PSEUDOTUMOR CEREBRI

(1)

PSEUDOTUMOR

CEREBRI

By Ralph Bryan Moore, M.D.

Department of Pediatrics, New York Hoital-Cornell Medical Center

(Submitted June 8, accepted August 21, 1956.)

PRESENT ADDRESS: 15 North 5th Street, Wilmington, North Carolina.

266

I

N THE past year, three patients were

admitted to the Children’s Service of

The New York Hospital with similar

histo-ries and clinical findings characteristic of an

expanding lesion in the posterior fossa. The

ultimate diagnoses were similar, and

un-usual-pseudotumor cerebri. Because of the

meager amount of information in the

pedi-atric literature on this syndrome, it seemed

worthwhile to report these three cases, sum

up the experience with three other children

over the past 15 years, and to review the

subject in detail.

Case 1

CASE REPORTS

HISTORY: R.S. was an 8-year-old white boy

who entered the hospital with the chief

com-plaint of double vision of 2 weeks’ duration.

Two and one-half weeks prior to admission he

complained of pain in the right ear, and had

intermittent frontal headaches. The bocal

physi-cian noted a temperature of 37.8#{176}Cand signs

of a respiratory infection. The patient was

given a sulfonamide preparation, with an

ap-parentby good response over the next few days.

Twelve days prior to admission he complained

of double vision when looking to the left. An

ophthalmologist noted paresis of the left lateral

rectus, paresis of the left bevator pabpebrae

and mild papilledema. These were considered

to be resolving, and the patient was placed on

bed rest for the next week. The dipbopia,

how-ever, persisted and ‘repeat ophthalmoscopic

examination 4 days prior to admission revealed

increased papilledema. There was no history

of gait disturbance, vomiting, or headaches

over the 2-week period prior to admission.

Pmsrci Fnmss : Examination revealed

a temperature of 37.6#{176}C, pulse of 94/mm,

respiratory rate of 24/mm, and blood pressure

of 112/74 mm Hg. The patient appeared

slightly lethargic, but otherwise seemed well.

Positive physical findings included injection

and scarring of the left tympanic membrane,

bilateral ptosis, paresis of the left lateral

rec-tus, and bilateral obliteration of the optic disc,

with the presence of hemorrhages and exudate.

The deep tendon reflexes and tests for

cere-bellar function were normal. Visual fields were

markedly constricted bilaterally, and acuity

tests indicated a vision of 20/20 on the right

and 20/30 on the left. Roentgenograms of the

skull and electroencephalogram were

inter-preted as normal. Ventricubograms showed

slightly dilated lateral ventricles with good

filling of the third ventricle, suggesting a

p05-tenor fossa block. The concentration of

pro-tein in the cerebral spinal fluid was 14 mg,”

100 ml.

CouitsE : At exploratory craniotomy no tumor

could be found. Biopsy of the meninges and

cerebellum revealed a fibrous thickening of the

arachiioid membrane, suggesting arachnoiditis.

Postoperatively the optic fundi showed

in-creasing scarring and edema, and repeat tests

of visual acuity 12 days after operation showed

a diminution of acuity to 20/200 on the right

and 18/200 on the left. Lumbar puncture

per-formed because of these progressing signs,

showed an initial pressure of 220 mm of

water, and 40 ml of spinal fluid were

re-moved. Over the next 2 weeks lumbar

punc-ture was repeated on five occasions, the last

two revealing normal pressure levels. Over this

period papilledema and venous engorgement

of the retinal vessels diminished considerably.

Visual acuity remained about the same, but

paresis of the left lateral rectus and dipbopia

disappeared. The patient was discharged 1

month after operation and has continued to do

well, except for some residual loss in visual

acuity because of glial proliferation about the

optic discs. The most recent tests show an

acuity of 20/70 on the right, 20/100 on the

left. Visual fields show improvement over the

postoperative examination. The patient is on

full activity, and by sitting in the front row in

school has no difficulty, without glasses.

Case 2

HISToRY: J.R., an 89i2-year-old white boy,

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head-aches and diplopia. Fifteen days before

admis-sion he had fallen and struck his forehead on

the floor. There was no loss of consciousness,

and no immediate symptoms, but the next day

he complained of frontal headache. This

per-sisted intermittently over the next 2 weeks,

usually being relieved by acetylsalicylic acid.

Nausea and vomiting accompanied the

head-aches. One week prior to admission an

ophthal-mobogist observed papibledema and retinal

hem-orrhages bilaterally. Four days prior to

admis-sion, dipbopia on forward gaze was noted.

Al-though the headaches became less severe, the

patient was referred to the hospital with the

suspicion of brain tumor. The only other

symptom was intermittent shooting pain in the

right arm. There was no fever, dizziness, or

gait disturbance.

PHYSICAL FINDINGS : Examination revealed

a temperature of 37.4#{176}C, pulse of 80/mm,

respiratory rate of 25/mm, and blood pressure

of 130/75 mm Hg. The positive findings

in-eluded palsy of the left lateral rectus muscle,

dipbopia in all directions but right lateral gaze,

papilledema of 2 to 3 diopters bilaterally, and

pain radiating down the right arm on turning

the head to the right. An electroencephalogram

was read as being within normal limits, with

no suggestion of a neoplasm. Roentgenograms

of the skull were considered to be normal, and

visual studies showed no diminution in visual

fields or in acuity. The neurosurgicab

consult-ant advised immediate ventricubograms which

were completely normal. A sagittab sinus

veno-gram, undertaken to rule out thrombosis

see-ondary to head trauma, was unremarkable, and

operation was postponed. The tentative

diag-nosis was pseudotumor cerebri. Concentration

of protein in the ventricular fluid was 17 mg/

100 ml.

CourtsE : The patient was observed cbosely

over the next 2 weeks, the only medication

being prophylactic antibiotics. There was

grad-ual disappearance of the palsy of the left

lateral rectus muscbe, diplopia, and

papil-bedema. The patient was placed on full activity

and discharged after 2% weeks, the only

resid-ual sign being papilledema of 1 to 2 diopters.

One month after discharge, papilledema had

completeby disappeared. The patient has

con-tinued to remain well.

Case 3

HISTORY: E.S. was a 6-year-old white male,

who entered with a history of headache for 3

weeks, and a shorter period of nausea,

vomit-ing and eventually diplopia. Twenty-three days

prior to admission, he fell from a bicycle,

strik-ing the left frontal area on a rock. There was

no boss of consciousness, but the patient

com-plained of frontal headache, and was slightly

lethargic. Ten days before admission, nausea

and vomiting were noted. The symptoms

per-sisted, and 3 to 4 days before admission,

irrita-bility and lethargy became more pronounced.

Dipbopia appeared 1 day later.

PHYSICAL Fuwcs: On admission, the

tem-perature was 37.8#{176}C, pulse, 70/mm,

respira-lions, 18/min, and blood pressure 120/76 mm

Hg. The patient was irritable and moderately

drowsy. He complained of headache and

pre-ferred to remain lying down. The significant

physical findings were a left pupil slightly

greater than the right, papilledema of 2 to 3

diopters bilaterally with retinal hemorrhages

on the left, and hyperactive left ankle jerk with

diminished abdominal and cremasteric reflexes

on the left. Visual acuity was normal.

Roent-genograms of the skull were normal. An elec-troencephabogram showed a pathologic

rec-ord in the occipital areas, the left more so than

the right. Ventricubograms were within

nor-mal limits, and no additional procedures were

done. The concentration of protein in the

yen-tricubar fluid was 10 mg/100 ml.

COURSE : Postoperatively, the patient showed

great improvement, with boss of all symptoms

in the course of a few days. The papilledema

remained stationary, but the venous

engorge-ment disappeared rapidly. A repeat

electro-encephalogram 1 week later showed much

improvement. A presumptive diagnosis of

pseu-dotumor cerebri was made, and the patient is

being followed closely.

In addition to these three patients, over

the

past

15 years three other children have

fulfilled the criteria for the diagnosis of

pseudotumor cerebri. A.W. was a

14-year-old white female whose outcome was good.

Now, some 13% years later, she is essentially

well but requires phenobarbital, 0.1 gm

twice daily; her electroencephalogram

shows a seizure disorder of the mixed type.

J.I. was a 4-year-old white male, who

cx-pired

1 month after admission to the

hospi-tal. The third patient was C.K., a

76,42-year-old white male, who expired 6 months after

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over-268 MOORE PSEUDOTUMOR CEREBRI

TABLE I

PRESENT SERIES OF PATIENTS

P&ients A.W. J.I. C.K. JLS.J.R.

‘

ES.

Sex Female Male Male Male Male Male

Time of admission November

194

April

1948

September

1931

March June April

1955 1955 1956

8 yr 8, yr

‘

6 yr

2 wk wk , 3 wk

Coo(l Good Good

Age at onset 14 yr 4 yr 7 yr

Duration of symptoms prior to admission

10 days 4 wk Q yr

Result Good Expired

after 1 mo

Expired* after 6 mo

Length of follow-up 13, yr - - 14 mo 11 mo I mo

* Meningitis due to Pseudomonas aeruginosa.

whelming meningitis due to Pseudomonas

aeruginosa. These cases, plus the three

re-ported above, have been grouped in Table

I.

DISCUSSION

These patients classically illustrate the

syndrome listed by Ford’ as pseudotumor

cerebri. In 1897, Quincke2 first described

it as “serous meningitis” and it became

known by that name or Quincke’s

meningi-tis. In the early 1900’s, fibrinous changes in

the arachnoid membrane, especially in the

cisternal area, were noted, and the term

arachnoiditis came into use. In further

attempts to associate the condition with a

possible etiology, Symonds3 in 1931,

de-scribed three cases of otitic hydrocephalus,

and McAlpine4 in 1937, reported five cases

of toxic hydrocephalus, the toxic

manifes-tations being secondary to nasopharyngeal

infections. To the neurosurgeons goes the

credit for the term pseudotumor cerebri,

associating the clinical picture with the

operative findings. Finally, because of the

multitude of terms for the syndrome, and

because the etiology remains obscure, the

most recent terminology has been

“intra-cranial hypertension of unknown

All the above terms recognize the basic fact

that there is an excessive amount of spinal

fluid in the subarachnoid space, with the

formation of small cysts in the arachnoid

membrane. Of all the terms, probably

pseu-dotumor cerebri is the most appropriate,

except in those cases where definite

patho-logic changes can be demonstrated in the

arachnoid membrane, when the term

arach-noiditis becomes more specific.

As mentioned before, the etiology of this

syndrome remains obscure in most

in-stances. Sometimes an antecedent mild

febrile illness of unknown origin may be

im-plicated; at other times such illnesses as

otitis media, sinusitis, nasopharyngitis, or

ocular infections; other implicated

precur-sors include cranial trauma and secondary

hemorrhage, spinal anesthesia, and infected

contusions of the scalp, but these are all

speculative and inconstant. There have

been reported cases of arachnoiditis, with

pseudotumor cyst formation, following

acute bymphocytic choriomeningitis,8

there-by revealing at beast one demonstrated

etio-logic factor. Ray and Dunbar,7 in 1951,

and Ford’ speculated on another possible

cause-that of an unsuspected dural sinus

thrombosis, occurring especially in those

cases preceded by otitis media. In

sum-mary, present day knowledge leaves the

precise etiology unsolved, although many

contributing factors may play a role.

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TABLE II

PRESENTING SIGNS AND SYMPTOMS

Patients A.JV. J.I. C.K. R.S. JR. ES. Totals

headache + + + ± + + 6/6

Vomiting + + 0 0 + + 4/6

1)iplopia 0 + 0 + + + 4/6

Gait disturbance 0 + + 0 (1 + 3/6

Lethargy 0 + 0 ± 1) + 3/6

Papilledetna (1 + + + + + 5/6

Cranial nerve palsies 0 + 0 + + ± 4/6

Antecedent history 0 0 0 _+* _{+ t} + t 3/6

Fever 0 #{149}0 + o o o 1/6

Convulsions + 0 0 0 0 0 1/6

0-absent

±-suggestive history +-present

* Otitis media, pharyngitis t Head trauma

our six patients are compiled in Tabbe II.

These compare closely with the description

presented by 8 The clinical picture

is one of increased intracranial pressure, as

manifested by headache, vomiting,

di-plopia, papilledema, and drowsiness. There

is usually an absence of focal signs,

al-though palsies of cranial nerves V, VI and

VII do occur. The patient is generally

afebrile. If the papilledema is sufficiently

severe and of long standing, visual acuity

may be affected, indicating a secondary

optic atrophy. Occasionally there may be

dizziness and unsteadiness, but convulsions

are rare. It is noteworthy that all of the

following signs or symptoms occurred in

at beast one-half of the six patients in our

series: headaches, vomiting, diplopia, gait

disturbance, cranial nerve palsies and

papilledema. Ventriculograms usually reveal

small or normal-sized ventricular systems,

and the spinal fluid, although excessive in

amount, has neither an increased cell count

nor an elevation in the protein content.

The arachnoid cysts are filled with clear

fluid and surrounded by membranes

re-sembling the arachnoid, but thicker and

more opaque. There may be some fibrosis,

indicating a mild inflammatory process,

but more often the meninges appear

nor-mab. The laboratory and operative data for

the six patients being reported are

sum-marized in Table III. Here again, with the

exception of the frequency of abnormal

ventricubograms, the laboratory picture fits

that described by various 6 In

the present series there were abnormal

ventricubograms in three of the five patients

in whom they were done. In one

(

R.S.)

there was minimal dilation of the lateral

ventricles, and in the other two the

arach-noiditis was so severe that obstruction in

the area of the cisterna magna was present.

It should be stressed at this point that,

unless ventricubograms and/or cranial

cx-ploration are carried out, it is impossible to

make a definite diagnosis of pseudotumor

cerebri. One must rule out brain tumor.

The prognosis in cases of pseudotumor

cerebri is generally fairly good. From Table

I it is noted that four of the six patients

have done well. The majority of cases, both

in the present series and in the literature,

fall into the acute type, with gradual

sub-sidence of symptoms over a period of a

few weeks to a few months. Management

of these patients consists of measures

pro-moting dehydration and repeated lumbar

punctures to protect against secondary

optic atrophy. Sahs and Hyndman6

recom-mend daily lumbar punctures for pressures

over 250 mm of water. In addition,

sub-temporal decompression may be used to

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270 MOORE PSEUDOTUMOR CEREBRI

TABLE 111

LABORATORY DATA

PatienLs A.W. J.I. C.K. R.S. JR. ES. ToIaLi

Loss in visual acuity 0 0 0 + 0 0 1/6

Abnormal roentgenograms 0 + + 0 0 0 l/6

of skull

Abnormal EEG + + + 0 0 + 4/6

Abnormal ventriculograins + Not Done + ± 0 0 3/5

Elevated CSF protein 0 0 0 0 0 0 0/6

Tumor found on explora- 0 0 0 0 Not Done Not l)one 0/4

tion

Arachnoiditis on explora- + + + + Not Done Not I)one 4/4

‘ tion (Severe) (Severe)

0-absent

±-suggestive or minimal

+-present

some patients in whom the pressure never

falls with the above measures, and these

are considered to be chronic cases. The

de-compression site bulges occasionally, but

there is no tendency toward progressive

optic atrophy. Statistics from the literature

bear out the favorable prognosis in most

cases. Horrax9 in 1924, reported 33 cases

from the Peter Bent Brigham Hospital; all

patients except 5 did well, and this

in-eluded 19 cases classified as chronic, in

terms of duration of illness. Frazier’#{176} in

1930, reported 22 cases from Philadelphia,

of which, all except 3 did webb. Finally,

Zuidema and Cohen’1 in 1954, reported a

long-term follow-up on 12 patients

origi-nally reported in 1937 by Dandy.’2 Ten of

the twebve were doing well. In addition,

the authors reported on 58 patients seen at

The Johns Hopkins Hospital between 1937

and 1953 that met the criteria for the

diag-nosis of pseudotumor cerebri. Four

subse-quently proved to have a brain tumor, and

19 others were eventually found to have

some other cause for the increased

intra-cranial pressure, leaving a total of 35 of the

58 that did well.

SUMMARY

The case records of three patients with

the diagnosis pseudotumor cerebri are

pre-sented.

In addition, data from the records of

three other children are included to give

a series of six patients.

The clinical picture and the laboratory

data are summarized and compared with

other reports.

A review of the literature is given, with

emphasis on the nomenclature, etiology,

clinical findings and prognosis.

REFERENCES

1. Ford, F. R. : Diseases of the Nervous Sys-tern in Infancy, Childhood and Adoles-cence. Springfield, Thomas, 1952, pp.

536 and 900.

2. Qumncke, H. : Ueber Meningitis serosa und

verwandte Zust#{228}nde. Deutsche Ztschr.

Nervenh., 9:149, 1897.

3. Syrnonds, C. P. : Otitic hydrocephalus. Brain, 54:55, 1931.

4. McAlpmne, D. : Toxic hydrocephalus. Brain,

60:180, 1937.

5. Ellcins, C. W., and Rack, F.

J.

: Cerebral pseudotumor or intracranial hyperten-sion of unknown cause, report of 3 cases.

New England

J.

Med., 244:171, 1951.

6. Sahs, A. L., and Hydnman, 0. R. :

Intra-cranial hypertension of unknown cause;

cerebral edema. Arch. Surg., 38:428,

1939.

7. Ray, B. S., and Dunbar, H. S. :Thrombosis

of the dural venous sinuses as a cause

of “pseudotumor cerebri.” Ann. Surg.,

134:376, 1951.

8. Barker, L. F., and Ford, F. R. : Chronic arachnoiditis obliterating the spinal

sub-arachnoid space. J.A.M.A., 109:785,

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9. Horrax, G. : Generalized cisternal arach-noiditis simulating cerebellar tumor; its surgicab treatment and end-results. Arch.

Surg., 9:95, 1924.

10. Frazier, C. H. : Cerebral pseudotumors. Arch. Neurol. & Psychiat., 24:1117,

1930.

11. Zuidema, G D., and Cohen, S.

J.:

Pseudo-tumor cerebri.

J.

Neurosurg., 11:433, 1954.

12. Dandy, W. E. : The brain, in Lewis’

Prac-tice of Surgery. Hagerstown, Prior, 1945,

p. 435.

SUMMARIO IN INTERLINGUA

Pseudotumor

Cerebral

Es presentate in detabio be protocolbos de tres patientes admittite in be curso de un anno

al Servicio Juvenil del Hospital New York.

Lor diagnoses esseva simile e similemente

in-usual: pseudotumor cerebral. In plus, in le

curso del passate 15 annos, tres juveniles

addi-tional satisfaceva be criterios del diagnose de

pseudotumor cerebral. Illes es inebudite in be

presente serie que assi consite de sex casos.

Tabuba I analysa le serie quanto al

distribu-tion sexual (5 mascubos, 1 feminina), al etate al

tempore del declaration del morbo (4 a 14

an-nos), al duration del symptomas ante be

admis-sion (2 septimanas a 23 annos), al resubtato

final (4 vive e ben, 2 morte: 1 per meningitis

a Pseudomonas aeruginosa sex menses plus tarde), e al periodo del observation consecutori

(

1 mense a 13 annos).

Altere terminos usate previemente pro iste

syndrome es meningitis serose, meningitis de

Quincke, arachnoiditis, hydrocephalo otitic,

hy-drocephabo toxic, e hypertension intracranial

a causa incognite. Super le base del stato presente de nostre cognoscentias, be termino

pseudotumor cerebral pare le plus satisfactori

in tanto que illo associa le aspectos clinic con be

constatationes operatori.

Le exacte etiobogia remane indeterminate,

ben que mubte factores ha essite adducite. Istos

include: Antecedente morbos febril de leve

grados e non-cognoscite origine; morbos como

otitis medie, sinusitis, nasopharyngitis, o

infec-tiones ocular; trauma cranial e hemorrhagia

secundari; anesthesia spinal; infection de

con-tusiones epicranial; acute choriomeningitis

bymphocytic; e non-suspicite thrombosis del

sino dural.

Le signos e symptomas de presentation in

nostre sex patientes es colligite in Tabula II.

Ilbos se trova in stricte correlation con

descrip-tiones in be bittertura. Omne le sequente signos

e symptomas se trovava in al minus un

medietate del sex patientes : Mal de capite;

vomito; diplopia; disturbationes del

ambuba-tura; lethargia; papilledema; e paralyse de

nervos cranial. In duo casos, trauma del capite

precedeva be morbo. In un patiente, otitis

medie precedeva le declaration del symptomas.

Febre e convulsiones es usualmente absente;

illos occurreva un vice cata un in be serie de

sex patientes.

Tabula III representa un summario del datos

laboratorial e operatori in be presente sene. Con

le exception del frequentia de

ventricubogram-mas anormal (3 ex 5), be datos tabulate se

correlationa con be datos del littertura.

Usual-mente, ventricubogrammas reveba ventriculos

normal o mesmo subdimensionate. Acuitate

visual non se perde (1 ex 6), anormal

roent-genogrammas cranial occurre infrequentemente

(2 ex 6), be proteina del fluido cerebro-spinal non es elevate (nulbo ex 6), e nulle tumor es

trovate per exploration (nub ex 4). Es a

sublinear que sin ventricubogramma e/o

ex-pboration cranial, il es impossibibe establir

definitivemente un diagnose de pseudotumor

cerebral. Le possibilitate de tumor cerebral

debe esser eliminate.

Le prognose in casos de pseudotumor

cere-bral es generalmente satis favorabile. Isto es

corroborate per 4 del 6 patientes in be presente

serie. Simile constatationes es reportate in be

litteratura. Le majoritate del casos representa

un typo acute, con regression gradual del

symptomas in be curso de alicun septimanas o menses. Le tractamento consiste in mesuras

dishydratatori, insimul con repetite puncturas

lumbar como protection contra le

disveboppa-mento secundari de atrophia optic. In plus,

discompression subtemporal pote esser

em-pleate pro arrestar be progresso de papilbedema.

Certe patientes ha chronic ebevationes del

pression de fluido spinal in despecto del

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1957;19;266

Pediatrics

Ralph Bryan Moore