neurol. · fractional gastric cells 5,000 per c.mm. analysis showed histamine-fast achlorhydria,...

J. Neurol. Neurosurg. Psychiat., 1951, 14, 40.

THE VISUAL FIELD DEFECTS IN SUBACUTE COMBINEDDEGENERATION OF TIE SPINAL CORD

BYG. H. H. BENHAM

From the Department ofNeurology, Manchester Royal Infirmary

The occurrence of optic atrophy as part of thesyndrome of subacute combined degeneration ofthe spinal cord is distinctly uncommon (Woltmann,1919; Smithburn and Zerfas, 1931; Young, 1932).A clinical review and resume of the literature wasgiven by Turner (1940), who reported three casesand was the first writer to comment specifically onthe characteristics of the visual field defects. Thepurpose of this communication is to record furtherobservations on the fields of vision in this syndrome,and to discuss their possible significance and clinicalimportance.

Review of the LiteratureIn only a few instances have the visual fields been

described. Bickel's (1914) patient had a centralscotoma in the left eye, and the case is quoted byTurner (1940) as a possible example ofthe condition.But the post-mortem findings comprised markedperivascular cellular infiltration in posterior andlateral columns, with proliferation of the vesselsthemselves, and it seems impossible to accept thecase as one of subacute combined degeneration.Hine (1936) reported full peripheral fields with acentral colour scotoma in each eye, most markedfor green and very difficult to chart accurately, inhis patient, whereas Talbot (1936) recorded fullfields for 1° white, but noted a large relative centralscotoma in each eye. Neither of these authorspublished charts, nor did they allude in the text tothe distinction between the pericentral and centro-caecal types of scotoma. Box (1936) reported " leftfield full in his patient, but the acuity in this eyewas as good as 6/12 after correction and it is notstated that a specific search was made for a scotoma.Cohen (1936) described slight concentric contractiononly in his two patients, but the gross reduction invisual acuity to 6/60, 6/36 and 6/, 6/W0 seemsinconsistent with this finding unless the centralfields were also involved by scotomata. Kampmeierand Jones (1938) mention the fields only in theirsecond patient, saying that there was no apparentgross defect; despite this the acuity was very

seriously reduced, and they indicate that the subjectwas somewhat uncooperative. Courville andNielsen (1938) report that their second patient hadmarked constriction of the visual fields, and thoughthey note that the Bjerrum screen was used they donot say whether the presence of scotoma wasexcluded. Turner (1940) published charts from twoof his three cases, all of which presented essentiallycentrocaecal defects barely covering the fixationpoint, or even falling short of it; there was slightor moderate peripheral constriction. Traquair(1946) pictures charts of almost identical appearancefrom a case which, though clinical details are notgiven, is described as " retrobulbar neuritis inpernicious anaemia with subacute combined degen-eration of the spinal cord ". In the text on anotherpage, however, he says that the visual fields inpernicious anaemia are similar to those of Leber'sdisease-with a dense scotoma freely involving thefixation area, possibly fibre bundle defects, andfrequent affection of the peripheral field-and donot present any distinguishing features. Walsh(1947) in his textbook mentions a case of perniciousanaemia showing bilateral field defects like thosedescribed by Turner (1940), but does not detail thesigns and symptoms.To sum up, the peripheral fields in these recorded

cases are usually full or nearly so and only excep-tionally do they show marked contraction. Reallyadequate data about the central fields are availableonly in the cases of Turner (1940) and Traquair(1946), all four showing a centrocaecal'pattern, butTraquair gives no clinical details to permit certainidentification of his case as one of subacute combineddegeneration. A fifth case (Walsh, 1947) is probablyidentical, but categorical information is lacking bothas to ocular and general clinical findings.

Clinical ObservationsDuring the examination of a series of 112 treated

and untreated cases of subacute combined degen-eration of the spinal cord five instances of optic

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VISUAL FIELD DEFECTS IN SUBACUTE COMBINED DEGENERATION

atrophy were encountered. Two of the five patientswere unable to cooperate satisfactorily in examina-tion of the visual fields, one on account of dementiaoccurring as part of the neurological syndrome andthe other on account of the onset of senile deteriora-tion. The remaining three cases are described below.

Case ReportsCase 1.-A woman aged 59 was admitted to hospital

in 1941 with a history of general weakness for six monthsand of weakness of the hands and difficulty in walkingfor two weeks. While in the ward she became awarefor the first time of impairment of vision. She hadincomplete pyramidal and posterior column signs,achlorhydria, and a red-cell count of 1,630,000 per

FIG. 1.-Visual fields in Case 1 (corrected for eccent= 3/60, L.V. = 6/36. Objects 10/2000, 3/2000 vblind spot 40/2000 white.

c.mm. with haemoglobin 42%, colour index 131, andwhite cells 5,000 per c.mm. The reticulocyte count was8-8%, and many nucleated red cells were present in theperipheral blood. There was a rapid response toparenteral liver therapy, which was continued indefinitely.When I first saw the patient in

1948 she reported slight numbnessand clumsiness of the hands,preventing the use of a sewingneedle, and considerable impair-ment of vision, so that small printcould not be distinguished and therecognition of faces at a distancewas difficult. On examination thevisual acuity was 3/60 right, 6/36 Uleft and both optic discs showedmarked pallor, maximal in thetemporal halves. The visual fields(Fig. 1) showed considerable con-centric contraction, greater in theright eye, and large dense scoto- --mata of centrocaecal type withsteep edges just overlapping thefixation point; on perimetry, fix- FIG. 2.-Visual fiel'ation in each eye was eccentric by Object 6/2000 v

about 3°. The intrinsic muscles of both hands were alittle weak and the power of opposition of the littlefingers was lost. Tone was a little increased in bothlower extremities. The tendon reflexes, with the exceptionof the ankle jerks, were much exaggerated, and sustainedpatellar clonus was present. The right plantar responsewas extensor and the left unobtainable, whilst theabdominal reflexes were absent. Two-point discrimina-tion was impaired in the fingers of both hands, andvibration sense lost below the level of the iliac crests;sensation was normal in other modalities. The gaitappeared normal. The other systems were normal andthe blood pressure 190/100, without evidence of peri-pheral arteriosclerosis. A blood count showed redcells 4,930,000 per c.mm., haemoglobin 98%, and whitecells 5,200 per c.mm.

The clinical features ofthis case, and its response totreatment, justified a diag-nosis of pernicious anaemiawith subacute combined sys-tem disease. Visual failuredeveloped during the courseof the illness and may fairlybe considered an integral part.Case 2.-A man aged 68 was

admitted to hospital with a his-tory of unsteadiness in walkingfor two years and of gradualvisual failure, tingling in the fin-ger tips, and nocturnal cramps inthe feet, for one year. He smoked

;ric fixation). R.V. one ounce of mild tobaccovhite, 1'/2000 red; weekly.

The visual acuity was 3/60 ineach eye, and both optic discs

showed marked pallor. The visual fields (Fig. 2)showed considerable concentric contraction and verylarge pericentral scotomata extending temporally toinclude the blind-spots. Both legs were grossly ataxic,and the sense of passive movement was impaired in all

ds in Case 2 before tiwhite; blind spot 15/

treatment. R.V. and L.V. = 3/60./2000 white.

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progressive visual failure for ninemonths; unsteadiness of gait foreight months; and an abdominalgirdle sensation for one month.

~~, Ten years before he had had(2x, _ > thyrotoxicosis but recovered

/rlf /\I g ''~- )hitXh,\1 following operative treatment.He had been in the habit of

2roJ i \ \ 3t/ smoking four ounces of twist_/)/ /__ J weekly, but had ceased to do so99/ six months before exatnination.

He had obvious exophthalmosand a red glazed tongue. Thevisual acuity was 6/60 right, 6/36left; the right optic disc showedtemporal pallor and the leftgeneral pallor. The legs were

FIG. 3.-Visual fields in Case 2 after treatment. R.V. and L.V. 6/18. ataxic, with impairment of theObjects 6/2000, 2/20J0 white, 10/2000 red; blind spot 40/2000 white. sense of passive movement and

of vibration sense, but super-the toes; vibration sense was lost only in the right ficial sensibility appeared intact and no musclefoot. The knee and ankle jerks were absent, but the tenderness was present. The reflexes were normalplantar responses were flexor and the abdominal apart from exaggeration of the knee and ankle jerks.reflexes intact. The gait was grossly ataxic with a broad The other systems were normal and the blood pressurebase, and Romberg's sign was positive. Arcus senilis was 130/80. The red cells numbered 4,620,000 per c.mm.,was prominent, but the other systems were normal with haemoglobin 92%, and white cells 7,600 per c.mm.and the blood pressure only 145/100, with no significant One month later the patient began to deteriorateperipheral arterial thickening. The cerebrospinal fluid rapidly, developing retention of urine and completewas normal and the Wassermann reaction negative. inability to walk. He was admitted to hospital, whereA blood count showed red cells 2,840,000 per c.mm., he was found to have low-grade fever, considerablehaemoglobin 58%, colour index 0-98, and white cells oedema of both legs, profound weakness and ataxia of2,050 per c.mm. The sternal marrow was normoblastic, the legs, exaggerated tendon reflexes and extensorbut it was discovered that liver extract had been given plantar responses with absent abdominal reflexes, absentjust before the patient's admission to hospital. A hista- vibration sense throughout the lower extremities, andmine-fast achlorhydria was demonstrated. loss of the sense of passive movement in toes, ankles,

Intensive liver therapy was given, and two and a half and knees. The cerebrospinal fluid was normal andmonths later the patient's paraesthesiae had almost dis- the Wassermann reaction negative. A blood countappeared, he was walking much better, and vision showed red cells 3,460,000 per c.mm., haemoglobin 68%,had greatly improved. The acuity was now 6/18 in colour index 0-98, and white cells 4,900 per c.mm.each eye and the fields (Fig. 3), though still showing with normal differential count. The absolute red cellmarked concentric contraction, displayed great diminu- indices were all within normal limits. There was ation in the size of the scotomata, which were now reticulocytosis which persisted throughout the illnessessentially centrocaecal in pattern, just falling short of at a level of3% to 4%, but the serum bilirubin and salinethe fixation points. The red cell count had risen to fragility were within normal limits and occult blood4,520,000 per c.mm., with haemoglobin 90% and white was always absent from the stools. Fractional gastriccells 5,000 per c.mm. analysis showed histamine-fast achlorhydria, but the

Pyramidal signs were lacking and posterior sternal marrow was normoblastic. Liver function tests

column signs incomplete but the neurotogical showed no abnormality.iclumn wasionsistnc pte,buth the nreuro ip

al Within a few days he developed pressure sores overpicture was consistentewith the erie phasesso the sacrum and buttocks and a urinary infection.subacute combined degeneration, and this diagnosIs Treatment with "hepastab ", 4 ml. daily, was begun,was supported by haematological findings and the and within a week the paraesthesiae had become much lessresponse to specific therapy. Visual failure presented marked. Progress was otherwise very slow, but afteras an integral part of the illness and likewise three and a half months all bedsores had healed andimproved remarkably with treatment; the con- he was able to stand though not to walk; the red cellsumption of tobacco was insufficient to have any count was 4,530,000 per c.mm., and the haemoglobinbearing on the amblyopia. 86%. After a further three months he could walk

200 yards with the aid of a stick. The visual acuityCase 3.-A man aged 47 attended the out-patient had improved to 6/36 right, 6/18 partly left; the visual

clinic complaining of tingling sensations of one year's fields (Fig. 4) showed bilateral centrocaecal scotomataduration, involving first the fingers and toes and later with a slight col between fixation point and blind spot,spreading up the legs and trunk to the waist level; whilst their peripheral extent was nearly full.

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FIG. 4.-Visual fields in Case 3. R.V. = 6/36, L.V. 6/18 pt. Objects2/2000 white, 10/2000 red; nucleus 10/2000 white blind spot'10/2000 white.

The gait continued steadily to improve and the red presented featurecell count reached 5,280,000 per c.mm. with a haemo- cases (Figs. 1, 3, aglobin of 102%, but the visual acuity and visual fields and centrocaecaldisplayed no further change. quite close to the

This man had a normocytic anaemia with a was variable, but Ipersistent reticulocytosis of 3 to 4%, and the case and in two instadid not show the haematological features of pernici- narrowing and dious anaemia. Neurologically, however, the illness of the blind spot.presented with obtrusive, slowly spreading par- steep edges, andaesthesiae in all four limbs, followed by ataxic present-findings

paraplegia with posterior column signs antedating dition of the lesicpyramidal signs. This picture, associated with The close resen

glossitis and histamine-fast achlorhydria, would in these three cas

seem to justify a diagnosis of subacute combined reported cases iIdegeneration of the cord, and this is further sup- material is still Iported by the response to liver therapy: slow but further observatiuninterrupted recovery followed the beginning of interest to examirthis treatment, whereas before its institution the any special signifisymptoms were steadily aggravated without remis- centrocaecal pattesion. The phase of rapid deterioration just before Significance oladmission to hospital, with crural paralysis, sphinc- According to Lillter disturbances, fever and oedema of the lower individuality, beiihalf of the body, is reminiscent of the classical of the symptomsdescription by Russell, Batten, and Collier (1900). aetiology, fromThe possibility that tobacco was the cause of the ultimate blindnes.amblyopia had to be considered, as the patient had occur in some irbeen a heavy smoker of twist, but cessation of the exceptions ; thushabit for six months had brought about no improve- metrical pericentiment in vision, and the onset of the paraesthesiae the major defect tof the extremities three months before the beginning blind spot and fiof visual failure strongly suggests that the latter centrocaecal scotsymptom was in fact part of the major neurological conditions whichdisorder. Moreover vision improved pari passU another, includingwith the other neurological functions when liver disease, diabetestherapy was given. pressure lesions

Discussion 1946). But, as

The Type of Field Defect.-The peripheral fields relatively infrequ4in these patients showed great variation, ranging in Leber's disease

from almost full extent inCase 3 to severe contractionin Case 2.Only in Case 2 was an

opportunity afforded to exam-ine the central fields beforetreatment, and here thepattern was one of pericentralscotoma of about 100 radiuslinked to the enlarged blindspot by a rather narrowerzone. Fixation and coopera-tion tended to be imperfectat this stage of the illness, andexhaustive examination usingmany different visual angleswas precluded by fatigue. Thecentral fields after treatment

-s of similarity in each of the threend 4). The scotomata were bilateralin position, with the nasal edgefixation point. The vertical extentthe long axis was always horizontal,ances (Cases 2 and 3) there wasiminished density to the nasal sideThe scotomata were dense, with

disproportion for colour was notconsistent with the stationary con-on at the time of examination.rnblance of the centrocaecal defectses to those found in the more fullyn the literature is evident. Thelimited to eight cases in all, andions are necessary, but it is ofne the question of whether there isicance in this frequently occurringern.r the Centrocaecal Scotoma.-lie (1934) this scotoma has no realng merely a stage in the progress;of an optic nerve lesion, of anyan initial pericentral scotoma to;s. But though this sequence maynstances, there are many obviouss in tobacco amblyopia a sym-ral scotoma does not occur andthroughout the illness lies betweenixation point. It is true that theoma has been reported in manyhave little in common with oneacute retrobulbar neuritis, Leber's

syphilis, arteriosclerosis, and(Duke-Elder, 1940; Traquair,noted by Traquair (1923), it isent in disseminated sclerosis andwhereas it is the rule in tobacco

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amblyopia, and it seems hardly possible to doubtthat it is an entity with some specific mechanism oforigin.No pathological studies appear to have been made

in cases of amblyopia associated with subacutecombined degeneration, and ideas as to the mechan-ism of production of the changes in the visual fieldscan therefore be only theoretical and speculative.But in tobacco amblyopia many observations havebeen made on the morbid anatomy (Parsons, 1908;Duke-Elder, 1940) and there is general agreementthat there is degeneration both ofthe papillo-macularbundle and also of retinal ganglion cells, but whichof these sites represents the primary lesion remainsunsettled. It is not easy fully to correlate thispathological incrimination of the papillo-macularfibres with the anatomical features of the scotoma.The papillo-macular bundle innervates the fovealarea of the retina, and damage to the bundle mightbe expected to produce a symmetrical pericentralscotoma of about 50 radius, which in fact occurswith many optic nerve lesions. Two deductionsthen seem reasonable concerning the centrocaecaltype of scotoma. First, involvement of macularfibres must be very unequal and asymmetrical.Second, that part of the scotoma between the 50and 130 circles must be due to damage to extra-macular fibres. As the retinal area in question isnasal to the macula these fibres must be crossedfibres situated in the nasal half of the optic nerve.The exact site of such juxtapapillary fibres in theoptic nerve is still disputed, but whether it be axialor peripheral it is very difficult to conceive of aunitary lesion within the nerve which could accounton any known anatomical basis for simultaneousdamage to papillo-macular fibres and to thiscuriously selected group of nasal extramacularfibres. Anatomical considerations in fact seemstrongly to favour a primary disturbance in theretina, where the affected neural elements aredisposed in contiguity.A primary retinal lesion was proposed by Parsons

(1908), who suggested, as possible mechanisms,poisoning of synapses between retinal elementsand retinal vascular spasm; but the selectivevulnerability of particular neurons remainedunexplained. Behr (1935) on the contrary, insistedthat the lesion was primary in the nerve, quotingpathological observations that the severity andextent of damage to the papillo-macular bundle wasinversely correlated with the richness of its bloodsupply and was histologically maximal in theintracranial portion of the nerve, but correlationwith the clinical features of the scotoma-referredto as " central "-was not attempted. Traquair(1923) thought that a specific anatomical site for

the lesion could not be assigned, and believed thatcertain nerve elements might have a special affinityfor the toxic substances: later (Traquair, 1946) headded that the pronounced tendency of the scotomato lie along the horizontal meridian was suggestiveof a nutritional and ultimately anatomical basis forthe special susceptibility.A new approach to the problem of a retinal basis

for this scotoma has been made by Evans (1940).He claims that in some circumstances the centro-caecal scotoma may undergo transient fluctuationin size paripassu with variation in the angioscotomaof the main retinal vessels, and he argues that avascular origin for this field defect is therebydemonstrated. The retinal area concerned is largelysupplied by the cilio-retinal system, whether or notfrank cilio-retinal arteries are visible with theophthalmoscope: these vessels must then supplythe macular fibres as they traverse the retina,though not the fovea itself which is avascular andnourished from the choroidal blood bed. A primaryfoveal lesion is therefore unlikely from vascularcauses, but if a toxic or degenerative lesion of themacular fibres occurs, Evans (1940) suggests that" there may be a call for greater metabolic activityof . . . paramacular vessels as a gesture towards therestoration of local function ", and that such" derangements of this minute vascular tree doubt-less cause interruptions in the passage of the nerveimpulse from the cells in the region it supplies",thus producing the centrocaecal field defect.

This hypothesis at once permits interpretation ofthe conditions found in Case 2 reported in thispaper. The severe concentric contraction and thelarge pericentral scotoma alike suggest neuronaldamage of exceptional degree. This may be sup-posed to call forth a response by the paramacularvessels which, though maximal, is unable to restorenormal macular function. After treatment it maybe presumed that the vascular response is adequateto restore function in the now less severely damagedcentral fibres, and thus the pericentral scotomadisappears, but the centrocaecal scotoma resultingfrom the vascular derangement persists, thoughreduced in extent. But although this theory ofEvans (1940) is thus helpful, and in many otherways attractive, it is still open to criticism. It doesnot account for the temporal field defects sometimesobserved for small visual angles between the 200and 300 circles (Traquair, 1946). It does not explainthe nature of the metabolic mechanism supposedto caU forth the vascular response, nor the restrictionof the phenomena to certain groups of cases withpapillo-macular damage. There is also a possiblenon sequitur in that the vascular changes are pre-sumed simultaneously responsible -for restoration

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of function in the macular fibres and for impairmentof function in the ganglion cells and synapses inthe same retinal area. However, till more detail isforthcoming than is available in the originator'sbrief formulation quoted above, a just assessmentwill not be possible.

If this hypothesis, of a dual origin, neural andvascular, for the centrocaecal scotoma, be tentativelyaccepted, has the occurrence of the latter in casesof subacute combined degeneration any specialsignificance ? The idea has indeed long been currentthat a vascular factor might explain the character-istic distribution of the lesions of subacute combineddegeneration within the nervous system (Russell,Batten, and Collier, 1900; Wilson, 1940; Biggart,1949) but a satisfactory precise formulation hasnever been achieved. Stannus (1944) claimed thatin ariboflavinosis the lesions were situated in thoseparts of the nervous system with the richest capillaryblood supply, and advanced the concept of a" capillary dysergia " manifesting itself by dilatationofvessels and retardation offlow, causing interferencewith normal cellular metabolism and derangementof tissue function. The tentative description givenby Evans (1940) of dilatation of the paramacularvessels is reminiscent of this hypothesis, but thetwo theories differ fundamentally: with Stannus(1944) the vessels dilate because of intrinsic capillarydamage of nutritional origin, whereas with Evans(1940) they dilate in response to some change inthe tissues they supply. Moreover, the concepts ofStannus (1944), whatever their relevance to aribo-flavinosis, cannot well explain the distribution oflesions in subacute combined degeneration, inwhich areas of low vascularity like the posteriorcolumns (Stannus, 1944) are commonly involved.Neither is the problem clarified by direct comparisonof the visual field changes in subacute combineddegeneration with those in other forms of nutritionalneuropathy. The centrocaecal form is uniform intobacco amblyopia, which many workers considerof nutritional origin (Carroll, 1936), whilst con-flicting findings are reported in the amblyopiassometimes associated with diabetes and chronicalcoholism (Duke-Elder, 1940; Traquair, 1946).In the nutritional neuropathies occurring in prisonersof war Spillane's (1947) cases presented the peri-central type of scotoma, though some were so largeas to engulf the blind spot, while Graveson (1947)reported nearly 15% incidence of centrocaecaldefects, 65% being pericentral and the remainderparacentral or annular.The problem therefore is very far from solution.

It can only be said that a retinal vascular factoras well as a neural factor seems probable in thegenesis of the centrocaecal scotoma. In the ambly-

opia of subacute combined degeneration it istempting to try to relate such a vascular factor tovascular theories of the genesis of the disease, butevidence on the question is conflicting and unin-formative.

The Field Changes and Differential Diagnosis.-The observations recorded in this paper have con-siderable clinical importance in relation to theproblem of diagnosis and treatment of the middle-aged patient presenting with subacute onset ofbilateral amblyopia with centrocaecal scotoma. Inmy experience the commonest conditions with thisclinical picture are tobacco amblyopia and a so-called chronic retrobulbar neuritis; but the possi-bility of subacute combined degeneration must beconsidered in every such case, and this is of greatimportance because of the chance of ameliorationor cure by specific therapy, which is more effectiveneurologically the earlier it is applied.The majority of cases of subacute combined

degeneration can be recognized by the presence ofother abnormal neurological signs. Neverthelessoptic atrophy from this cause may occur before thedevelopment of signs elsewhere in the nervous system(Turner, 1940), or even before blood changes becomemanifest (Cohen, 1936), and in such cases diagnosismay be very difficult. Achlorhydria is a sine qua non,but occurs in a considerable proportion of elderlysubjects without pernicious anaemia (Oliver andWilkinson, 1933), and such points as soreness ofthe tongue and a family history of perniciousanaemia may assume considerable importance. Ifall these fail a controlled therapeutic test withliver extract may be justifiable.

Writers on tobacco amblyopia have tended per-haps to oversimplify the diagnosis. Thus Traquair(1930, 1946) emphasizes the importance of otherdiseases in precipitating the onset of amblyopia,and Carroll (1935) refers to a case associated withpernicious anaemia. Whether or not such patientsmake full visual recovery, it seems to me difficult tobe sure that the essential factor was not perniciousanaemia rather than tobacco; this problem arose inCase 3 of the present series. Secondly Traquair(1930, 1946) claims that the special characters ofthe visual fields in tobacco amblyopia are virtuallypathognomonic. But these characters, whichinclude diffuseness of the scotoma with slopingedges and marked disproportion for colours, aremerely expressive of a diffuse lesion producingpartial impairment of function of many nerveelements, but destroying only a few. Now manycases of subacute combined degeneration showvery slow progression of spinal signs and symptoms,and if the ocular lesions advance at a similar rate

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it is clearly not impossible that a case in the earlystages of amblyopia might present signs of partialimpairment of retinal function similar to those oftobacco amblyopia. The description of Hine (1936),quoted earlier in this paper, is illustrative of thispoint, even though he does not detail the exactlocation of the scotoma.

" Chronic retrobulbar neuritis " is not a very welldefined entity. One group of cases is alluded to byDuke-Elder (1940), who emphasizes the resemblancesto the toxic amblyopias rather than to the inflam-matory lesions, the frequent bilateral involvement,the slow onset, chronicity, and poor prognosis.Traquair (1930) also describes similar cases present-ing centrocaecal scotomata, and during the periodin which the cases of subacute combined degenera-tion here reported were collected I have seen fourcases approximating to this clinical picture. Therewas no indication of the aetiology. All were over

50 and the age incidence is not dissimilar to thatof pernicious anaemia. Where free acid is present inthe gastric juice, diagnosis is less difficult, but ifthere is achlorhydria, a controlled therapeutictrial with liver extract may be desirable.

SummaryThe literature of optic atrophy associated with

subacute combined degeneration of the spinal cordis reviewed with reference to the changes reported inthe fields of vision.

In a group of 112 cases of subacute combineddegeneration five instances of optic atrophy wereencountered; in three of these accurate assessmentof the visual fields was possible, the essential defectsbeing scotomata of centrocaecal type with varyingdegrees of peripheral contraction.The significance of these observations is discussed

and the diagnostic importance noted.

I wish to thank Dr. F. R. Ferguson and Dr. J. F.Wilkinson for permission to quote cases under their

care in the Departments of Neurology and Haematologyat the Manchester Royal Infirmary.

REFERENCESBehr, C. (1935). v. Graefes Arch. Ophthal., 134, 227.Bickel, H. (1914). Arch, Psychiat. Nervenkr., 53, 1106.Biggart, J. H. (1949). " Pathology of the Nervous Sys-tem ", 2nd ed. Edinburgh.

Box, C. R. (1936). Lancet, 2, 1269.Carroll, F. D. (1935). Arch. Ophthal., Chicago, 14,421.- (1936). Ibid., 16, 919.Cohen, H. (1936). Lancet, 2, 1202.Courville, C. B., and Nielsen, J. M. (1938). Bull. Los

Angeles neurol. Soc., 3, 83.Duke-Elder, W. S. (1940). "Text-book of Ophthal-mology." Vol. 3. London.

Evans, J. N. (1940). Arch. Ophthal., Chlicago, 24, 532.Graveson, G. S. (1947). M.D. thesis. University ofCambridge.

Hine, M. L. (1936). Proc. R. Soc. Med., 29, 386.Kampmeier, R. H., and Jones, E. (1938). Amer. J. med.

Sci., 195, 633.Lillie, W. I. (1934). Amer. J. Ophthal., 17, 110.Oliver, T. H., and Wilkinson, J. F. (1933). Quart. J.Med. 26, 431.

Parsons, J. H. (1908). " The Pathology of the Eye.'"Vol. 4. London.

Russell, J. S. R., Batten, F. E., and Collier, J. (1900).Brain, 23, 39.

Smithburn, K. C., and Zerfas, L. G. (1931). Arch.Neurol. Psychiat., Chicago, 25, 1100.

Spillane, J. D. (1947). " Nutritional Disorders of theNervous System." Edinburgh.

Stannus, H. S. (1944). Brit. med. J., 2, 103 and 140.Talbot, G. (1936). Brit. J. Ophthal., 20, 619.Traquair, H. M. (1923). Trans. ophthal. Soc. U.K.,

43, 480.(1930). Ibid., 50, 351.

--(1946). " An Introduction to Clinical Perimetry."'5th ed. London.

Turner, J. W. A. (1940). Brain, 63, 225.Walsh, F. B. (1947). " Clinical Neuro-Ophthalmology."

Baltimore.Wilson, S. A. K. (1940). " Neurology." Vol. 2.London.

Woltmann, H. W. (1919). Amer. J. med. Sci., 157, 400.Young, R. H. (1932). J. Amer. med. Ass., 99, 612.

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