7. USAUSA
0.9 to 1.0 / 100,000 in general population0.9 to 1.0 / 100,000 in general population
1.6-3.5 /100,000 in women1.6-3.5 /100,000 in women
7.9-20 /100,000 in overweight women7.9-20 /100,000 in overweight women
UKUK
1.56/100,000/year1.56/100,000/year
2.86/ 100,000 in women2.86/ 100,000 in women
11.9/100,000 in obese women11.9/100,000 in obese women
7Bakhsh A15-05-05
8. LibyaLibya
2.2/100,000 in general population2.2/100,000 in general population
12/100,000 in women aged 15–44 years12/100,000 in women aged 15–44 years
21.4/ 100,000 in obese women21.4/ 100,000 in obese women
OmanOman
2.18/100,000 in general population2.18/100,000 in general population
3.25/100,000 women in all age groups3.25/100,000 women in all age groups
4.14/ 100,000 in the age group of 15–44 years4.14/ 100,000 in the age group of 15–44 years
IsraelIsrael
2.02/100,000 in general population2.02/100,000 in general population
3.17/100,000 in women3.17/100,000 in women
5.49/100,000 in reproductive age group5.49/100,000 in reproductive age group
Sumayya J et el.Sumayya J et el. Idiopathic intracranial hypertension in theIdiopathic intracranial hypertension in the
Middle East: A growing concern.Middle East: A growing concern. Saudi Journal ofSaudi Journal of
Ophthalmology (2015) 29, 26–31.Ophthalmology (2015) 29, 26–31.
15-05-05 Bakhsh A 8
14. Priapism is pathologicalPriapism is pathological elevation of venouselevation of venous
pressurepressure of the male genitalia due to venousof the male genitalia due to venous
out flowout flow obstructionobstruction oror compressioncompression..
Pathophysiology of idiopathic intracranialPathophysiology of idiopathic intracranial
hypertension may be analogous to that of priapismhypertension may be analogous to that of priapism
Bateman GABateman GA11..
Idiopathic intracranial hypertension: priapism ofIdiopathic intracranial hypertension: priapism of
the brain?the brain? Med Hypotheses.Med Hypotheses. 2004;63(3):549-52 2004;63(3):549-52..
15-05-05 Bakhsh A 14
19. With treatment, there is gradual improvement but notWith treatment, there is gradual improvement but not
necessarily recoverynecessarily recovery
Many patients have persistent papilledemaMany patients have persistent papilledema
High ICP on lumbar punctureHigh ICP on lumbar puncture
Residual visual field deficitsResidual visual field deficits
57 patients were followed for 5 to 41 years57 patients were followed for 5 to 41 years
24 % developed blindness24 % developed blindness
Corbett JJ.Corbett JJ. Visual loss in pseudotumor cerebri. Follow-up of 57
patients from
five to 41 yearsfive to 41 years and a profile of 14 patients with permanent severe
visual loss.
Arch NeurolArch Neurol 1982; 39:461
19Bakhsh A15-05-05
20. 40% recurrence rate over period of 6.2 years40% recurrence rate over period of 6.2 years
20 patients were followed up for over 10 years20 patients were followed up for over 10 years
3 patients had recurrence about 12–78 months3 patients had recurrence about 12–78 months
6 patients experienced delayed worsening6 patients experienced delayed worsening
about 28–135 months after an initial stable courseabout 28–135 months after an initial stable course
15-05-05 Bakhsh A 20
21. No evidence based guidelinesNo evidence based guidelines
Alleviation of headacheAlleviation of headache
Preservation of visionPreservation of vision
Early referral to ophthalmologistEarly referral to ophthalmologist
21Bakhsh A15-05-05
23. Patients continue to have headaches despitePatients continue to have headaches despite
improvement in papilledema and visual functionimprovement in papilledema and visual function
Analgesic overuse or rebound headaches may beAnalgesic overuse or rebound headaches may be
common in patientscommon in patients
23Bakhsh A15-05-05
24. A low-sodium weight reduction program alleviate symptomsA low-sodium weight reduction program alleviate symptoms
but not in all patientsbut not in all patients
Visual fields & papilledema improve more quickly in weightVisual fields & papilledema improve more quickly in weight
loss group.loss group.
Weight loss takes some time to achieve, other treatmentsWeight loss takes some time to achieve, other treatments
are required at the same timeare required at the same time
Kupersmith MJKupersmith MJ . Effects of weight loss on the course of idiopathic
intracranial hypertension in women. Neurology 1998Neurology 1998; 50:1094.
Johnson LNJohnson LN. The role of weight loss and acetazolamide in the
treatment of idiopathic intracranial hypertension (pseudotumor
cerebri). Ophthalmology 1998Ophthalmology 1998; 105:2313
24Bakhsh A15-05-05
25. First line treatmentFirst line treatment
1- 4 g / day1- 4 g / day
Effective in 47 to 67 %Effective in 47 to 67 %
MethazolamideMethazolamide( carbonic anhydrase Inhibitors) can( carbonic anhydrase Inhibitors) can
be used in acetazolamide intolerant patientsbe used in acetazolamide intolerant patients
Diamox sequelsDiamox sequels sustained release formulationsustained release formulation
expensiveexpensive
Sulfa allergy is relative contraindicationSulfa allergy is relative contraindication
25Bakhsh A
27. Treatment options are limitedTreatment options are limited
Caloric restrictionCaloric restriction && diureticsdiuretics areare
contraindicatedcontraindicated
Acetazolamide is a contraindication in firstAcetazolamide is a contraindication in first
20 weeks20 weeks
TeratogenicTeratogenic effects have been reported witheffects have been reported with
high doses in animals and a single case of ahigh doses in animals and a single case of a
teratomateratoma was seen in humanswas seen in humans
27Bakhsh A15-05-05
28. Pregnant patientsPregnant patients
OnlyOnly diagnosticdiagnostic notnot therapeutictherapeutic
CSF reforms within 6 hoursCSF reforms within 6 hours
Uncomfortable & painfulUncomfortable & painful
Technically difficult in obeseTechnically difficult in obese
Complications:Complications:
Low pressure headaches (30%)Low pressure headaches (30%)
Bakhsh A.
Role of conventional lumbar myelography in the management
of sciatica: An experience from Pakistan. Asian J Neurosurg. 2012
Jan;7(1):25-8..
28Bakhsh A15-05-05
29. Commonly used in the pastCommonly used in the past
Long-term side effects, weight gainLong-term side effects, weight gain
Withdrawal causes rebound intracranialWithdrawal causes rebound intracranial
hypertensionhypertension
Steroids are not routinely recommendedSteroids are not routinely recommended
Short courseShort course of intravenous corticosteroidsof intravenous corticosteroids
in conjunction with acetazolamide severe,in conjunction with acetazolamide severe,
acute visual lossacute visual loss
Liu GT.Liu GT. High-dose methylprednisolone andHigh-dose methylprednisolone and
acetazolamide for visual loss in pseudotumor cerebri.acetazolamide for visual loss in pseudotumor cerebri.
Am J Ophthalmol 1994Am J Ophthalmol 1994; 118:88; 118:88
29Bakhsh A15-05-05
30. Deteriorating vision is a universallyDeteriorating vision is a universally
accepted indicationaccepted indication
IntractableIntractable headache, unresponsive toheadache, unresponsive to
medicationmedication
30Bakhsh A15-05-05
32. HeadacheHeadache relief occurs in all patientsrelief occurs in all patients
50% having recurrent severe headaches50% having recurrent severe headaches
within 3 years of surgery, despite a workingwithin 3 years of surgery, despite a working
shuntshunt
95 to 100 % achieve remission of95 to 100 % achieve remission of visualvisual
ProblemsProblems
Vision continued to worsen in 32 %Vision continued to worsen in 32 %
32Bakhsh A15-05-05
33. Provide long-term relief in majority ofProvide long-term relief in majority of
patientspatients
Endoscopic operative techniques haveEndoscopic operative techniques have
improved our ability to place cathetersimproved our ability to place catheters
Shunt revision 40 to 60 %.Shunt revision 40 to 60 %.
McGirt M . Frameless stereotactic ventriculoperitoneal
shunting for pseudotumor cerebri: an outcomes
comparison versus lumboperitoneal shunting.
Neurosurgery 2004; 55:458-9
33Bakhsh A15-05-05
35. Records of all shunt placement procedures done at oneRecords of all shunt placement procedures done at one
institution between 1973 and 2003 were reviewedinstitution between 1973 and 2003 were reviewed
Based on their 30-year experience, authors found thatBased on their 30-year experience, authors found that
CSF shunts were extremely effective in the acuteCSF shunts were extremely effective in the acute
treatment providing long-term relief in the majority oftreatment providing long-term relief in the majority of
patients.patients.
The use of ventricular shunts was associated with aThe use of ventricular shunts was associated with a
lower risk of shunt obstruction & revision than the uselower risk of shunt obstruction & revision than the use
ofof LP shuntsLP shunts..
McGirt MJMcGirt MJ.. Cerebrospinal fluid shunt placement for pseudotumorCerebrospinal fluid shunt placement for pseudotumor
cerebri-associated intractable headache: predictors of treatmentcerebri-associated intractable headache: predictors of treatment
response and an analysis of long-term outcomes.response and an analysis of long-term outcomes. JJ
Neurosurg.Neurosurg. 2004 ;101(4):627-32.2004 ;101(4):627-32.
15-05-05 Bakhsh A 35
36. Remission of symptoms 92%Remission of symptoms 92%
Papilledema resolves 97%Papilledema resolves 97%
Effects start after 1 to 3 years afterEffects start after 1 to 3 years after
surgerysurgery
With mean weight loss of 45 to 58 kgWith mean weight loss of 45 to 58 kg
12 studies class IV have been published12 studies class IV have been published
with 66 patientswith 66 patients
Jared FridleyJared Fridley . Bariatric surgery for the treatment of idiopathic. Bariatric surgery for the treatment of idiopathic
intracranial hypertension. Jintracranial hypertension. J Neurosurg, 2010Neurosurg, 2010
36Bakhsh A15-05-05
38. OOpticptic NNerveerve SSheathheath FFenestrationenestration
Preservation of vision is primary goalPreservation of vision is primary goal
It does not reduce ICPIt does not reduce ICP
Patients with bilateral papilledema needPatients with bilateral papilledema need
bilateralbilateral OONNSSFF
Shunting may still be requiredShunting may still be required
Alsuhaibani AH, et el.Alsuhaibani AH, et el. Effect of optic nerve sheath fenestration onEffect of optic nerve sheath fenestration on
papilledema of thepapilledema of the
operated and the contralateral nonoperated eyes in idiopathic intracranialoperated and the contralateral nonoperated eyes in idiopathic intracranial
hypertension.hypertension.
Ophthalmology. 2011Ophthalmology. 2011 ; 118:412–414; 118:412–414
38Bakhsh A15-05-05
39. DiplopiaDiplopia
Extraocular muscle injury or to their nerve orExtraocular muscle injury or to their nerve or
blood supply) in 29 to 35 %blood supply) in 29 to 35 %
Pupillary dysfunction 11 %Pupillary dysfunction 11 %
Transient Vision loss 11 %Transient Vision loss 11 %
Permanent in 1.5 to 2.6Permanent in 1.5 to 2.6
Long-term follow up shows deterioration in VFLong-term follow up shows deterioration in VF
39Bakhsh A15-05-05
40. Many patients have
transverse sinus narrowing
at
Distal transverse sinusDistal transverse sinus
Transverse/sigmoid sinusTransverse/sigmoid sinus
JunctionJunction
UnilaterallyUnilaterally
OrOr
BilaterallyBilaterally
15-05-05 Bakhsh A 40
41.
Cerebral venography and manometry in 99 patients with
idiopathic intracranial hypertension consistently showed
venous hypertensionvenous hypertension in
superior sagittal sinus &superior sagittal sinus &
proximal transverse sinusesproximal transverse sinuses
significant drop in venous pressure at the level of lateralsignificant drop in venous pressure at the level of lateral
third of transverse sinusthird of transverse sinus
The abnormality, clearlyclearly demonstrated by manometry, was not
well shown on the venous phase of cerebral angiography.
The appearance of the transverse sinus on venography varied
from smooth tapered narrowing to discrete intraluminal
filling defects
King JOKing JO11
.Cerebral venography and manometry in idiopathic.Cerebral venography and manometry in idiopathic
intracranial hypertension.intracranial hypertension. Neurology.Neurology. 19951995 ;45(12):2224-8.;45(12):2224-8.
15-05-05 Bakhsh A 41
42. May 5, 2015 Bakhsh ABakhsh A 42
Farb have identified venous sinus stenosisFarb have identified venous sinus stenosis
in >in >90%90% of patients with PTCof patients with PTC
6.8%6.8% in the control asymptomatic groupin the control asymptomatic group
In another recent studyIn another recent study 90%90% of 51 PTCof 51 PTC
patients had bilateral transverse sinuspatients had bilateral transverse sinus
stenosis on MR venography, withstenosis on MR venography, with ATECOATECO
MRV techniqueMRV technique
Farb RI Farb RI . Idiopathic intracranial hypertension: the prevalence. Idiopathic intracranial hypertension: the prevalence
and morphology of sinovenous stenosis. and morphology of sinovenous stenosis. Neurology. 2003;Neurology. 2003;
60:1418–142460:1418–1424
43. May 5, 2015 Bakhsh A 43
The conventional MR venography suffers fromThe conventional MR venography suffers from
artifacts in the region of the distal transverse sinus.artifacts in the region of the distal transverse sinus.
This is why venous stenosis in PTC has been missedThis is why venous stenosis in PTC has been missed
in the past.in the past.
Higgins et al.Higgins et al. reanalyzedreanalyzed the MRVs of 20 PTCthe MRVs of 20 PTC
patients that were initially interpreted aspatients that were initially interpreted as normalnormal
Bilateral lateralBilateral lateral sinus flow gapssinus flow gaps were identified inwere identified in
13 of 20 patients with PTC13 of 20 patients with PTC
None of 40 controls.None of 40 controls.
47. May 5, 2015 Bakhsh A 47
In venous sinuses,In venous sinuses, increaseincrease inin numbernumber
andand sizesize with advancing age and canwith advancing age and can
obstruct transverse sinusesobstruct transverse sinuses
Cause focal intra-luminal filling defects inCause focal intra-luminal filling defects in
24% of CT and 13% of contrast enhanced24% of CT and 13% of contrast enhanced
MR studies in normal populationsMR studies in normal populations
49. 20 transverse sinuses were explored (in a pilot20 transverse sinuses were explored (in a pilot
study of 10 human cadavers) in order to determinestudy of 10 human cadavers) in order to determine
the anatomical basis of this stenosis.the anatomical basis of this stenosis.
The presence of septa of varying sizes wasThe presence of septa of varying sizes was
observed.observed.
We conclude might be one of the aetiological factorsWe conclude might be one of the aetiological factors
involved in idiopathic intracranial hypertension.involved in idiopathic intracranial hypertension.
Subramaniam RM. Transverse sinus septum: a
new aetiology of idiopathic intracranial
hypertension? Australas Radiol. 2004
Jun;48(2):114-6.
15-05-05 Bakhsh A 49
50. A total ofA total of 102 cadavers102 cadavers && living patientsliving patients were usedwere used
53% of the subjects had structures in their53% of the subjects had structures in their
transverse sinuses that could be potential venoustransverse sinuses that could be potential venous
filling defectsfilling defects..
The septa were found to be more dominant inThe septa were found to be more dominant in
central (30%) and lateral (22%) thirds ofcentral (30%) and lateral (22%) thirds of
right transverse sinusesright transverse sinuses
30% of the subjects presented with arachnoid30% of the subjects presented with arachnoid
granulations in the right transverse sinusgranulations in the right transverse sinus.
Strydom MA et el.Strydom MA et el. The anatomical basis of venographic fillingThe anatomical basis of venographic filling
defects of the transverse sinus.defects of the transverse sinus. Clin Anat. 2010Clin Anat. 2010;23(2):153-;23(2):153-
99
50Bakhsh A15-05-05
55. May 5, 2015 Bakhsh ABakhsh A 55
Transverse sinus stenosis may occur as a
secondary phenomenon in response to
elevated ICP
Resolved stenosis with CSF drainage
reversal of the venous sinus stenoses either
by means of lumbar puncture or by CSF
shunting
57. May 5, 2015 Bakhsh A 57
The first stent placement in the transverseThe first stent placement in the transverse
sinus for the treatment of IIH wassinus for the treatment of IIH was
attempted in 2002 by Higgins in an obeseattempted in 2002 by Higgins in an obese
woman with bilateral stenosis of the sinuseswoman with bilateral stenosis of the sinuses
and intracranial hypertension refractory toand intracranial hypertension refractory to
any form of treatmentany form of treatment
Higgins JN.Higgins JN. Idiopathic intracranial hypertension:12 cases treated byIdiopathic intracranial hypertension:12 cases treated by
venous sinus stenting.venous sinus stenting. J Neurol Neurosurg Psychiatry 2003J Neurol Neurosurg Psychiatry 2003;;
74:1662-74:1662-
05/05/15 Bakhsh A 57
59. May 5, 2015 Bakhsh A 59
Outcomes in 207 patientsOutcomes in 207 patients
2 Months to 136 Months2 Months to 136 Months
81% headaches81% headaches
87% papilledema87% papilledema
95% pulsatile tinnitus95% pulsatile tinnitus
Follow up periodsFollow up periods
Albuquerque FC, et alAlbuquerque FC, et al . Intracranial venous sinus. Intracranial venous sinus
stenting for benign intracranial hypertension: clinicalstenting for benign intracranial hypertension: clinical
indications, technique, and preliminary results.indications, technique, and preliminary results. WorldWorld
Neurosurg. 2011;Neurosurg. 2011; 75:648–65275:648–652
60. May 5, 2015 Bakhsh A 60
Stent migrationStent migration
Sinus perforationSinus perforation
In-stent thrombosisIn-stent thrombosis
Subdural hemorrhageSubdural hemorrhage
Intracranial hemorrhageIntracranial hemorrhage
Recurrent stenosis proximal to stentRecurrent stenosis proximal to stent
Puffer RC.Puffer RC. Venous sinus stenting for idiopathicVenous sinus stenting for idiopathic
intracranial hypertension: a review of the literature.intracranial hypertension: a review of the literature. JJ
Neurointerv Surg 2013Neurointerv Surg 2013; 5:483.; 5:483.
61. May 5, 2015 Bakhsh ABakhsh A 61
Stent patency may be evaluated by CTStent patency may be evaluated by CT
venographyvenography
Six-month period of anticoagulation isSix-month period of anticoagulation is
required post stentingrequired post stenting
BeBe alert to the recurrence of PTC symptomsalert to the recurrence of PTC symptoms
Require re-stentingRequire re-stenting
62. May 5, 2015 Bakhsh A 62
Costs of PTC patients have exceeded $444Costs of PTC patients have exceeded $444
million/ year in U S Amillion/ year in U S A
A recent study looked at the economic burden of CSFA recent study looked at the economic burden of CSF
shunting proceduresshunting procedures versusversus venous sinus stentingvenous sinus stenting
There was no cost difference for the initial procedureThere was no cost difference for the initial procedure
for both shunts and stentsfor both shunts and stents
The costs of shunt revisions and treatment related toThe costs of shunt revisions and treatment related to
shunt infections made the shunting procedureshunt infections made the shunting procedure
approximatelyapproximately five times more costly overall.five times more costly overall.
63. May 5, 2015 Bakhsh A 63
The Idiopathic Intracranial
Hypertension Treatment Trial
A multicenter, double-blind, placebo-controlled clinical trial,
is currently enrolling patients in the US
(http://www.nordicclinicaltrials.com/).
This trial compares the efficacy of acetazolamide and
placebo in the treatment of IIH patients with moderate visual
field defects.
All patients are also treated with a low-sodium diet and
participate in a standardized weight loss program.
This trial will clarify the efficacy of acetazolamideefficacy of acetazolamide and weightweight
lossloss in IIH
Additional outcomes measured yearly up to 4 years
Wall et al, The Idiopathic Intracranial HypertensionWall et al, The Idiopathic Intracranial Hypertension
Treatment Trial, JAMA Neurology, 2014, Vol 71, No. 6Treatment Trial, JAMA Neurology, 2014, Vol 71, No. 6
64. The importance of venous sinus disease in the etiology ofThe importance of venous sinus disease in the etiology of
idiopathic intracranial hypertension is probablyidiopathic intracranial hypertension is probably
underestimated.underestimated.
Patients in whom a venous sinus stenosis isPatients in whom a venous sinus stenosis is
demonstrated by a noninvasive radiologic workupdemonstrated by a noninvasive radiologic workup
should be evaluated with direct retrograde cerebralshould be evaluated with direct retrograde cerebral
venography & manometryvenography & manometry..
In patients with aIn patients with a lesion of the venous sinuseslesion of the venous sinuses whowho
experiencedexperienced medical treatment failuremedical treatment failure,,
endovascular stent placement seems to be anendovascular stent placement seems to be an
interesting alternativeinteresting alternative toto classic surgicalclassic surgical
approaches.approaches.
Donnet ADonnet A.. Endovascular treatment of idiopathicEndovascular treatment of idiopathic
intracranial hypertension: clinical and radiologic outcomeintracranial hypertension: clinical and radiologic outcome
of 10 consecutive patientsof 10 consecutive patients. Neurology 2008; 70:641. Neurology 2008; 70:641.
15-05-05 Bakhsh A 64
MR images from the case of a 9-year-old male patient with IIH without papilledema. A, Transverse T2-weighted image (4000/96/2 [TR/TE/excitations]), obtained at admission, shows small cortical veins and superior sagittal, straight, transverse and sigmoid sinuses but no intracranial mass lesion, ventricular dilation, or sinus thrombosis.B, Sagittal fat-saturated T2-weighted image (4000/100/2) of the right optic nerve, obtained at admission, shows flattening of the posterior sclera, vertical tortuosity and elongation of the nerve, and distension of the perioptic subarachnoid space.C, Sagittal T1-weighted image (500/9/2), obtained at admission, shows a partially empty sella.D, Transverse T2-weighted image, obtained 4 months after admission and after three lumbar punctures and improvement of the clinical symptomatology, reveals a decrease in the subarachnoid space and normalization of the sizes of the cortical veins and superior sagittal, straight, transverse, and sigmoid sinuses.E, Sagittal fat-saturated T2-weighted image of the right optic nerve, obtained 4 months after admission and after three lumbar punctures and improvement of the clinical symptomatology, shows a normally round orbit and a normally straight nerve.F, Sagittal T1-weighted image, obtained 4 months after admission and after three lumbar punctures and improvement of the clinical symptomatology, shows that the previously compressed pituitary gland had reexpanded to fill the sella turcica.
Image shows appearance of septum within dural sinus in a 68-year-old woman with normal results of an MR imaging examination. Reconstruction image from a 3D contrast-enhanced MPRAGE image shows a linear structure (arrows), consistent with a septum within the straight sinus.
Image shows septa within dural sinuses in a 39-year-old man with normal results of an MR imaging study. Axial source image from a 3D contrast-enhanced MPRAGE image shows curvilinear septa (arrows) that have intermediate signal intensity in both transverse sinuses. The septa seen in this study were usually smooth, thin, and not limited to the center of the sinus.
Images reveal arachnoid granulations in a 54-year-old man with headaches who had normal results of an MR imaging study.A, Sagittal reconstruction image obtained from 3D contrast-enhanced MPRAGE imaging sequence shows a large CSF-isointense filling defect, consistent with an arachnoid granulation (black arrows), at the junction point of the vein of Galen and straight sinus. Note also the presence of smaller arachnoid granulations within the superior sagittal sinus (white arrowheads).B, Axial T2-weighted MR image shows the defect seen in A as a discrete, focal CSF-isointense filling defect within the straight sinus.
Mechanism by which transverse sinus stenosis leads to increase intracranial pressure. ICP, intracranial pressure.
Resolution of bilateral transverse sinus stenosis after lumbo-peritoneal shunt in a young obese woman with idiopathic intracranial hypertension.
