GRAND ROUND  Cc. Headache of 04 months - globbal,dullaching,inc. in severity - Sts. awaken her from sleep - temporal improv’t with analgesics  ass’d nausea and vomiting  diplopia and blurring of vision of 2 months  tinnitus but no dizziness or vertigo

Ctd.  no similar history in the past  no abnormal body mov’t or weakness of extremitis  no history of fever  no chronic cough;no intake of drugs  increase 8kg of wt. Over 1yr  no chronic illnesses in the past

ctd  has regular menses  single and lives with her family P\E GA:healthy looking BP=100\80 PR=80 RR=16 BMI=24.7  no pallor, NIS  no LAP

ctd  Chest,Cvs,Abdomen/NAD  CNS:conscious,oriented to TPP - language,memory,attention/Intact -cranial nerves: Normal findings -Fundoscopy:swollen disc with blurred disc marigin -visual acuity:6/6

CNS exam’n ctd Visual field-normal by confront’n method motor and sensory :normal findingsr reflexes:2/4 allover plantar-downgoing bilaterally cerebellar signs-absent

summary 24 yrs old female patient with 04 months history of headache and 02 months history of visual complaints Fundoscopy showing evidence of papilledema

Differential Diagnosis Intracranial mass Hydrocephalus Meningeal process(infectious,inflammatory, neoplastic) Inc’d venous press./Cerbral venous thromb. Idiopathic intracranial HPNm

Lab. Results WBC=4500 Hgb=14.8gm/dl Plt=76000 ESR=45mm/hr serum VDRL-NR CT scan of brain-Normal CT findings LP-opening press. >300mm of water clear CSF No cell,glucose 70mg/dl,protein0.2gm/l CSF VDRL-NR,gram s.and AFB-No organism.

Idiopathic intracranial HPN also called pseudotumor cerebri,benign ICH a disorder of unknown etiology primary problem is chronically inc. ICP most important neurologic manifestation is papilledema

Pathophysiology Unclear multiple studies with conflicting results some of proposed mechanisms increased CSF production;decd. absorp’n cerebral edema elevated cerebral venous pressure role of obesity

Frequency  variable from country to country  Annual incidence at Mayo clinic( ) 0.9/ pop’n 1.6/ women 3.3/ females aged yrs 7.9/ obese women aged yrs  F:M=8:1 obese women of child bearing age

Clinical Findings  Symptoms of increased ICP - headache,pulsatile tinnitus,diplopia  symptoms of papilledema - transient visual obscursions,progressive - loss of vision,blurring of vision - sudden visual loss  There are pts. with IIH without papilledema - In one study of 65 adults with refractory migrane,12(18% had IIH without papilledema

Cont’d  Visual function testing - fundoscopy,visual field,visual acuity - color vision,ocular motility  characteristics,Sxs,Sns in pts. with IIH - pt. Characterstics - symptoms female(65-95%) Headache(75-99%) Age peak:21-34yrs Visual dist.(30-68%) obesity (44-94%) diplopia (20-38%) Intracranial noises(0-80%)

Cont’d  Signs - papilledema(98-100%) - VF defects (3-51%) - abducent palsy(14-35%) - Dec’d VA (2-25%) Risk Factors Conditions Endocrine diseases female sex Addisons disease Reproductive age gp. Cushing’s disease Obesity Hypoparathyroidism Recent weight gain Hypothyroidism

Risk Factors cont’d Miscellaneous diseases CRF,SLE,Anemia,Hypervitaminosis A,Dural AV malf. Medications - Multivitamines(vit. A),steroids and steroid withdrwal TTC,sulfa Abics.,cimetidine,naldixic acid,nitrofurantoin amiodarone,tamoxifen,cyclosporine,lithium carbonate

Diagnosis  a dignosis of exclusion  Based on modified Dandy criteria 1.signs and symptoms of raised ICP 2.No localizing neurologic signs,in an alert patient, other than abducens n. palsy 3.Normal neuroimaging studies,except for small ventricles and empty sella 4.Documented inc’d opening pressure(>250mm of water) but normal CSF composition 5.Primary structural or systemic causes of elevated intracranial venous sinus pressure excludedM

Diagnosis cont’d  Neuroimaging - for structural abns. or mass lesions - Brain MRI with gadolinium enhancement - MRI venography,CT scan  LP  Orbital ultrasonography  Other lab tests - CBC,ESR,ACLA,ANA,Full procoagulant profile

Treatment  Joint Mx with ophthalmologist and neurologist  Treatment goals to detect and prevent visual loss to reduce ICP to relieve headache  Medical and surgical options

Medical therapy  Treatment of associated condition - withdrawal of offending agent - treatment of obesity as low as 6%loss of wt. results in dec’d ICP and papilledema  Diuretics - Acetazolamide- first line medical therapy -250mg po qid or 500mg po bid - Loop diuretics,Eg. Furesemide: as an adjunct to acetazolamide

Medical therapy cont’d  Corticosteroids -rapidly lower ICP -long term use not recommended -for patients who continue to have visual loss  Repeated LP - in patients with infrequent exacerbations of symptoms

Surgical therapy  When visual function is severly impaired  To those with incapacitating headache  Options - optic nerve sheath decompression (fenestration) -lumboperitoneal or ventriculoperitoneal shunting

Prognosis  Encouraging in early intervetion  Prognosis for visual loss,varied in d/f series - studies of 1960’s and 1970’s,<25% of pts. Had significant blindness -recent study,visual dysfunction in close to ½ of patients