the earliest manifestations ofthe disease. The few pathological findings whichhave been reported have concerr.ed cases whichhave progressed to late atrophy of the nerve. Theyhave shown that the nerve fibres are involvedsecondarily to an inflammatory reaction in thesheaths and septa of the nerve. This reaction hasbeen most marked in the intracranial portion of thenerve and less extensive in the intraorbital part.Such facts are difficult to reconcile with the clinicalpicture, for it would appear altogether probablefrom this that the initial focus of disease issituated in the papilla, and that spread of thedisease may occur either into the retina and choroid,

or backwards along the nerve itself. In the firstcase neuroretinitis or- choroiditis juxta papillarismay result, in the second perioptic neuritis, or evenopto-chiasmal arachnoiditis. Should the processbe very intense then actual gumma formation inthe papilla might occur. Another rare but inter-esting syndrome is that described by Fuchs (1926)as neuritis papulosa, a syndrome consisting ofenormous swelling of the disc, periphlebitis andchoroiditis. In this condition fibrous adhesionsmay later develop between the papilla and thepatches of choroiditis, giving rise to a picture ofretinitis proliferans. Case 5 here would seem tofall into this category, but it is felt that the initiallocalization of the disease in this case was essentiallysimilar to that in the other four cases, the differencein the clinical picture being due to the intensity ofthe papillitis and to a spread of inflammation toinvolve the retinal veins on the one hand and thechoroid on the other.

In the absence of precise pathological details ithas, therefore, been felt desirable to describe thecondition as one of optic neuritis. The term retro-bulbar neuritis has little to commend it, for it seemsextremely doubtful whether the classical picture ofthis condition, namely, a central field defect occur-ring without alteration of the optic nerve headever occurs in syphilis. When central scotomataoccur with primary syphilitic optic atrophy, theunderlying pathology is one of meningeal involve-ment. Papillitis may possibly prove to be a moreexact description, but it suggests a restriction ofthe disease process which is, as yet, unsupported bypathological findings.The literature on the subject is scanty. Apart

from a few reports of single cases, the only con-siderable contributions have been those of Wilbrandand Saenger (1913), Igersheimer (1918) and, morerecently, Walsh (1947). Wilbrand and Saenger'sthree cases each had unilateral post-neuritic atrophywith central scotoma or concentric constriction ofthe visual field. Two were examined post-mortemand showed fibrous thickening and cellular infil-tration of the sheath and septa of the nerves withatrophy ofthe margin nerve fibres. They consideredthat the initial lesion was in the papilla withsubsequent spread backwards to the optic nerve,and that the optic neuritis was not part of a morewidespread syphilitic meningitis. Igersheimer (1918)gives brief details of 13 cases of optic neuritis.All but two recovered normal vision after treatment,and in most cases the fundus also returned tonormal. Igersheimer also thought that the in-flammatory disturbance was focused at the papilla.He stated that it occurred more commonly in theabsence of other neurological abnormalities, some-

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times in the secondary stage of infection, but moreoften later. He explained the field changes as dueto nerve bundle defects, central scotomata beingthe result of involvement of the papillo-macularbundle as it lies superficially in and behind thepapilla.Walsh (1947) attempts to distinguish between

perioptic neuritis, neuroretinitis, retrobulbar neuritisand gumma of the nerve head. The result is notvery satisfactory, as the clinical features of theseseveral conditions overlap. He describes fourcases; one clearly had a chiasmal lesion, anotherpossibly had tobacco amblyopia. The other twowere not examined in the early stages and atrophyof the nerve had developed associated with centralfield defects. Two improved with anti-syphilitictreatment and two did not.

Sloan and Woods (1938), in a study of visualfield defects in syphilis, record the changes in threecases of active optic neuritis, one occurring in thesecondary stage, two due to neurorecurrence. Allshowed enlargement of the blind spot and eithernerve bundle lesions or central scotomata.

Mention must also be made of syphilitic opto-chiasmal arachnoiditis, a condition dealt with inparticular by Bollack, David, and Puech (1937) andHausman (1937, 1941, and 1942). Such a diagnosiscan only be established by surgical exploration,and although this has mainly been done in cases ofoptic atrophy unresponsive to treatment, yet somepatients have presented with papilloedema. Thecombination of this with diminished visual acuityand field defects raises the question of its differentialdiagnosis from syphilitic optic neuritis as here

described. Clinically, this would seem at times tobe impossible until the effects of anti-syphilitictreatment have been observed, for whereas opticneuritis responds well to therapy, arachnoiditis isseemingly refractory.

SummaryFive cases of syphilitic optic neuritis are described.

The condition is one which may occur in thesecondary or tertiary stages of infection. Its modeof presentation is similar to the commoner demy-elinating acute retrobulbar neuritis, in that unilateralfailure of vision is complained of but, character-istically, bilateral ocular changes are found. Theclinical features are described and the favourableresults of treatment contrasted with the lack ofresponse in syphilitic optic atrophy.

I wish to thank Dr. F. R. Ferguson and Dr. G. E.Smyth for permission to record the details of patientsunder their care.

REFERENCESBollack, J., David, M., and Puech, P. (1937). "Les

Arachnoldites opto-chiasmatiques." Paris.Fuchs, A. (1926). Z. Augenheilk., 58, 315; 59, 213.Hausman, L. (1937). Arch. Neurol. Psychiat., Chicago,

37, 929.(1941). Amer. J. Ophthal. 24, 119.(1942). J. Mt. Sinai Hosp., 9, 544.

Igersheimer, J. (1918). "Syphilis und Auge." Berlin.Sloan, L. L., and Woods, A. C. (1938). Arch. Ophthal.,

Chicago, 20, 201.Walsh, F. B. (1947). " Clinical Neuro-ophthalmology."

Baltimore.Wilbrand, H., and Saenger, A. (1913). "Die Neurologie

des Auges," vol. 5. Wiesbaden.

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syphilitic optic neuritis · syphilitic optic neuritis out-patient treatment at his local clinic...

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