Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) David I. Kaufman, D.O. Michigan State University Department of Neurology and Ophthalmology

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1 Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) David I. Kaufman, D.O. Michigan State University Department of Neurology and Ophthalmology

2 26 year old 5 3, 300 pound female with papilledema, headache and TVO. Visual acuity 20/20 OU. Increased blind spots with nasal depression OD on fields. MRI and CSF are normal. Opening pressure 320.

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5 Idiopathic Intracranial Hypertension IIH or pseudotumor cerebri is a disease of women in the childbearing years characterized by elevated intracranial pressure of unknown cause Incidence 1 per 100,000 per year / 100,000 in obese women in the childbearing years Over 90% of patients are women Over 90% are obese

6 Idiopathic Intracranial Hypertension Diagnosis Modified Dandy Criteria Signs and symptoms of increased intracranial pressure. Absence of localizing findings on neurologic examination. Normal imaging* and neurodiagnostic studies except for evidence of elevated cerebrospinal fluid pressure.

7 Idiopathic Intracranial Hypertension What Experience Has Taught Me 90% or more are ladies age 14 to 50 that are obese. Men rarely involved but when they are, they re huge or have sleep apnea.

8 Idiopathic Intracranial Hypertension What Experience Has Taught Me Pulsitile tinnitus is common. Headache is very common. Transient visual obscurations with bending over or bowel movement is common.

9 IIH Symptoms (%) Prospective data collection Giuseffi et al., Neurology 1991

10 IIH Symptoms A case control study Best symptom to differentiate Giuseffi et al., Neurology 1991

11 Idiopathic Intracranial Hypertension What Experience Has Taught Me Sixth nerve palsy is uncommon but not rare. Third nerve palsy can occur but very rarely. No additional neurologic signs other than papilledema.

12 Idiopathic Intracranial Hypertension Interventions Tempo of visual loss dictates how aggressive therapy needs to be.

13 Visual Field Loss based on prospective study 25% reported symptom of visual loss confrontation abnormal in 32% Goldmann abnormal in 96% Automated abnormal in 92% Brain 1991;114:

14 Blindness in IIH 5-10% of patients

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16 Mechanisms of Visual Loss in IIH axoplasmic flow stasis leading to axonal ischemia at the optic disc spread of edema into macula (seen best with OCT) transient hypotension watershed infarct choroidal folds (refractive) elevated peripapillary retina (refractive)

17 The Prototype Inferonasal Step

18 Grades of visual loss in IIH Grade I Grade II Grade III Stages of progression Grade IV Grade V

19 Idiopathic Intracranial Hypertension Treatment Weight Loss, Low Na + Diet Diamox 500 to 1500mg BID Additional medication Surgery

20 Idiopathic Intracranial Hypertension McCarthy and Reed showed Diamox reduced CSF production Lubow and Kuhr used Diamox and weight loss successfully

21 Idiopathic Intracranial Hypertension Resolution of papilledema with Diamox documented by Tomsak

22 Idiopathic Intracranial Hypertension Interventions There is bias that weight loss and Diamox are cornerstones of therapy. No strategy verified by properly designed clinical trials.

23 IIH Treatment There is insufficient information to generate an evidence-based management strategy for idiopathic intracranial hypertension. Of the various treatments available, there is inadequate information regarding which are truly beneficial and which are potentially harmful. Properly designed and executed trials are needed. Lueck C, McIlwaine G. Interventions for Idiopathic Intracranial Hypertension. Cochrane Database of Systematic Reviews 2002

24 The Need for a Trial IIH treatment data is anecdotal Optimal management is unknown Long-term outcomes are unclear Etiology unknown

25 Idiopathic Intracranial Hypertension Treatment Trial NEI Sponsored Double Blind, Placebo-controlled Clinical Trial Supported by NIH U10 EY017281

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27 Multiple Principal Investigators in USA and Canada Wayne Cornblath, MD Ann Arbor, MI Eric Eggenberger, DO East Lansing, MI Robert Granadier, MD Royal Oak, MI

28 Idiopathic Intracranial Hypertension Treatment Trial 154 Individuals. Multi-center, double masked placebo controlled study. Diet and Diamox versus diet and placebo.

29 Main entry criteria Age years Meets modified Dandy criteria of IIH Newly diagnosed (6 weeks or less) Presence of papilledema PMD -2 db to -5 db in the worst eye

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31 Identification of the cause of IIH : Specific Aim 2 screen a cohort of IIH patients and controls for diseasecausing mutations and IIH risk alleles. determine if polymorphisms in these genes are disproportionately represented in IIH patients. potential hormonal mediators and biomarkers will be analyzed based on the SNP s

32 Grades of visual loss in IIH Grade I Grade II Grade III Stages of progression Grade IV Grade V

33 Idiopathic Intracranial Hypertension Poor Prognostic Signs Rapid tempo of visual loss. Recent onset of associated headache with significant papilledema and progressive visual loss.

34 Grade V Papilledema Criteria of Grade IV + partial or total obscuration of all vessels on the disc

35 Idiopathic Intracranial Hypertension Surgical Interventions Optic nerve sheath fenestration. CSF shunting procedures.

36 Idiopathic Intracranial Hypertension What Experience Has Taught Me

37 Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) Obese women Age 14 to 50 Normal imaging* Elevated intracranial pressure with normal formula

38 Idiopathic Intracranial Hypertension Associated Symptoms Headache Pulsitile Tinnitus Sleep Apnea Sixth Nerve Palsy Transient Visual Obscurations

39 Idiopathic Intracranial Hypertension Interventions Tempo of visual loss dictates how aggressive therapy needs to be.

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41 Idiopathic Intracranial Hypertension Treatment Trial NEI Sponsored Double Blind, Placebo-controlled Clinical Trial Supported by NIH U10 EY017281

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43 Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) David I. Kaufman, D.O. Michigan State University Department of Neurology and Ophthalmology