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