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.

1. Neuro-ophthalmology
Symposium: Clinical Scenarios
and Evidence Review
Ra’ed Behbehani, MD, FRCSC,
Dipl.ABO

2. Objectives
 Review important and common neuro-
ophthalmic entities with emphasis on
diagnosis, and management based on the
current evidence.

3. Papilledema or No
Papilledema ?

4. Initial Presentation
 19 y/o AAF
 Pressure in retro-orbital and frontal regions
bilaterally
 Blurry vision
 Lightheadedness
 Symptoms worsen with any change in posture

5. Initial Presentation
 PMH: Seizure disorder
 POH: None
 Medications: Topamax 50mg BID
 Allergies: NKDA
 SH: ½ ppd tobacco, No EtOH, or drug use
 FH: NC
 ROS: negative

6. Initial Presentation
 VA sc: 20/20 OD 20/25 OS
 Pupils: No RAPD
 EOM: Full OU
 TA: 12 OD 12 OS
 SLE: WNL

7. Initial Presentation

8. Visual Fields

9. MRI

10. MRV

11. Lumbar Puncture
 Opening pressure of 45 cm H2O
 Glucose WNL
 WBC WNL
 Protein WNL

12. Modified Dandy Criteria
(Smith et al. J Clin Neuro-ophthalmol 1985)
1. Signs and symptoms of increased ICP.
2. No localizing neurological signs (except uni/
bilateral VI nerve palsy)
3. No evidence of an intracranial mass lesion
( by CT )
4. Normal CSF composition

13. Criteria for Idiopathic Intracranial
Hypertension
Friedman et al. Neurology 2002
1) If symptoms and signs are present, they
should reflect only increased ICP.
2) Documented increased ICP in the lateral
decubitus position (> 250 cm/H2O).
3) Normal CSF composition.
4) No evidence of hydrocephalus, mass, structural
or vascular lesion on CT with contrast for
typical patients, and MRI/MRV for all others.
5) No other cause of increased ICP found.

14. Secondary IIH
 Medical disorders : COPD, Severe hypertension,
sleep apnea syndrome, renal failure, Addison’s
disease, Hypoparathyroidism
 Medications : Tetracycline, Vitamin A, Anabolic
steroids, Nalidixic acid, Lithium, Levenorgestral
implant system.
 Venous obstruction: Cerebral venous sinus
thrombosis, jugular venous thrombosis.

15. Spinal Tap
 Keep the patient’s head neutral and legs
passively extended after needle in place.
 If the patient’s head is flexed and knees
drawn up Increased cephalic venous
pressure (Valsalva) compression of
Jugular vein and hpoventilation (CO2
retention)  Increased CSF pressure.
 An IIH patient needs only one spinal tap !

16. Is CT adequate ?
 Can detect hydrocephalus and most intra-
cranial lesions.
 Misses venous sinus thrombosis,
radiographic signs of meningeal
infiltration, and isodense tumors.
 If only alternative (weight, availability),
order contrast enhanced CT.

17. MRI and MRV
 Ideally for all patients.
 For atypical patients : (children with
recent sinus/ear infection, fulminant
course, non-responders to treatment).

18. “Benign intracranial hypertension”
(BIH).
Really ?!
http://medlib.med.utah.edu

19. Papilledema ?
Blood vessels
not obscured
by NFL
Anamolous
trifurcation
Drusen
Drusen
http://library.med.utah.edu/NOVEL/Hoyt

20. Papilledema ?
Drusen
Vessels not
obscured by
NFL
http://library.med.utah.edu/NOVEL/Hoyt

21. Features of Anamolous discs
(Peseuopapilledema)
Psudopapilledema True Papilledema
Disc vessels obscured Disc margin vessels clear
Elevation of peripapillary Elevation confined to the
NFL disc
Small cupless disc Loss of cup late
Anamolous disc vessels Normal vessels
(tri-, quadrifurcation)
No hemorrhage or NFL hemorrhage, cotton
exudates wool spots, exudate

22. IIH Treatments
 Weight loss ( gastric surgery)
 Diuretics (Acetazolamide, Freusamide,
? Topirmate)
 Steroids
 Procedures
-Optic nerve sheath fenestration (10% worse, 90%
stabilize or improve)
-Lumbo-peritoneal shunt (50% failure rate)
- Venous sinus stenting
- Repeated lumbar puncture ? “Schatz quote”

23. IIH Treatment
“ There is insufficient information to
generate evidence-based management
strategy for IIH. Of the Various treatments
available, there is inadequate information
regarding which are truly beneficial and
which are potentially harmful”.
Cochrane Review 2002

24. Idiopathic Intracranial Hypertension
Treatment Trial
 Randomized clinical trial.
 In patients with mild visual loss ( MD -2 to -5
db), is Diamox better than placebo ?
 In patients with moderate visual loss (-5 to -14
db), is ONSF with Diamox better than Diamox
alone ?
 All patients will be on low salt, fluid-restricted
diet.

25. Venous Sinus Stenting
 Focal stenotic lesions in the lateral
sinuses.
Higgins et al.
Journal of Neurology
Neurosurgery and
Psychiatry 2004;75:621-625

26. Venous Stenting
 Four patients underwent stenting had improved
headache (Owler et al. J Neurosurg 2003).
 Direct retrograde cerebral venography (DRCV)
is more sensitive than MRV in detecting venous
thrombosis.
 Manometry showed a pressure gradient with
raised proximal venous pressure.

27. Does venous stenting really work ?
 King et al. showed that in IIH patients, a
pressure gradient across a venous stenotic
lesion can be eliminated by removal of
CSF through a cervical puncture .
 More longitudinal data are needed to
evaluate the efficacy of venous stenting.

28. Which shunt is better ?
 115 patients with IIH: 79 had LP and 36 had ventriculo-
atrial (VAT) or ventriculoperitoneal (VP) shunts.
 Headache only and no visual deficit.
 LP shunts had higher revision rate (RR=2.5) and
obstruction (RR=3.0).
 LP shunt 86% vs VAT/VP 44% revision rate after 24
months.
 Ventricular shunts can placed by stereo-tactic, MR-
guided system.
(McGirt et al. J Neurosug 101:627-632, 2004).

29. Case-Follow Up
 Improvement on Diamox
 Resolution of symptoms
 Papilledema improved
 Diamox tapered
 Nine months later
 Recurrence of symptoms
 No imaging done
 Diamox restarted
 Two therapeutic lumbar punctures

30. Four Months Later
 Altered Mental Status
 Combative
 Complaining of severe headache
 Screaming, “I can’t see”
 EMS called
 Sedated and intubated in ED
 Unable to attain VA

31. MRI

32. MRI

33. MRV

34. MRV

35. Hospital Course
 Venous Sinus Thrombosis  Positive Lupus
 Acute Renal Failure Anticoagulant
 Respiratory Failure  Negative: Anti-
 Liver Failure Cardiolipin antibody
 Thrombocytopenia  Negative: ANA, anti-
 Anemia dsDNA, pANCA
 Coagulation factors
WNL

36. Diagnosis
 Venous Sinus Thrombosis (VST) secondary
to Anti-phospholipid Syndrome.

37. Venous Sinus Thrombosis
 Thrombosis and occlusion of major dural sinus
(saggittal, transverse, or sigmoid)
 Increased intracranial pressure
 Pseudotumor Cerebri Syndrome
 36-47% of patients
 Cortical hemorrhagic venous infarctions
 Seizures
 Focal neurological symptoms

38. Causes of VST
 Infections  Medications
 Pregnancy related  Oral contraceptives
 Behcet’s Disease
 L-asparaginase
 Tamoxifen, Ecstasy
 Malignancies  Androgenic steroids
 Coagulopathies  Dehydration
 30 %
 Mechanical
 SLE  Post Lumbar puncture
 Idiopathic  Trauma
 17-27%

39. Treatment of VST
 Treatment of underlying cause
 Anticoagulation
 Systemic Heparin
 Coumadin (INR 2.0-3.0)
 Direct endovascular thrombolysis
 Urokinase
 tPA
 Rheolytic
 Endovascular recanalization

40. Follow Up
 Thirty day admission
 Treated for APS
 Anticoagulation (Heparin  Coumadin)
 High dose steroids
 Plasmaphoresis
 Discharged
 Coumadin (INR 2.0-3.0)
 Prednisone
 No recurrence
 Bare LP OU

41. Case
 Two separate presentations of
Pseudotumor cerebri syndrome in one
patient.
 Idiopathic Intracranial Hypertension
 Venous Sinus Thrombosis

42. Summary
 IIH should be diagnosed using specific criteria.
 Pseudopapilledema should be ruled out.
 MRI/MRV is the neuro-imaging of choice.
 The treatment of IIH is medical and surgery is
reserved for progressive visual loss and/or
persistent headache.
 Consider repeat imaging in atypical cases
( fulminant course, poor response to treatment,
focal neurologic signs).