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After this presentation, the reader should be able to describe features of papilledema with main causes and investigations needed in the work up and differentiate it from pseudopapilledema.
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Neuro-ophthalmology Neuro-ophthalmology Symposium: Clinical Symposium: Clinical
Scenarios and Evidence Scenarios and Evidence ReviewReview
Ra’ed Behbehani, MD, FRCSC, Ra’ed Behbehani, MD, FRCSC, Dipl.ABODipl.ABO
ObjectivesObjectives
Review important and common Review important and common neuro-ophthalmic entities with neuro-ophthalmic entities with emphasis on diagnosis, and emphasis on diagnosis, and management based on the current management based on the current evidence.evidence.
Papilledema or No Papilledema or No Papilledema ?Papilledema ?
Initial PresentationInitial Presentation
19 y/o AAF19 y/o AAF Pressure in retro-orbital and frontal Pressure in retro-orbital and frontal
regions bilaterallyregions bilaterally Blurry visionBlurry vision LightheadednessLightheadedness Symptoms worsen with any change in Symptoms worsen with any change in
postureposture
Initial PresentationInitial Presentation
PMH: Seizure disorderPMH: Seizure disorder POH: NonePOH: None Medications: Topamax 50mg BIDMedications: Topamax 50mg BID Allergies: NKDAAllergies: NKDA SH: ½ ppd tobacco, No EtOH, or drug SH: ½ ppd tobacco, No EtOH, or drug
useuse FH: NCFH: NC ROS: negativeROS: negative
Initial PresentationInitial Presentation
VVA scA sc: 20/20 OD: 20/20 OD 20/25 OS20/25 OS Pupils: No RAPDPupils: No RAPD EOM: Full OUEOM: Full OU TTAA: 12 OD: 12 OD 12 OS12 OS SLE: WNLSLE: WNL
Initial PresentationInitial Presentation
Visual FieldsVisual Fields
MRIMRI
MRVMRV
Lumbar PunctureLumbar Puncture
Opening pressure of 45 cm HOpening pressure of 45 cm H22OO Glucose WNLGlucose WNL WBC WNLWBC WNL Protein WNLProtein WNL
Modified Dandy CriteriaModified Dandy Criteria(Smith et al. J Clin Neuro-ophthalmol 1985)(Smith et al. J Clin Neuro-ophthalmol 1985)
1.1. Signs and symptoms of increased ICP.Signs and symptoms of increased ICP.2.2. No localizing neurological signs (except No localizing neurological signs (except
uni/bilateral VI nerve palsy)uni/bilateral VI nerve palsy)3.3. No evidence of an intracranial mass No evidence of an intracranial mass
lesion ( by CT )lesion ( by CT )4.4. Normal CSF compositionNormal CSF composition
Criteria for Idiopathic Criteria for Idiopathic Intracranial HypertensionIntracranial Hypertension
Friedman et al. Neurology 2002Friedman et al. Neurology 2002
1)1) If symptoms and signs are present, they If symptoms and signs are present, they should reflect only increased ICP.should reflect only increased ICP.
2)2) Documented increased ICP in the lateral Documented increased ICP in the lateral decubitus position (> 250 cm/H2O).decubitus position (> 250 cm/H2O).
3)3) Normal CSF composition.Normal CSF composition.4)4) No evidence of hydrocephalus, mass, No evidence of hydrocephalus, mass,
structural or vascular lesion on CT with structural or vascular lesion on CT with contrast for typical patients, and contrast for typical patients, and MRI/MRV for all others.MRI/MRV for all others.
5)5) No other cause of increased ICP found.No other cause of increased ICP found.
Secondary IIHSecondary IIH
Medical disorders : COPD, Severe Medical disorders : COPD, Severe hypertension, sleep apnea syndrome, hypertension, sleep apnea syndrome, renal failure, Addison’s disease, renal failure, Addison’s disease, HypoparathyroidismHypoparathyroidism
Medications : Tetracycline, Vitamin A, Medications : Tetracycline, Vitamin A, Anabolic steroids, Nalidixic acid, Lithium, Anabolic steroids, Nalidixic acid, Lithium, Levenorgestral implant system.Levenorgestral implant system.
Venous obstruction: Cerebral venous sinus Venous obstruction: Cerebral venous sinus thrombosis, jugular venous thrombosis.thrombosis, jugular venous thrombosis.
Spinal TapSpinal Tap
Keep the patient’s head neutral and legs Keep the patient’s head neutral and legs passively extended after needle in place.passively extended after needle in place.
If the patient’s head is flexed and knees If the patient’s head is flexed and knees drawn updrawn up Increased cephalic venous Increased cephalic venous pressure (Valsalva)pressure (Valsalva) compression of compression of Jugular vein and hpoventilation (CO2 Jugular vein and hpoventilation (CO2 retention) retention) Increased CSF pressure. Increased CSF pressure.
An IIH patient needs only one spinal tap !An IIH patient needs only one spinal tap !
Is CT adequate ?Is CT adequate ?
Can detect hydrocephalus and most Can detect hydrocephalus and most intra-cranial lesions.intra-cranial lesions.
Misses venous sinus thrombosis, Misses venous sinus thrombosis, radiographic signs of meningeal radiographic signs of meningeal infiltration, and isodense tumors.infiltration, and isodense tumors.
If only alternative (weight, If only alternative (weight, availability), order availability), order contrast enhancedcontrast enhanced CT.CT.
MRI and MRVMRI and MRV
Ideally for all patients.Ideally for all patients. For atypical patients : (children with For atypical patients : (children with
recent sinus/ear infection, fulminant recent sinus/ear infection, fulminant course, non-responders to course, non-responders to treatment).treatment).
““Benign intracranial Benign intracranial hypertension” (BIH).hypertension” (BIH).
Really ?!Really ?!
http://medlib.med.utah.edu
Papilledema ? Papilledema ?
http://library.med.utah.edu/NOVEL/Hoyt
Drusen
Drusen
Anamolous trifurcation
Blood vesselsnot obscuredby NFL
Papilledema ?Papilledema ?
http://library.med.utah.edu/NOVEL/Hoyt
Vessels notobscured byNFL
Drusen
Features of Anamolous discs Features of Anamolous discs (Peseuopapilledema)(Peseuopapilledema)
Psudopapilledema True PapilledemaDisc vessels obscured Disc margin vessels clear
Elevation of peripapillary NFL
Elevation confined to the disc
Small cupless disc Loss of cup late
Anamolous disc vessels (tri-, quadrifurcation)
Normal vessels
No hemorrhage or exudates
NFL hemorrhage, cotton wool spots, exudate
IIH TreatmentsIIH Treatments
Weight loss ( gastric surgery)Weight loss ( gastric surgery) Diuretics (Acetazolamide, Freusamide, Diuretics (Acetazolamide, Freusamide, ? Topirmate)? Topirmate) SteroidsSteroids ProceduresProcedures
-Optic nerve sheath fenestration (10% worse, -Optic nerve sheath fenestration (10% worse, 90% stabilize or improve)90% stabilize or improve)
-Lumbo-peritoneal shunt (50% failure rate)-Lumbo-peritoneal shunt (50% failure rate)- Venous sinus stenting- Venous sinus stenting- Repeated lumbar puncture ? “Schatz quote”- Repeated lumbar puncture ? “Schatz quote”
IIH TreatmentIIH Treatment
“ “ There is insufficient information to There is insufficient information to generate evidence-based generate evidence-based management strategy for IIH. Of the management strategy for IIH. Of the Various treatments available, there is Various treatments available, there is inadequate information regarding inadequate information regarding which are truly beneficial and which which are truly beneficial and which are potentially harmful”. are potentially harmful”.
Cochrane Review 2002Cochrane Review 2002
Idiopathic Intracranial Idiopathic Intracranial Hypertension Treatment Trial Hypertension Treatment Trial
Randomized clinical trial.Randomized clinical trial. In patients with mild visual loss ( MD -2 to In patients with mild visual loss ( MD -2 to
-5 db), is Diamox better than placebo ?-5 db), is Diamox better than placebo ? In patients with moderate visual loss (-5 to In patients with moderate visual loss (-5 to
-14 db), is ONSF with Diamox better than -14 db), is ONSF with Diamox better than Diamox alone ?Diamox alone ?
All patients will be on low salt, fluid-All patients will be on low salt, fluid-restricted diet.restricted diet.
Venous Sinus StentingVenous Sinus Stenting
Focal stenotic lesions in the lateral Focal stenotic lesions in the lateral sinuses.sinuses.
Higgins et al.Journal of Neurology Neurosurgery and Psychiatry 2004;75:621-625
Venous StentingVenous Stenting
Four patients underwent stenting had Four patients underwent stenting had improved headache (Owler et al. J improved headache (Owler et al. J Neurosurg 2003).Neurosurg 2003).
Direct retrograde cerebral venography Direct retrograde cerebral venography (DRCV) is more sensitive than MRV in (DRCV) is more sensitive than MRV in detecting venous thrombosis.detecting venous thrombosis.
Manometry showed a pressure gradient Manometry showed a pressure gradient with raised proximal venous pressure.with raised proximal venous pressure.
Does venous stenting really Does venous stenting really work ?work ?
King et al. showed that in IIH King et al. showed that in IIH patients, a pressure gradient across patients, a pressure gradient across a venous stenotic lesion can be a venous stenotic lesion can be eliminated by removal of CSF eliminated by removal of CSF through a cervical puncture .through a cervical puncture .
More longitudinal data are needed to More longitudinal data are needed to evaluate the efficacy of venous evaluate the efficacy of venous stenting.stenting.
Which shunt is better ?Which shunt is better ?
115 patients with IIH: 79 had LP and 36 had 115 patients with IIH: 79 had LP and 36 had ventriculo-atrial (VAT) or ventriculoperitoneal (VP) ventriculo-atrial (VAT) or ventriculoperitoneal (VP) shunts.shunts.
Headache only and no visual deficit.Headache only and no visual deficit. LP shunts had higher revision rate (RR=2.5) and LP shunts had higher revision rate (RR=2.5) and
obstruction (RR=3.0).obstruction (RR=3.0). LP shunt 86% vs VAT/VP 44% revision rate after LP shunt 86% vs VAT/VP 44% revision rate after
24 months.24 months. Ventricular shunts can placed by stereo-tactic, Ventricular shunts can placed by stereo-tactic,
MR-guided system.MR-guided system. (McGirt et al. J Neurosug 101:627-632, 2004).(McGirt et al. J Neurosug 101:627-632, 2004).
Case-Follow UpCase-Follow Up
Improvement on DiamoxImprovement on Diamox Resolution of symptomsResolution of symptoms Papilledema improvedPapilledema improved Diamox taperedDiamox tapered
Nine months later Nine months later Recurrence of symptomsRecurrence of symptoms No imaging doneNo imaging done Diamox restartedDiamox restarted Two therapeutic lumbar punctures Two therapeutic lumbar punctures
Four Months LaterFour Months Later
Altered Mental StatusAltered Mental Status CombativeCombative Complaining of severe headacheComplaining of severe headache Screaming, “I can’t see”Screaming, “I can’t see”
EMS calledEMS called Sedated and intubated in EDSedated and intubated in ED
Unable to attain VUnable to attain VAA
MRIMRI
MRIMRI
MRVMRV
MRVMRV
Hospital CourseHospital Course
Venous Sinus Venous Sinus ThrombosisThrombosis
Acute Renal FailureAcute Renal Failure Respiratory FailureRespiratory Failure Liver FailureLiver Failure ThrombocytopeniaThrombocytopenia AnemiaAnemia
Positive Lupus Positive Lupus AnticoagulantAnticoagulant
Negative: Anti-Negative: Anti-Cardiolipin Cardiolipin antibodyantibody
Negative: ANA, Negative: ANA, anti-dsDNA, pANCAanti-dsDNA, pANCA
Coagulation factors Coagulation factors WNLWNL
DiagnosisDiagnosis
Venous Sinus Thrombosis (VST) Venous Sinus Thrombosis (VST) secondary to Anti-phospholipid secondary to Anti-phospholipid Syndrome.Syndrome.
Venous Sinus ThrombosisVenous Sinus Thrombosis
Thrombosis and occlusion of major dural Thrombosis and occlusion of major dural sinus (saggittal, transverse, or sigmoid)sinus (saggittal, transverse, or sigmoid)
Increased intracranial pressureIncreased intracranial pressure Pseudotumor Cerebri SyndromePseudotumor Cerebri Syndrome 36-47% of patients36-47% of patients
Cortical hemorrhagic venous infarctionsCortical hemorrhagic venous infarctions SeizuresSeizures Focal neurological symptomsFocal neurological symptoms
Causes of VSTCauses of VST
InfectionsInfections Pregnancy relatedPregnancy related Behcet’s DiseaseBehcet’s Disease MalignanciesMalignancies CoagulopathiesCoagulopathies
30 %30 % SLESLE IdiopathicIdiopathic
17-27%17-27%
MedicationsMedications Oral contraceptivesOral contraceptives L-asparaginase L-asparaginase Tamoxifen, Ecstasy Tamoxifen, Ecstasy Androgenic steroidsAndrogenic steroids
DehydrationDehydration MechanicalMechanical
Post Lumbar Post Lumbar puncturepuncture
TraumaTrauma
Treatment of VSTTreatment of VST
Treatment of underlying causeTreatment of underlying cause AnticoagulationAnticoagulation
Systemic HeparinSystemic Heparin Coumadin (INR 2.0-3.0)Coumadin (INR 2.0-3.0)
Direct endovascular thrombolysisDirect endovascular thrombolysis UrokinaseUrokinase tPAtPA RheolyticRheolytic
Endovascular recanalizationEndovascular recanalization
Follow UpFollow Up
Thirty day admissionThirty day admission Treated for APSTreated for APS
Anticoagulation (Heparin Anticoagulation (Heparin Coumadin) Coumadin) High dose steroidsHigh dose steroids PlasmaphoresisPlasmaphoresis
DischargedDischarged Coumadin (INR 2.0-3.0)Coumadin (INR 2.0-3.0) PrednisonePrednisone
No recurrenceNo recurrence Bare LP OUBare LP OU
CaseCase
Two separate presentations of Two separate presentations of Pseudotumor cerebri syndrome in Pseudotumor cerebri syndrome in one patient.one patient. Idiopathic Intracranial HypertensionIdiopathic Intracranial Hypertension Venous Sinus ThrombosisVenous Sinus Thrombosis
SummarySummary
IIH should be diagnosed using specific IIH should be diagnosed using specific criteria.criteria.
Pseudopapilledema should be ruled out.Pseudopapilledema should be ruled out. MRI/MRV is the neuro-imaging of choice.MRI/MRV is the neuro-imaging of choice. The treatment of IIH is medical and The treatment of IIH is medical and
surgery is reserved for progressive visual surgery is reserved for progressive visual loss and/or persistent headache.loss and/or persistent headache.
Consider repeat imaging in atypical cases Consider repeat imaging in atypical cases ( fulminant course, poor response to ( fulminant course, poor response to treatment, focal neurologic signs).treatment, focal neurologic signs).