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).