Management of pseudotumor cerebri

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Pseudotumor cerebri

• By
• Ahmed Bakhsh
• ahmedbakhsh_at_gmail.com

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Pseudotumor cerebri
Pseudotumor cerebri

• Syndrome of raised intracranial pressure
• without any
• clinical
• laboratory
• radiological evidence of
• intracranial pathology
• Presents with symptoms of increased ICP
• headache
• pulsatile tinnitus
• transitory visual obscuration
• diplopia

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• Obese females
• Intractable headaches
• vision problems
• Papilledema
• Think of
• Pseudotumor cerebri

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Age at diagnosis
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Epidemiology

• USA
• 0.9 to 1.0 / 100,000 in general population
• 1.6-3.5 /100,000 in women
• 7.9-20 /100,000 in overweight women 
• UK
• 1.56/100,000/year
• 2.86/ 100,000 in women
• 11.9/100,000 in obese women

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Middle East

• Libya
• 2.2/100,000 in general population
• 12/100,000 in women aged 1544 years
• 21.4/ 100,000 in obese women
• Oman
• 2.18/100,000 in general population
• 3.25/100,000 women in all age groups
• 4.14/ 100,000 in the age group of 1544 years
• Israel
• 2.02/100,000 in general population
• 3.17/100,000 in women
• 5.49/100,000 in reproductive age group
• Sumayya J et el. Idiopathic intracranial
  hypertension in the Middle East A growing
  concern. Saudi Journal of Ophthalmology (2015)
  29, 2631.

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History Nomenclature

• Meningitis serosa
  Quincke 1893
• Pseudotumor cerebri
  Nonne 1904
• Benign intracranial hypertension
  Foley 1955
• Idiopathic intracranial hypertension
  Corbett 1989
• Primary intracranial hypertension
• Secondary intracranial hypertension

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Medical conditions

• Anemia
• Sleep Apnea
• Hypertension
• Hypoparathyridism
• Chronic renal failure
• Cushings Addisons

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Drugs

• Tetracycline. Minocycline
• Anabolic steroids
• Growth hormone
• Nitrofurantoin
• Nalidixic acid
• Isotretinoin
• Tamoxifen
• Vitamin A
• Lithium
• Steroid

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Possible causes of high ICP
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Brain Priapism

• Priapism is pathological elevation of venous
• pressure of the male genitalia due to venous
• out flow obstruction or compression.
• Pathophysiology of idiopathic intracranial
• hypertension may be analogous to that of priapism
  
• Bateman GA1.Idiopathic intracranial hypertension
  priapism of the brain? Med Hypotheses. 200463(3)
  549-52.

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Pathophysiology in Obesity
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Diagnostic criteria
Diagnostic criteria
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Modified Dandy criteria by Smith in 1985 

• 1)Dandy WE. Intracranial pressure without brain
  tumordiagnosis and treatment. Ann Surg
  1937106492513.
• 2) Smith JL. Whence pseudotumor cerebri? J Clin
  Neuroophthalmol 19855556
• 3) Friedman DI. Diagnostic criteria for
  idiopathic intracranial hypertension. Neurology
  20025914925
• Awake patient
• Symptoms signs of high ICP
• Elevated ICP lateral decubitus position (gt20 cm
  H2O)
• Normal CSF composition
• Normal routine neuroimaging

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MR images from the case of a 9-year-old male
patient with IIH without papilledema.
Radiological signs
Hiroko Suzuki et al. AJNR Am J Neuroradiol
200122196-199
2001 by American Society of Neuroradiology
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Prognosis

• With treatment, there is gradual improvement but
  not
• necessarily recovery
• Many patients have persistent papilledema
• High ICP on lumbar puncture
• Residual visual field deficits
• 57 patients were followed for 5 to 41 years
• 24 developed blindness
• Corbett JJ. Visual loss in pseudotumor cerebri.
  Follow-up of 57 patients from
• five to 41 years and a profile of 14 patients
  with permanent severe visual loss.
• Arch Neurol 1982 39461

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Recurrence

• 40 recurrence rate over period of 6.2 years
• 20 patients were followed up for over 10 years
• 3 patients had recurrence about 1278 months
• 6 patients experienced delayed worsening
• about 28135 months after an initial stable course

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General management 

• No evidence based guidelines
• Alleviation of headache
• Preservation of vision
• Early referral to ophthalmologist

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Medical management
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Headache prophylaxis 

• Patients continue to have headaches despite
  improvement in papilledema and visual function
• Analgesic overuse or rebound headaches may be
  common in patients

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Weight loss

• A low-sodium weight reduction program alleviate
  symptoms but not in all patients
• Visual fields papilledema improve more quickly
  in weight loss group.
• Weight loss takes some time to achieve, other
  treatments are required at the same time
• Kupersmith MJ . Effects of weight loss on the
  course of idiopathic intracranial hypertension in
  women. Neurology 1998 501094.
• Johnson LN. The role of weight loss and
  acetazolamide in the treatment of idiopathic
  intracranial hypertension (pseudotumor cerebri).
  Ophthalmology 1998 1052313

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Acetazolamide 

• First line treatment
• 1- 4 g / day
• Effective in 47 to 67
• Methazolamide( carbonic anhydrase Inhibitors) can
  be used in acetazolamide intolerant patients
• Diamox sequels sustained release formulation
• expensive
• Sulfa allergy is relative contraindication

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Side effects

• Anorexia
• Metallic taste
• Kidney stones
• Metabolic acidosis
• Nausea vomiting
• Electrolytes change
• Digital oral paresthesias

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Pregnancy

• Treatment options are limited
• Caloric restriction diuretics are
  contraindicated
• Acetazolamide is a contraindication in first 20
  weeks
• Teratogenic effects have been reported with high
  doses in animals and a single case of a teratoma
  was seen in humans

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Serial Lumbar Puncture

• Pregnant patients
• Only diagnostic not therapeutic
• CSF reforms within 6 hours
• Uncomfortable painful
• Technically difficult in obese
• Complications
• Low pressure headaches (30)
• Bakhsh A. Role of conventional lumbar myelography
  in the management
• of sciatica An experience from Pakistan. Asian J
  Neurosurg. 2012
• Jan7(1)25-8..

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Corticosteroids 

• Commonly used in the past
• Long-term side effects, weight gain
• Withdrawal causes rebound intracranial
• hypertension
• Steroids are not routinely recommended
• Short course of intravenous corticosteroids
• in conjunction with acetazolamide severe,
• acute visual loss
• Liu GT. High-dose methylprednisolone and
• acetazolamide for visual loss in pseudotumor
  cerebri.
• Am J Ophthalmol 1994 11888

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Indications of surgery

• Deteriorating vision is a universally accepted
  indication
• Intractable headache, unresponsive to medication

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Surgical Options

• Ventriculoperitoneal shunt
• Lumboperitoneal shunt
• Repeated lumbar punctures
• Bariatric surgery
• Optic nerve sheath fenestration
• Dural venous sinus stenting

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CSF Shunting 

• Headache relief occurs in all patients
• 50 having recurrent severe headaches
• within 3 years of surgery, despite a working
• shunt
• 95 to 100 achieve remission of visual
• Problems
• Vision continued to worsen in 32

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Ventriculoperitoneal shunt

• Provide long-term relief in majority of patients
• Endoscopic operative techniques have improved our
  ability to place catheters
• Shunt revision 40 to 60 .
• McGirt M . Frameless stereotactic
  ventriculoperitoneal shunting for pseudotumor
  cerebri an outcomes comparison versus
  lumboperitoneal shunting. Neurosurgery 2004
  55458-9

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Lumboperitoneal shunt

• Shunt failure 86
• Shunt revisions 38
• Low pressure
• headaches
• Burgett RA. Lumboperitoneal shunting for
  pseudotumor cerebri. Neurology 1997 49734-9

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VP or LP

• Records of all shunt placement procedures done at
  one
• institution between 1973 and 2003 were reviewed
• Based on their 30-year experience, authors found
  that
• CSF shunts were extremely effective in the acute
• treatment providing long-term relief in the
  majority of
• patients.
• The use of ventricular shunts was associated with
  a
• lower risk of shunt obstruction revision than
  the use
• of LP shunts.
• McGirt MJ. Cerebrospinal fluid shunt placement
  for pseudotumor cerebri-associated intractable
  headache predictors of treatment response and an
  analysis of long-term outcomes. J Neurosurg. 2004
  101(4)627-32.

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Bariatric surgery

• Remission of symptoms 92
• Papilledema resolves 97
• Effects start after 1 to 3 years after surgery
• With mean weight loss of 45 to 58 kg
• 12 studies class IV have been published with 66
  patients
• Jared Fridley . Bariatric surgery for the
  treatment of idiopathic intracranial
  hypertension. J Neurosurg, 2010

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Optic nerve sheath fenestration 
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Optic Nerve Sheath Fenestration

• Preservation of vision is primary goal
• It does not reduce ICP
• Patients with bilateral papilledema need
• bilateral ONSF
• Shunting may still be required
• Alsuhaibani AH, et el. Effect of optic nerve
  sheath fenestration on papilledema of the
• operated and the contralateral nonoperated eyes
  in idiopathic intracranial hypertension.
• Ophthalmology. 2011 118412414

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Complications

• Diplopia
• Extraocular muscle injury or to their nerve or
• blood supply) in 29 to 35
• Pupillary dysfunction 11
• Transient Vision loss 11
• Permanent in 1.5 to 2.6
• Long-term follow up shows deterioration in VF

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Venous sinus stenosis

• Many patients have
• transverse sinus narrowing at
• Distal transverse sinus
• Transverse/sigmoid sinus
• Junction
• Unilaterally
• Or
• Bilaterally

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New aetiology

• Cerebral venography and manometry in 9 patients
  with idiopathic intracranial hypertension
  consistently showed
• venous hypertension in
• superior sagittal sinus
• proximal transverse sinuses
• significant drop in venous pressure at the level
  of lateral third of transverse sinus
• The abnormality, clearly 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 JO1.Cerebral venography and manometry in
  idiopathic intracranial hypertension.
  Neurology. 1995 45(12)2224-8.

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• Farb have identified venous sinus stenosis in
  gt90 of patients with PTC
• 6.8 in the control asymptomatic group
• In another recent study 90 of 51 PTC patients
  had bilateral transverse sinus stenosis on MR
  venography, with ATECO MRV technique
• Farb RI . Idiopathic intracranial hypertension
  the prevalence
• and morphology of sinovenous stenosis. Neurology.
  2003
• 6014181424

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• The conventional MR venography suffers from
  artifacts in the region of the distal transverse
  sinus. This is why venous stenosis in PTC has
  been missed in the past.
• Higgins et al. reanalyzed the MRVs of 20 PTC
  patients that were initially interpreted as
  normal
• Bilateral lateral sinus flow gaps were identified
  in 13 of 20 patients with PTC
• None of 40 controls.

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Image shows appearance of septum within dural
sinus in a 68-year-old woman with normal results
of an MR imaging examination.
Luxia Liang et al. AJNR Am J Neuroradiol
2002231739-1746
2002 by American Society of Neuroradiology
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Image shows septa within dural sinuses in a
39-year-old man with normal results of an MR
imaging study.
Luxia Liang et al. AJNR Am J Neuroradiol
2002231739-1746
2002 by American Society of Neuroradiology
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Arachnoid granulations
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• In venous sinuses, increase in number and size
  with advancing age and can obstruct transverse
  sinuses
• Cause focal intra-luminal filling defects in 24
  of CT and 13 of contrast enhanced MR studies in
  normal populations

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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, c...
Luxia Liang et al. AJNR Am J Neuroradiol
2002231739-1746
2002 by American Society of Neuroradiology
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Cadaveric studies

• 20 transverse sinuses were explored (in a pilot
  study of 10 human cadavers) in order to determine
  the anatomical basis of this stenosis.
• The presence of septa of varying sizes was
  observed.
• We conclude might be one of the aetiological
  factors involved in idiopathic intracranial
  hypertension.
• Subramaniam RM. Transverse sinus septum a new
  aetiology of idiopathic intracranial
  hypertension? Australas Radiol. 2004
  Jun48(2)114-6.

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Cadaveric studies

• A total of 102 cadavers living patients were
  used
• 53 of the subjects had structures in their
• transverse sinuses that could be potential venous
• filling defects.
• The septa were found to be more dominant in
• central (30) and lateral (22) thirds of
• right transverse sinuses
• 30 of the subjects presented with arachnoid
• granulations in the right transverse sinus.
• Strydom MA et el. The anatomical basis of
  venographic filling defects of the transverse
  sinus. Clin Anat. 201023(2)153-9

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Mechanism by which transverse sinus stenosis
leads to increase intracranial pressure.
Valérie Biousse et al. J Neurol Neurosurg
Psychiatry 201283488-494
2012 by BMJ Publishing Group Ltd
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Primary or Secondary

• 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

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Resolution of bilateral transverse sinus stenosis
after lumbo-peritoneal shunt in a young obese
woman with idiopathic intracranial hypertension.
Valérie Biousse et al. J Neurol Neurosurg
Psychiatry 201283488-494
2012 by BMJ Publishing Group Ltd
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Venous stenting 

• The first stent placement in the transverse sinus
  for the treatment of IIH was attempted in 2002 by
  Higgins in an obese woman with bilateral stenosis
  of the sinuses and intracranial hypertension
  refractory to any form of treatment
• Higgins JN. Idiopathic intracranial
  hypertension12 cases treated by
• venous sinus stenting. J Neurol Neurosurg
  Psychiatry 2003 741662-

10/2/2019
Bakhsh A
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A meta-analysis of 19 studies

• Outcomes in 207 patients
• 2 Months to 136 Months
• 81 headaches
• 87 papilledema
• 95 pulsatile tinnitus
• Follow up periods
• Albuquerque FC, et al. Intracranial venous sinus
  stenting for benign intracranial hypertension
  clinical indications, technique, and preliminary
  results. World Neurosurg. 2011 75648652

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Complications

• Stent migration
• Sinus perforation
• In-stent thrombosis
• Subdural hemorrhage
• Intracranial hemorrhage
• Recurrent stenosis proximal to stent
• Puffer RC. Venous sinus stenting for idiopathic
• intracranial hypertension a review of the
  literature. J
• Neurointerv Surg 2013 5483.

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Post-stent Care

• Stent patency may be evaluated by CT venography
• Six-month period of anticoagulation is required
  post stenting
• Be alert to the recurrence of PTC symptoms
• Require re-stenting

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Shunts or Stents

• Costs of PTC patients have exceeded 444
• million/ year in U S A
• A recent study looked at the economic burden of
  CSF
• shunting procedures versus venous sinus stenting
• There was no cost difference for the initial
  procedure
• for both shunts and stents
• The costs of shunt revisions and treatment
  related to
• shunt infections made the shunting procedure
• approximately five times more costly overall.

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The Idiopathic IntracranialHypertension
Treatment Trial
The Idiopathic IntracranialHypertension
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
  acetazolamide and weight loss in IIH
• Additional outcomes measured yearly up to 4 years
• Wall et al, The Idiopathic Intracranial
  Hypertension Treatment Trial, JAMA Neurology,
  2014, Vol 71, No. 6

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Take Home Message

• The importance of venous sinus disease in the
  etiology of idiopathic intracranial hypertension
  is probably underestimated.
• Patients in whom a venous sinus stenosis is
  demonstrated by a noninvasive radiologic workup
  should be evaluated with direct retrograde
  cerebral venography manometry.
• In patients with a lesion of the venous sinuses
  who experienced medical treatment failure,
  endovascular stent placement seems to be an
  interesting alternative to classic surgical
  approaches.
• Donnet A. Endovascular treatment of idiopathic
  intracranial hypertension clinical and
  radiologic outcome of 10 consecutive patients.
  Neurology 2008 70641.

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Thanks