Morning Report: Tuesday, March 6th

AKA: Pseudotumor Cerebri

Elevated ICP without any evidence of neurologic disease

Adults Female predilection Rare in adults older than 45 (most common ages 20-44) Strong association with obesity Female predilection after puberty Rare before age 10 (before puberty) Association with obesity increases with age Children *No racial predisposition or genetic locus

Elusive!! Absence of an increase in ventricular size despite increased ICP also puzzling Vision loss Transmission of high ICP to optic nerve head  axoplasmic stasis and microvascular compromise

Diagnosis of EXCLUSION!! No other identifiable neurologic disease Numerous “associations” with IIH Nomenclature dictates that identifiable causative factors be excluded from the diagnosis of IIH and be referred to as “secondary causes of intracranial HTN.”

History HA Worse in AM Awaken patient from sleep Increase with Valsalva Nausea/ vomiting Ophthalmic symptoms Decreased/ blurred vision Diplopia Transient visual obscurations Other: ataxia, dizziness, neck/shoulder/back pain, stiff neck, facial or limb paresthesias, facial nerve palsy, pulsatile tinnitus

Physical Exam Ophthalmologic exam Papilledema*

Physical Exam Ophthalmologic exam (con’t) Afferent pupillary defect Color vision defecit Loss of visual acuity Uni-or bilateral sixth nerve palsy Third or fourth nerve paresis Neurologic exam Excluding ophthalmic findings, exam should be NORMAL!

MRI/MRV Imaging studies of choice for IIH Exclude the possibility of herniation prior to LP (older children and adults) Identify secondary causes of increased ICP Sinus or venous thrombosis Malignancy Meningeal abnormalities Gliomatosis cerebri Should be normal except for signs of increased ICP Ventricles should be of normal to small size

CSF studies Elevated opening pressure (> mm H20) Normal cell count, protein and glucose Absence of infection Ancillary studies Lots of ophtho specific testing that I have NEVER heard of….AND Visual field testing More sensitive than visual acuity and contrast sensitivity testing in the detection of worsening disease

Medical Acetazolamide Carbonic anhydrase inhibitor that reduces CSF production mg/kg/d, max 2g/d Contraindicated in sulfa allergy and in significant renal or liver dz Furosemide Can be used in combination with or instead of acetazolamide Effect on CSF production weaker than acetazolamide Corticosteroids Should be administered with caution Used in conjunction with diuretics to treat children who’s response to diuretics was poor

Surgical Optic nerve sheath decompression CSF shunting Weight management +/- bariatric surgery

*Vision loss can be permanent!* Predictors of vision loss in IIH Recent weight gain Subretinal hemorrhage Significant visual field loss at presentation HTN High-grade papilledema Disc appearance cannot be used to predict final outcome CLOSE follow-up 1 month intervals for 6-12 mos after the disease has stabilized, then less frequently for a minimum of 5 years

Noon Conference: Mead Johnson Webinar, Pediatric Mental Health (LUNCH PROVIDED!!!)