sign hydrocephalus · sylvian aqueduct syndrome as asign ofacute obstructive hydrocephalus in...

Journal of Neurology, Neurosurgery, and Psychiatry, 1975, 38, 288-296

Sylvian aqueduct syndrome as a sign of acuteobstructive hydrocephalus in childrenAMRIK S. CHATTHA AND G. ROBERT DELONG

From the Department of Neurology, Massachusetts General Hospital,Boston, Massachusetts 02114, U.S.A.

SYNOPSIS Eight cases of obstructive hydrocephalus manifesting palsy of upward gaze and otherfeatures of the Sylvian aqueduct syndrome are reported. During the crisis of intracranial hyper-tension, all of them developed upward gaze palsy and variable abnormalities of the convergencemechanism such as paralysis, spasm, and convergence nystagmus. The frequent apparent blindnesswas probably related to gaze paralysis, since visual evoked responses were present. All these ocularabnormalities disappeared after shunting. Periaqueductal dysfunction on the basis of raised intra-cranial pressure is postulated as the possible mechanism for the above ocular manifestations. The' setting sun' sign is frequently seen in infants and children with hydrocephalus and has been con-

sidered in the past to result from displacement of eyeballs by pressure from the orbital roof plate.Our observations would suggest periaqueductal dysfunction rather than the mechanical displace-ment as the possible mechanism for this sign.

Impairment of upward gaze is a well knownneurological sign of diseases which affect thebasal ganglia, mesencephalon, and the fourthventricle (Cogan, 1956). The exact clinicallocalization is assisted by the accompanyingfeatures. When the paralysis of upward gaze iscombined with abnormalities of the pupil,paralysis or spasm of convergence and nystag-mus retractorius, a periaqueductal localization isindicated and this constellation of findings iscalled the Koerber-Salus-Elschnig Sylvian aque-

duct syndrome (Kestenbaum, 1946). Paralysis ofupward gaze alone is commonly called Parinaud'ssyndrome, though some authors (Walsh andHoyt, 1969) use Parinaud's synonymously withthe Sylvian aqueduct syndrome. The partial or

complete Sylvian aqueduct syndrome has beenrecorded in patients with pinealoma, vascularlesions, encephalitis, trauma, multiple sclerosis,neurosyphilis (Cogan, 1956), and on a congenitalbasis (Fredericks and Van Nuis, 1967). Theparalysis of upward gaze has also been reportedin association with raised intracranial pressure

(Schwarz and Rosner, 1941), particularly inhydrocephalus. Pennybacker (1940) first reportedfive patients of adult onset hydrocephalus due toAccepted 5 July 1974.)

aqueductal stenosis who had paralysis of up-ward gaze and pupillary abnormalities. Subse-quently, there have been isolated case reports ofupward gaze paralysis occurring in hydro-cephalus, mostly of later onset (Globus andBergman, 1946; case 1; Beckett et al., 1950, case8; Cassinari et al., 1963; Nag and Falconer,1966, case 8; Lerner et al., 1969; Jerva andNelson, 1971; Shallat et al., 1973; and Swash,1974); however, this reported association has notfound its way into the leading textbooks ofneuro-ophthalmology of Kestenbaum (1946),Cogan (1956), and Walsh and Hoyt (1969) andthe review articles of Holmes (1921), Smith et al.,(1959), Segarra and Ojeman (1961), and Hatcherand Klintworth (1966).The purpose of the present communication is

to report the presence, in association with acutehydrocephalus in children, of a continuum ofocular abnormalities which disappeared whenthe pressure was lowered by shunting procedures.Subsequent follow-up in these cases did notreveal any evidence of mass lesion. The mode ofpresentation and neuro-ophthalmological ob-servations of cases 2 and 3 in this series arereported below.

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Sylvian aqueduct syndrome as a sign of acute obstructive hydrocephalus in children

CASE 2

M.E., a white, male infant, was admitted to theMassachusetts General Hospital on 3 October 1968,for the first time for increasing lethargy, irritability,and vomiting of five days' duration. He was born ofa full-term normal pregnancy and was delivered byCaesarean section with a birth weight of 3-6 kg(7 lb 15 oz). The early neonatal period was un-remarkable.

Examination on admission revealed an irritablemale child with a head circumference of 45 cm (97thpercentile). The anterior fontanelle measured 5 x 5cm and was tense and bulging. Transillumination ofthe skull was negative. The chest, cardiovascularsystem, and abdomen were normal. The child waslethargic and irritable and had a high pitched cry. Hecould not sit and had poor head control. Visualfixation and following were absent and there was noblink to visual threat. The disc margins of opticfundi were blurred and venous pulsations were poor.Both eyes were directed downwards ('setting sun'sign). The lateral gaze was normal to the left side.There was inturning of the right eye and limitationof abduction. Opticokinetic nystagmus could not beelicited. The other cranial nerves were normal. Thechild moved all the extremities well. The deep tendonreflexes were bilaterally brisk and the plantar re-sponses were extensor.

It was concluded that the child had high intra-cranial pressure. He was put on intravenous manni-tol and steroid therapy and lumbar puncture wasdone carefully, which revealed an opening pressureof 330 mmH20. The closing pressure was 200mmH20. There were 65 erythrocytes and no whitecells in 1 mm3 of cerebrospinal fluid (CSF). TheCSF protein content was 48 mg and sugar 58 mg/dl. The other laboratory investigations, includingblood sugar, blood urea nitrogen, Na, K, Cl, andserum protein levels were normal.The skull radiographs were suggestive of diastasis

of sutures. The pneumoencephalogram revealeddilatation of both lateral ventricles, the 3rd and the4th ventricle. The cisterna magna was large. Most ofthe air remained trapped in the infratentorial com-partment suggesting communicating hydrocephalus.A ventriculoatrial shunt was installed on 16

October 1968. The anterior fontanelle became flatafter the shunt and the child appeared more alert butstill did not fix or follow visually and a question ofblindness was raised.The neuro-ophthalmological examination re-

vealed normal extraocular movements on lateralgaze, but paralysis of vertical gaze. The pupils were4-5 mm, equal, and reacting to light. Some degree ofopticokinetic nystagmus was present when the drum

was moved from right to left, suggesting that thechild could probably see. Visual evoked responsesdone the next day were normal. At this time thechild began to recognize his parents and thereaftercould fix and follow well, and the 'setting sun' signhad disappeared. He was sent home.The child was admitted again to the Massa-

chusetts General Hospital on 8 November 1969, withthe history of vomiting of two days' duration andlethargy. The anterior fontanelle was full. Underobservation he was intermittently irritable and un-responsive. The pupils now were 4-5 mm, equal, andsluggish in reacting to light. The extraocular move-ments, on oculocephalic reflex, were full on lateraland downward gaze but no upward movement couldbe elicited. Blockage of the shunt was consideredresponsible for the clinical state and shunt revisionwas done on 9 November 1968. The ventricular fluidpressure at the time of shunting was 240 mm CSF.The sensorium and extraocular movements im-proved after the shunt revision but on 19 November1968 the child again became more lethargic andstarted vomiting.The fundus examination revealed flame-shaped

haemorrhages on both sides. The child was againobserved to have paralysis of upward gaze and weak-ness of the right lateral rectus muscle. He lackedvisual fixation and following and there was no blinkto visual threat. The chest radiograph revealed dis-placement of the distal end of the shunt and replace-ment was considered. The ventricular pressure at thetime of shunting was more than 600 mmH20 andCSF was haemorrhagic. The child showed initialimprovement for a short time but again becamelethargic, refused to eat, and started vomiting. On 23November 1968 a shunt revision was done for thethird time and revealed occlusion of the ventricularend by fibrinous material. Even after the revision,the shunt was not functioning very well. There wereintermittent periods of raised pressure for whichfrequent lumbar punctures were done revealing highCSF pressures. The CSF contained 2,300 WBCs permm3, the majority being polymorphonuclear cells.The CSF sugar content was 25 mg, proteins 73 mg/dl, and the child was treated with antibiotics formeningitis. The CSF cultures were negative.A neuro-ophthalmological examination on 11

December 1968, revealed that the child did notrespond to light or visual threat. He moved his eyesfrom side to side, but not upwards. Lid retractionwas present, as was Bell's phenomenon. The rightpupil was 5 mm with sluggish reaction to light. Theleft pupil was 6 mm with no reaction to light. Bothoptic discs looked pale, the left showing more pallorthan the right. Opticokinetic nystagmus was absent.There was frequent, quick, episodic divergence of

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Amrik S. Chattha and G. Robert DeLong

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STROBE I I I Ii-Jl-IIIJ-fLA -A-A-AJ-AFIG. 1. Case 4. Electroencephalogram s -wevokedrespons-et p Ic stui

FIG. 1. Case 4. Electroencephalogram showing evoked response to photic stimulation.

the eyes, which would come back slowly to theprimary position, without any convergence nystag-mus.

The electroencephalogram (EEG) revealed irregu-lar 2-3 Hz slowing on both sides posteriorly withfrequent 1-2 Hz high voltage rhythmic bursts.Photic driving was present. The child gradually im-proved with frequent lumbar punctures and anti-biotic therapy. All the above ocular abnormalitiesdisappeared in one week.The next episode of blockage of the shunt occurred

at the age of 9 months and was again accompaniedby absence of following, fixation, and opticokineticnystagmus.The child was last seen at the age of 2 years, when

the shunt tube was lengthened. The child was alertand could follow objects and pick up toys. Theextraocular movements were intact and pupillaryresponses were normal.

COMMENT This child had communicating hydro-cephalus. The posterior fossa had a large cisternamagna or a cyst. He had four episodes of shuntobstruction all accompanied by ocular mani-festations characterized by the paralysis of up-ward gaze, dilated, poorly reacting pupils, andabsence of fixation and following responses.There was repeated weakness of the right lateralrectus muscle and one episode of divergence

nystagmus with paralysis ofupward gaze. Ocularabnormalities disappeared after shunting.

CASE 3

L.R. was a 1 week old Portuguese female child whowas first admitted to the Massachusetts GeneralHospital in April 1968 for neonatal seizures due toE. coli meningitis. She was born to a 21 year oldmother who had hypertension during pregnancy.The child was delivered by mid-forceps and sufferedleft lower facial paralysis as a consequence; thelatter disappeared in one week. The child's birthweight was 3-1 kg (6 lb 13 oz) and head circumfer-ence was 35 cm. The lumbar puncture at that timerevealed blood in the CSF. The cultures of urine,blood, and CSF grew E. coli and she was treated withampicillin. Because seizures had occurred she wasgiven phenobarbitone 7 5 mg twice daily and pheny-toin 5 mg three times daily. When discharged after 12days she was free of seizures.The child was admitted a second time to the

Massachusetts General Hospital at the age of 10weeks for evaluation of increasing head size. Thehead circumference was 42 cm (97th percentile) and a' cracked pot' sound was present on percussion.There was mild head transillumination. The lumbarpuncture revealed an opening pressure of 200mmH2O. The CSF protein was 150 mg and sugar30 mg/dl. The eyes were abnormal in that they

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STROBEFIG.____________-2C-a5.Ec s o i thph

FIG. 2. Case 5. Electroencephalogram showing the presence of evoked response to photic stimulation.

were directed straight ahead and vertical move-ments were nearly absent. In contrast, the lateralmovements were intact. The pupils were 6 mm andsluggishly reacting. The fundi were normal. Thelimbs moved normally and the deep tendon reflexeswere brisk but the plantar responses were extensor.A pneumoencephalogram revealed aqueductal

stenosis and possibly post-meningitic communicatinghydrocephalus. A ventriculoatrial shunt was done on2 May 1968. Thereafter she enjoyed good health andcontinued to develop but was somewhat slow for herage. There were no abnormal neurological findingsand extraocular movements were intact.

She was admitted to the Massachusetts GeneralHospital again in October 1970, at the age of 2 years9 months, for irritability of three days' duration and adraining sinus in the neck near the shunt catheter. Atthat time the neurological examination revealed herto be less alert and irritable. The head circumferencewas 48 cm. The fundi were normal and the extra-ocular movements were intact. The pupils were 4 mmequal and reactive. The other cranial nerves werenormal. The patient moved all the extremities well.The deep tendon reflexes were brisk, equal, and sym-metrical. The coordination was normal and thesensations were intact. The plantar responses were

bilaterally upgoing. The lumbar puncture revealed anopening pressure of 150 mmH2O and the CSF wasslightly xanthochromic. There were 2 lymphocytes

and 10 erythrocytes per mm3. The protein contentwas 110 mg and sugar 60 mg/dl. The CSF culturerevealed no growth. The urine culture grewGram negative rods and the blood culture grewStaphylococcus aureus and Clostridia perfringens.The skull radiographs and electrolytes were normal.It was decided that the shunt was infected so it wasremoved on 9 October 1970, and intravenous oxacil-lin 400 mg every four hours was started.On 13 October 1970, some leakage from the scalp

was noted and the child appeared less alert. Lumbarpuncture was done and revealed an opening pressureof 200 mmCSF with good relaxation. The proteincontent was 58 mg/dl and sugar was 64 mg/dl.There were five red cells and two polymorpho-nuclear cells per mm3. The next day the childbecame less alert and was lethargic. The pupilswere 4 mm and equal, but reacted sluggishly to light.The venous pulsations of the optic fundi werereduced. The cranial nerves were normal. There was

bilateral ankle clonus with extensor plantar reflexes.In the following hours she gradually became moreobtunded. Repeat lumbar puncture revealed anopening pressure of 180 mmCSF without any cellularresponse. The lateral movements were normal to thedoll's manoeuvre, but no upward movements couldbe elicited. The electroencephalogram revealeddiffuse 2-3 Hz arrhythmic polymorphic slowingbilaterally mixed with 4-5 Hz rhythmic theta waves.

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The slowing was more prominent posteriorly andslight reaction to photic stimulation was present.

It was concluded that the child had high intra-cranial pressure, hence a right frontal burr hole wasdone on the same day and emergency ventriculardrainage was done with Scott's cannula. The CSFdrained with a gush under very high pressure. Apneumoencephalogram done the next day demon-strated dilated lateral and third ventricles. The childbecame more alert but still did not follow and fixatewell. She had a blank stare with retraction of upperlids. The pupils were 6 mm, equal and reactedsluggishly. The fundus examination revealed sharpdisc margins.On 23 October 1970, the visual acuity was adequate

for seeing small objects and she was able to turn theeyes laterally and downwards but not upwards. Shedid not converge. The pupils were 6 mm each andsluggish in reaction. Opticokinetic nystagmus wasnormal horizontally. There were slow oscillationswith the drum moving upwards but retractionnystagmus occurred when the drum was moveddownwards. Bell's phenomenon was present. Thesefindings were considered consistent with lesions inthe periaqueductal grey matter of the upper mid-brain.The child gradually improved. Ventriculoperi-

toneal shunt was later done. The ocular abnormali-ties gradually disappeared in about a week.

COMMENT This child had two episodes of raisedintracranial pressure on the basis of aqueductalstenosis. In both episodes, there was paralysis ofupward gaze. The second episode was charac-terized by all the features of the Sylvian aqueductsyndrome. She also had blank stare with no fixingand following responses. These abnormalitiesdisappeared after shunting.

DISCUSSION

These eight patients presented various degreesof ocular disorder ranging from simple paresis ofupward gaze to the complete syndrome of theSylvian aqueduct (Tables 1 and 2). The paresis ofupward gaze was the common feature in all casesand the abnormalities of pupil and convergencemechanism were superimposed. Two patients(cases 1 and 6) had spasm of convergence alongwith upward gaze paralysis. A similar ocularabnormality has been described by Fisher (1967)in patients with thalamic haemorrhage where the

TABLE 1CLINICAL FINDINGS IN EIGHT PATIENTS

No. Name Age Sex Aetiology of raised CSF Upward Convergence Lid Nystag- Doll's headICP* pressure gaze retrac- mus manoeuvre

(mmH20) palsy tion retrac-torius

1 L.F. 7 wk F Posterior fossa cyst 340 + Spasm (eyes + - No responsemaintained inand down)

2 M.E. 5 mo. M Large cisterna 600 at ven- + Episodic diverg- + - No responsemagna, posterior tricular tap ence move-fossa cyst? ments

3 L.R. 29/12 yr F Post-meningitic 180 at L.P. + Absent + + Lateral andhydrocephalus; and very down gazeaqueductal high at ven- presentstenosis tricular tap

4 M.D. 16 yr F Aqueductal stenosis - + Absent - + Lateral andpost-op. subdural down gazehaematoma present

5 H.B. 34 yr M Haemophilus influenza 600 + Poor - - Lateral andmeningitis down gaze

present6 A.B. 2 yr M Tuberculous menin- 400 + Spasm (eyes + - No response

gitis maintained inand down)

7 C.P. 5 d F Aqueductal stenosis - + Not possible to + - Lateral gazetest present

bilaterally8 P.B. 2 yr M Aqueductal stenosis - + Poor - + Lateral and

with shunt obstruc- down gazetion present

* ICP: raised intracranial pressure.

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TABLE 2VISUAL AND PNEUMOENCEPHALOGRAPHIC FINDINGS IN EIGHT PATIENTS

No. Name Age Sex Pupil Fundus Visual VER* OKN+ Pneumoencephalogram(mm) function

1 L.F. 7 wk F 2. Both sides Blurred disc Does not fix +L.R. poort margins or follow

2 M.E. 5 m M 5. Both sides Blurred disc Does not fix +L.R. sluggish margins or follow

3 L.R. 2 9/12 a F 5. Both sides Absent Does not fixL.R. sluggish venous or follow;

pulsations corticalblindness?

Photic drivingpresent

Absent Combined lumbar AEG andventriculogram revealedlarge posterior fossa arach-noid cyst; obstructivehydrocephalus, due to ob-struction, at rostral 4thventricle level

Absent Lumbar AEG shows dilata-tion of lateral, 3rd and 4thventricles with largecisterna magna. Impressionwas communicating hydro-cephalus

Nystagmus Ventriculogram revealedretractor- aqueductal stenosis andius on possibly also post-menin-downward gitic communicatingdrum hydrocephalus

4 M.D. 16 a F 5. Both sides BilateralL.R. sluggish papill-

oedema

5 H.B. 34 a M 4. Both sidesL.R. present

6 A.B. 2 a M 2. Both sidesL.R. poor

7 C.P. 5 d F 2. Both sidesL.R. present

8 P.B. 2 a M 5. Both sidesL.R. sluggish

Vacant stare;corticalblindness?

Ph

Venous Does not fix Phpulsations or follow preabsentAbsent Does not fix VE

venous or follow r

pulsationsand hazydisc mar- c

ginsPallor of Does not fix VE

optic discs or follow c

bilaterallyVenous en- Does not fix +gorgement or followpresentwith absentpulsations

rotationotic driving Nystagmuspresent retrac-(Fig. 1) torius on

downwardrotationof drum

iotic driving Absentesent(Fig. 2)ER asym- Absentmetrical.Loweramplitudeon left side

ER notdone

Ventriculogram revealedaqueductal stenosis

No air study done

No air study done

Absent Ventriculogram revealedaqueductal stenosis

Poor re-sponse onlateralrotationand nystag-mus re-tractoriuson down-ward drumrotation

Ventriculogram revealedaqueductal stenosis

* VER: visual evoked response. f L.R.: light reflex. I OKN: opticokinetic nystagmus.

subject peers at the nose. One patient (case 2)has quick episodic divergence movements of theeyes followed by slow return to the neutralposition. This patient could be considered tohave divergence nystagmus, though exophoriaresulting from momentary lack of fixation couldnot be ruled out completely. Cases 3, 4, and 8exhibited all the characteristic features of theSylvian aqueduct syndrome: paralysis of up-ward gaze, dilated poorly reacting pupils, andretraction nystagmus on attempted upward gaze.The disorder of ocular movements was not

explained by the reduced level of consciousnessand poor cooperation on the part of thesepatients, a point which must be taken into con-sideration, since it is well known that obtundedand uncooperative patients have more difficultyin looking up than to the side or down.

Impairment of visual acuity was detected in allof these patients. Although the patients made nocomplaints of blindness, they did not appear tolook at or recognize objects or faces and opto-kinetic nystagmus was absent or poor. However,some preservation of visual function was evi-

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denced by the presence of computer averagedvisual evoked responses and photic driving ofthe EEG (Figs 1, 2). This visual loss was alsounrelated to the level of consciousness. Lorber(1967) had also reported temporary blindness inhydrocephalic children during a crisis of intra-cranial hypertension with complete recovery ofvisual function after shunting. This visual loss hasbeen variously attributed to compression of theoptic chiasma from the distended third ventricle(Dott, 1936), ischaemia ofthe occipital lobes fromdistortion of the posterior cerebral arteries(Lindenberg, 1955), and interstitial retrobulbarneuritis (Walsh and Hoyt, 1969). None of thesehypotheses has been fully substantiated by patho-logical study. We suggest that the apparent'blindness' may be primarily the consequence ofparalysis of the reflexes of fixing and following,rather than impairment of visual sensory path-ways per se; this is supported by the finding ofpreserved visual evoked responses. Myers (1964)observed similar visual behaviour in cats afterstereotactic lesions in the superior colliculus,ventral periaqueductal grey matter, and theventral tegmentum: 'These animals were sittingand staring straight ahead. The normal highlyaccurate movements of fixation and followingwere completely abolished, so that animalsfailed to move either head or eye to seek or fol-low objects of interest brought into view'. Assimilar visual behaviour was observed in ourpatients, one can speculate that dysfunction insuperior colliculus, ventral tegmentum, and peri-aqueductal grey matter was probably responsiblefor this visual behaviour as well as paralysis ofupward gaze and various abnormalities of con-vergence observed in these patients.Of course the 'setting sun' ocular disturbance

is a familiar accompaniment of hydrocephalus(Paine and Oppe, 1966; Ford, 1969; Matson,1969). This refers to a downward turning of theeyes and retraction of upper lids (Collier's sign).The latter, when on a neurogenic basis, is con-sidered to be due to a lesion of the posteriorcommissure (Collier, 1927). This, when com-bined with upward gaze palsy, would explain thepathogenesis of the 'setting sun' sign on thebasis of periaqueductal dysfunction rather thanthe fanciful explanation ofdownward pressure ofthe expanded frontal lobes on the roof plates ofthe orbit (Ford, 1969; Matson, 1969).

Analysis of these patients revealed that thevertical gaze paralysis and the other ocular andvisual disturbances were seemingly connected insome manner with the expansion of the ventricu-lar system (pressure hydrocephalus), worseningas it progressed, lessening and finally disappearingas it was relieved by effective shunting. Thedisease which caused hydrocephalus and whichvaried from case to case was only indirectlyresponsible since it remained after the hydro-cephalus was controlled. Cassinari et al. (1963),Walsh and Hoyt (1969), and recently Swash(1974) also postulated dilatation of the thirdventricle and rostral aqueduct as the possiblemechanism for upward gaze palsy observed inhydrocephalus. However, Lerner et al. (1969),Jerva and Nelson (1971), and recently Shallat etal. (1973) have postulated compression of thetectal and pretectal region from the distendedsuprapineal recess as the possible mechanism.The presence of other features of the Sylvianaqueduct syndrome along with upward gazepalsy in several of our cases would suggest peri-aqueductal dysfunction rather than the com-pression of the tectal region. It is also well known(Cogan, 1956) that in cases of pinealoma theparalysis of upward gaze disappears when shunt-ing relieves the hydrocephalus caused by thetumour. Since the tumour was still there, thedirect compression of the tectal region from itcould not have been responsible for the gazepalsy. Moreover, it is now known through thestudies of Spiegel and Scala (1937), Christoff etal. (1960), Segarra and Ojeman (1961), andNashold and Gills (1967) that the mechanism forvertical gaze is in the tegmentum of the uppermesencephalon and not in the superior collicularregion.

If dilatation of the ventricular system causesthese ocular and visual phenomena, one mustinquire into its mechanism. The organization ofall these functions is known to reside just anteriorto the aqueduct of Sylvius and below the posteriorpart of the third ventricle. The centre of organi-zation of downward gaze is caudal to that ofthe upward gaze. The susceptibility of theseeye signs to hydrocephalic expansion varies butalways seem to appear in an orderly fashion,upward gaze failing before the downward andthe lateral gaze. The pupillary dilatation and theparalysis of the light reflex tend to occur when

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the hydrocephalus is advanced and at times whenthe level of consciousness is usually impaired.The depression of consciousness, seen in thefull-blown hydrocephalic syndrome, may resultfrom compression of the midbrain reticularsystem as the dysfunction extends beyond theimmediate periaqueductal region. One mightpostulate a gradient of force from the expandingaqueduct and the third ventricle which mustdiminish from the ependyma outwards anddifferentially stretch and compress the neuronalfibre systems at varying distances from theventricle and aqueduct. A nice point in thepathophysiology ofventricular dilatation is raisedfrom a more practical side: the ocular disordersmay be numbered among the more delicatemanifestations of hydrocephalic paralysis ofcerebral function.Swash (1974) suggested that these ocular

abnormalities were more likely to occur inhydrocephalus due to aqueductal stenosis thanin the communicating variety. Most of our casesdid have aqueductal stenosis; however, our case2, with no evidence of aqueductal stenosis, stilldeveloped all these ocular abnormalities. Wetend to believe that it is the acuteness andseverity of the hydrocephalus rather than the siteof block which is the determining factor. Lack ofthese ocular abnormalities in adult-onset com-municating hydrocephalus is not surprising,since most of these cases have normal or lowcerebrospinal fluid pressure (Hakim and Adams,1965).

Finally, it should be remembered that upwardgaze palsy and the other ocular, pupillary, andvisual disturbances described in our patientshave proved to be valuable indices of raisedintracranial pressure, particularly in cases wherehigh pressure in the ventricular system is notreflected in the lumbar subarachnoid space (case3). The eye signs should always be looked forwhen a child with a shunt becomes drowsy orapathetic, disinterested in food, or nauseated, orif older children complain of headache or un-steadiness of gait. If the ocular signs appearrapidly, they signal a crisis of intracranialhypertension that demands urgent surgicalintervention. At the same time their recognizedoccurrence in conjunction with a diversity ofdiseases of variable anatomical topography de-prives them of localizing value, with reference to

the causative disease. They belong, in thelanguage of Collier (1904), to a large group of'false localizing signs' in neurology.

The authors are grateful to Dr Raymond D. Adamsand Dr David G. Cogan for their kind criticism andsuggestions and to Mrs Pamela Picariello forsecretarial assistance.

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Cassinari, V., Pagni, C. A., and Vitale, A. (1963). Sindromedi Parinaud in corso di idrocefalo cronico iperteso:sofferenza della regione mesencefalica da dilatazione dellaporzione posteriore del III ventricolo? Minerva Neuro-chirurgica, 7, 84-88.

Christoff, N., Anderson, P. J., and Bender, M. B. (1960).Convergence and retractory nystagmus. Transactions ofthe American Neurological Association, 85, 29-32.

Cogan, D. G. (1956). Neurology of the Ocular Muscles, pp.123 and 126. Thomas: Springfield, Ill.

Collier, J. (1904). The false localising signs of intracranialtumour. Brain, 27, 490-508.

Collier, J. (1927). Nuclear ophthalmoplegia, with especialreference to retraction of the lids and ptosis and to lesionsof the posterior commissure. Brain, 50, 488-498.

Dott, N. (1936). Bitemporal hemianopia. British MedicalJournal, 2, 296.

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Ford, F. R. (1969). Diseases of the Nervous System in Infancy,Childhood and Adolescence, 5th edn, p. 75. Thomas:Springfield, Ill.

Fredericks, E. J., and Van Nuis, C. (1967). Diverticulum ofthe rostral cerebral aqueduct with ocular dysfunctions.Archives of Neurology, 16, 32-36.

Globus, J. H., and Bergman, P. (1946). Atresia and stenosisof the aqueduct of Sylvius. Journal of Neuropathology andExperimental Neurology, 5, 342-363.

Hakim, S., and Adams, R. D. (1965). The special clinicalproblem of symptomatic hydrocephalus with normalcerebrospinal fluid pressure. Journal of the NeurologicalSciences, 2, 307-327.

Hatcher, M. A., Jr, and Klintworth, G. K. (1966). Thesylvian aqueduct syndrome. A clinicopathologic study.Archives of Neurology, 15, 215-222.

Holmes, G. (1921). Palsies of the conjugate ocular move-ments. British Journal of Ophthalmology, 5, 241-271.

Jerva, M. J., and Nelson, R. (1971). Parinaud's syndrome inprogressive hydrocephalus. Mercy Medical Journal, 1,19-20.

Kestenbaum, A. (1946). Clinical Methods ofNeuro-ophthalmo-logic Examination, p. 255. Grune and Stratton: New York.

Lerner, M. A., Kosary, I. Z., and Cohen, B. E. (1969).Parinaud's syndrome in aqueduct stenosis: its mechanismand ventriculographic features. British Journal ofRadiology,42, 310-312.

Lindenberg, R. (1955). Compression of brain arteries aspathogenic factor for tissue necroses and their areas ofpredilection. Journal of Neuropathology and ExperimentalNeurology, 14, 223-243.

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