This document summarizes pseudotumor cerebri (PTC), also known as idiopathic intracranial hypertension (IIH). It describes a case of a 39-year-old obese female with PTC symptoms including headaches, visual issues, and papilledema. PTC is characterized by increased intracranial pressure without a tumor. Treatment involves weight loss, medications like acetazolamide, surgical procedures like optic nerve sheath fenestration or shunt placement, and managing any underlying causes. Complications can include permanent vision loss if not properly treated.

1. Pseudotumor cerebri
Dr. Amit

2. • A 39-year-old obese female laboratory technician
has been complaining of headache x2 years.
• Transient visual obscuration x 6 month
• B/l diplopia x 4 month
• O/E- E4V5M6/PEARL/B/L 6 NERVE PALSY GRADE
4 PAPILLEDEMA
• EVALUATION-CT BRAIN- small ventricle
• MRI WITH MRV = NORMAL

3. condition characterized by increased
intracranial pressure (ICP) without evidence
of dilated ventricles or a mass lesion by
imaging, normal cerebrospinal fluid (CSF)
content, and papilledema occurring in most
cases

4. • The condition was probably first described by
Quincke in 1897
• the term pseudotumor cerebri was given by
NONNE in 1914
• Foley suggested the condition “benign
intracranial hypertension

5. Pseudotumor Cerebri
• idiopathic intracranial hypertension (IIH)
• secondary pseudotumor cerebri

6. EPIDEMIOLOGY
• The idiopathic form ( IIH) represents about
90% of cases
• A disorder of obese females of childbearing
age

7. EPIDEMIOLOGY
• In the United States
• Annual incidence in Iowa and Louisiana (Durcan,
1988)
• the incidence to be approximately 0.9 per
100,000 in the general population
• female-to-male ratio of 8 : 1.
• The incidence increases to 3.5 per 100,000 in
women aged 20 to 44 years
• 13 per 100,000 in women who are 10% over ideal
weight

8. Secondary PTC
• About 10% of cases
• May develop at any age
• Equal frequency in both sexes

12. PATHOPHYSIOLOGY
• Mechanical theory: Obesity--intraabdominal
pressure – decrease venous return- increase
dural sinus pressure –decrease CSF resorption
– increase intracranial pressure
• Hormonal theory: adipocytes convert
androstenedione-- estrone---- CSF production

13. • Recent theory suggest defect at the level of
archanoid granulation
• Transverse sinus / Sup.sagital sinus
thrombosis, stenosis

14. SYMPTOMS AND SIGNS
• Headache-most common symptom
• Occurs in approximately 90% of cases

15. transient obscurations of vision (TOVs
• TOVs indicate the presence of optic disc
swelling
• 70% of patients
• unilateral or bilateral
• generally last only a few seconds

16. • A small percentage of patients with PTC
complain of visual loss

17. • Diplopia- present in approximately 40% of
patients
• Pulsatile tinnitus
• Focal neurological deficits in patients with PTC
are extremely uncommon

18. Papilledema
• Papilledema is the diagnostic hallmark of PTC
• Present in almost all patients.
• It is almost always bilateral and symmetrical

20. Papilledema Grading System (Frisén
Scale)
• STAGE 0—NORMAL OPTIC DISC
• Blurring of the nasal, superior, and inferior
poles in inverse proportion to disc diameter
• Radial nerve fiber layer without tortuosity
• Rare obscuration of a major blood vessel,
usually on the upper pole

21. STAGE 1—VERY EARLY PAPILLEDEMA
Obscuration of the nasal border of the disc
No elevation of the disc borders
Disruption of the normal arrangement of
radial nerve fiber layers with
a grayish opacity accentuating the nerve fiber
layer bundles
Normal temporal disc margin

23. STAGE 2—EARLY PAPILLEDEMA
• Obscuration of all borders
• Elevation of the nasal border
• Complete peripapillary halo

25. STAGE 3—MODERATE PAPILLEDEMA
• Obscurations of all borders
• Increased diameter of the optic nerve head
• Obscuration of one or more segments of
major blood vessels leaving the disc
• Peripapillary halo—irregular outer fringe with
finger-like extensions

27. STAGE 4—MARKED PAPILLEDEMA
• Elevation of the entire nerve head
• Obscuration of all borders
• Peripapillary halo
• Total obscuration on the disc of a segment of a
major blood vessel

29. STAGE 5—SEVERE PAPILLEDEMA
• Dome-shaped protrusions representing
anterior expansion of the optic nerve head
• Narrow and smoothly demarcated
peripapillary halo
• Obliteration of the optic cup

31. COMPLICATIONS
• The most feared complication of PTC is
permanent visual loss
• In one long-term study performed before the era
of modern neuroimaging,
• 57 patients with a diagnosis of PTC were
monitored for 5 to 41 years.
• Severe visual impairment occurred in one or both
• eyes in 14 patients (24.5%)
• 7 patients had the visual loss

32. • In a more recent prospective study of 50
patients treated for IIH
• visual acuity was worse than 20/20 in 13%.

35. Modified Dandy's criteria for IIH
• Documented increased ICP
• No intracranial or spinal mass
• Normal CSF contents
• No evidence of hydrocephalus

36. Neuroimaging

37. CT BRAIN
• Usuallu normal
• Occasionally slit ventricles may seen

38. MRI
• Intracranial abnormalities are usually absent
or minimal

39. • 1. Flattening of the posterior sclera: occurs in
80%
• 2. Enhancement of the prelaminar optic nerve: in
50%
• 3. Distention of the perioptic subarachnoid space:
in 45%
• 4. Vertical tortuosity of the orbital optic nerve: in
40%
• 5. Intraocular protrusion of the prelaminar optic
nerve: in 30%

40. Venography
• To rule-out dural sinus or venous thrombosis

41. Examination of Cerebrospinal Fluid
• CSF pressure: > 25 diagnostic
• Pressures 20-24.9 are nondiagnostic
• Pressure < 20 is normal

42. CSF composition:
• Normal glucose and cell count

43. TREATMENT
• Treatment of PTC is a teamwork requiring
concurrent management from more than one
physician.
• A neurologist, ophthalmologist, primary care
physician, and neurosurgeon

44. • The presence and severity of symptoms such
as headache
• The degree of visual loss at initial examination
• The rate of progression of visual loss
• The presence of an identifiable underlying
cause
• Detection of factors known to be associated
with a poorer visual prognosis

45. • Pt with mild papilledema with no headache
• Close observation
• Treat underlying cause

46. Treatment Related to Obesity
• Kupersmith et al. reported improvement in
headaches and papilledema in 58 obese
females with IIH who lost 7% to 10% of their
weight over a 3-month period

47. MEDICAL MANAGEMENT
• Mild- moderate headche
• Good visual function

48. • Acetazolamide (Diamox)
• Methazolamide
• Topiramate
• Diuretics such as furosemide
• Systemic corticosteroids

49. Surgical Procedures
• Moderate or severe papilledema
• Deterioration of visual acuity or worsening of
visual fields despite medical therapy

50. • CSF diversion to lower ICP
• ONSF
• stenting of venous sinuses
• Bariatric surgery for obese patients

51. Shunt Surgery
Moderate to sever headache
progressive visual disturbance

52. • Ventri peritoneal shunt
• Lumboperitoneal shunt
• Ventri atrial shunt

53. • Tulipan et al placed ventriculoperitoneal (VP)
shunts in seven patients
• Papilledema resolved in five patients
• two patients had mild residual papilledema
• Headaches resolved in all

54. Optic Nerve Sheath Fenestration
• Moderate to sever visual disturbance
• Mild headache

55. • Banta et al. described the outcomes of 158
ONSFs performed in 86 patients with IIH
• Improvement or stabilization of visual acuity
and visual fields was observed in 94% and 88%
patients
• Most common being diplopia (34.86%) and
anisocoria (7.5%)

56. • Sergott et al. reported the use of a modified
ONSF IN 32 pt. wherein multiple longitudinal
fenestrations were created and subarachnoid
adhesions lysed. These authors noted
improvement in 21 of 23 patients (91.3%) with
IIH

57. COMPLICATION
• Early/Late visual failure

58. Venous Sinus Stenting
• Moderate to sever headache
• Progressive visual disturbance
• MRV – Dural sinus thrombosis / stenosis
• Manometer showing raised pressure gradient
across stenosis

59. • Bussiere et al. reported transverse sinus
stenosis in 13 patients with IIH, 10 of whom
underwent stenting of the affected sin
• Almost complete resolution of papilledema
was seen in eight patients and significantly
improved in two others

60. Management of IIH in Pregnancy
• Rule out dural sinus thrmbosis
• Avoid use of acetazolamide in first trimester
• Shunt revision

61. Fulminant Pseudotumor Cerebri
significant visual field loss, central visual
acuity loss, and marked papilledema on initial
examination.
• Insertion of a lumbar drain until a definitive
shunt procedure or ONSF

62. CONCLUSION
• Early diagnosis
• Early treatment
• Follow up

63. • Thank you