University Clinic of

Schleswig-Holstein

Section Neuroradiology

Campus Kiel

Objectives: To distinguish between idiopathic and secondary intracranial

hypertension by the means of cranial MRI and MR-venography and to use MRI in

the follow up.

Methods: 14 patients (age 20-68, mean 39,5 y) with typical signs and symptoms

of pseudotumor cerebri (PC) were prospectively enrolled (table1). Two patients

were thought to have secondary intracranial hypertension (SIH) due to impaired

cranial venous outflow, one sufferd from a meningioma occluding the left transverse sinus (No.4), the other had an occlusion of the left jugular vein after an

operation (No.14). The other 12 patients were classified as idiopathic intracranial

hypertension (IIH). Cranial MRI with special emphasis an the optic nerve sheaths

and the pituitary and phase contrast venous MR-angiography (MRV) was

performed when increased CSF pressure was documented by lumbar puncture (LP, mean CSF pressure 34 cm H2O ± 6,9). A second MR examination was done

after elevated CSF pressure was reduced to normal levels by LP. Treatment

included oral acetazolamide (n = 9/14) or surgical shunting procedures (n = 7/14).

One patient (No.14) improved after LP and was discharged with Aspirin. In the

follow up of patients (1-22 , mean 6 months) clinical data and follow up MRIs(Reversibility of sinuvenous stenoses, diameter of the perioptic nerve sheath,

height of the pituitary) were correlated. A total of 76 MRIs were evaluated.

Results: MRI / MR venography of all patients showed intracranial venous

stenoses in the intital examination, when CSF pressure was abnormally high. This was also true for the two patients with SIH: They exhibited stenoses of the

transverse sinus opposite to the occluded sinus/jugular vein. There were no signs

of an acute thrombosis. Radiological signs of increased intracranial pressure such

as widening of the optic sheaths and flattening of the pituitary (“empty sella”) were in all patients in accordance with elevated CSF pressure. In 10/14 patients, CSF

diversion by lumbar puncture lead to partial or total abolishment of intracranial

sinus stenoses, indicating that intracranial pressure induced the stenosis and not

vice versa (group A). In these patients, signs of elevated intracranial pressure

(widening of optic nerve sheaths and “empty sella”) were also reversed. In the other 4 patients (group B), no substantial reversibility was observed concerning the sinusvenous stenosis and the intracranial pressure signs despite

normalisation of lumbar CSF pressure.

Follow up: All of the patients of group A improved clinically (4 of who were free of symptoms on follow up) and MRI improved accordingly. Furthermore, two patients

of group B (whose MRI intitially did not improve after LP) also improved clinically

and MRI improved in the follow up after another LP was performed. In total, 12/14

patients improved clinically and on MRI. One patient (No.13) of group B experienced temporary relief of headache after LP but on follow up headaches did

not improve despite acetazolamide therapy. Vision remained stable (0,8/0,8). Follow up LP showed no diminishment of CSF pressure (27 cm H2O). MRI/MRV

did not improve either: She was regarded as a non-responder and might actually

have SIH due to chronic stenoses of the TS. The last patient of group B (No.14) with occlusion of the left jugular vein had relatively mild symptoms initially

(headaches that were reported to be 2-3 on the visual analogue scale, no visual disturbances that could be attributed to PC, no papilledema). He was treated with

LP and was discarded with Aspirin. Headaches almost completely resolved but follow up MRI did not improve. Furthermore, Shunt dysfunction was seen in one patient (fig. 1), shunt overdrainage was seen in two patients (No. 4 and 10, fig. 2,

a shunt assistant needed to be employed in one). A clinical relapse with

corresponding MRI signs was seen in one patient under acetazolamide therapy

(No.11, CSF pressure 24 cm H2O), improving on follow up. Acteazolamidetherapy was in part guided by MRI (fig. 3).

Conclusions: MRI and MR-venography prior to and after CSF diversion can help

to verify the diagnosis “pseudotumor cerebri” and to distinguish between

idiopathic and secondary intracranial hypertension. We demonstrated for the first time that MRI can be used as a valuable non-invasive tool in the follow up of

these patients.

MRI and MR-venography in patients with

pseudotumor cerebri:Reversibility of intracranial venous sinus stenoses and follow upRohr A*, Alfke K*, Bartsch T#, Dörner L§, Jansen O*

*Section of Neuroradiology # Clinic of Neurology §Clinic of Neurosurgery

Fig. 1) Failure of ventriculoperitoneal shunt

A 32y old female patient (No.7) suffered from disabling

neck pain and severe visual impairment. Normalising

CSF pressure by repeated LPs resulted in clinical

improvement. At CSF pressure of 11cm H2O,

morphology of intracrnial sinuses (Ai), optic nerves and

pituitary (Aii, height in mm in red) was normal. Three days

after insertion of a vp-shunt, radiological sign of increased

pressure recurred (TS stenoses encircled in Bii, nerv

sheath hydrops as indicated by arrows and „empty sella“

in Bii) and shunt failure with extraperitoneal ending of the

distal part of the vp-shunt was confirmed operatively.

After shunt revision, good clinical and radiological

improvement (C) was documented.

4,5 mm 8 mm

Ai Bi

Aii

Aiii

Bii

Biii

Fig. 2) Overdrainage by vp-shunt in SIH

A 68 y old female patient (No.4) with venous outflow

impairment due to occlusion of the left TS by a meningioma (curved arrow in Ai, open arrow in Aii on

axial mri / mr-venography) and contralateral TS

stenosis (circle in Aii). Signs of increased intracrnial

pressure were seen on mri (optic nerve sheath fluid

accumulation as indicated by arrows in Aiii and

flattening of the pituitary, height in mm in red) and

confirmed by LP. After insertion of a ventriculo-

peritoneal shunt into the left ventricle, papilledema

resolved but headache persisted. The reason was

found to be overdrainage by the shunt: Typical signs

of overdrainage on mri were dural thickening and

enhancement after application of i.v.-contrast agent

(small arrows in Bi), engorgement of the pituitary and

lack of fluid in the perioptic nerve sheaths (Biii).

Intracranial venous sinuses also engorged (Bii).

Clearly the right TS stenosis resolved (Bii) and was

thus proved to be secondary to increased pressure.

stableimproving3ASSnoxxx1027nonoxSIHm14

stablestable6xnoxxx927xxxIIH35w13

improvingimproving5xx*xxx1033xxxIIH27w12

improvingimproving3xx*xnox1231xxxIIH39w11

goodimproving1xxxxxx1226VII, VIIIxxnoIIH28m10

improvingimproving1xxxxxx***xxxIIH29w9

improvinggood1xxxxxx1030IxxxIIH29w8

improvingimproving1xxxxx1130xxxIIH51w7

improvinggood2xxxxx634xxxIIH26w6

improvingimproving5xxxxx***xxxIIH41w5

goodimproving15xxxxx624xxxSIH26w4

goodgood15xxxxx1644IxxxIIH23w3

improvinggood16xxxxx947xxxIIH35w2

improvingimproving22xxxxx1441VI, VIIxxxIIH23w1

MR

I/MR

-Venogra

phy

Clin

ical

Month

s

VP

-shunt

Aceta

zola

mid

e

Reversib

ility

Nerv

e sh

eath

hydro

ps

Em

pty

sella

Venous

steno

ses

Afte

r LP

Befo

reLP

Cra

nia

lN

palsy

Papille

dem

a

Visu

al im

pairm

ent

Headache

IIH/S

IH

BM

I

Sex

Patie

nt N

o

OutcomeOutcomeFollowupTreatmentMRI / MRV

CSF pressureSigns & symptomsPatient data

Table 1) Patient data, MR imaging and outcome

IIH (idiopathic intracranial hypertension), SIH (secondary intracranial hypertension) LP (lumbar puncture)

* (not documented but high) ** (not documented but low), x* (reversible after second LP), CFS pressure in cm H2O

2,3 mm 5,2 mm9 mm

Ai

Aii

Bi

Bii

Ci

Cii

3,6

mm*

4,9 mm*

4,5 mm*

4,5 mm*

4,5 mm*

4,3 mm*

4,4 mm*

Day 0 1 35 125 219 399 448CSF pressure 44 cm 16 cm 22 cm 16 cm

Headache yes no no no no no no

Vision reduced, papilledema good good good good good

Therapy LP diamox 2g diamox 0,5g diamox 0,75g no diamox 1g no

Fig. 3) Long term follow-up of a medically treated patient

A 20 y old female patient (No. 3) with a history of headache and visual blurring (bilateral pailledema confirmed). At presentation A) mri (coronal view) demonstrated widening of the optic nerve sheaths

(arrows in A ii) and some excavation of the pituitary (insert, height in mm in red) and bilateral transverse sinus (TS) stenoses (red circles in A i) as shown in MR-venography (sligthly oblique coronal

view). Lowering CSF pressure from 44 to 16 cm H2O by lumbar puncture (LP) (b) resulted in normalisation of the sinuvenous anatomy (B i), led to a marked reduction of perioptic fluid accumulation (B ii)

and height of the pituitary was restored to some degree (B ii insert). Headache vanished and vision was restored within days. Acetazolamide (Diamox©) therapy was guided by MR imaging during follow

up (C-G). No syptoms recurred, but restenosis of TS occurrd after acetacolamid was tapered (circles in F i) and the optic nerve sheaths began to show some widening (arrows in F ii). These changes

reversed after acetazolamide therapy was reinitiated (G).

Ai

Aii

Bi

Bii

Ci

Cii

Di

Dii

Ei

Eii

Fi

Fii

Gi

Gii

Corresponding author

Axel Rohr, MD

Section NeuroradiologyClinic of NeurosurgeryUKSH Campus Kiel

Schittenhelmstr. 10

24105 KielGermanyTel ++49-431-5974806

Axel.Rohr@neurorad.uni-kiel.de