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neuro.idiopathic-intracranial-hypertension.v1

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

neurologyacutesubacutechronicadultpregnancyacuteoutpatientinpatienttransition
Start encounter →Print handoutPRODUCTION

Lane F id+neuro-acute campaign new-build (2026-05-26). IIH is a vision-threatening chronic-acute neurology engine with strong young-female + elevated-BMI + drug-trigger pivots. Engine encodes the Friedman 2013 revised diagnostic criteria (clean MRV REQUIRED), the Wall IIHTT acetazolamide RCT, the Mollan IIH:WT bariatric-surgery RCT, the Mollan 2018 JNNP consensus management guidelines, the four-pillar treatment ladder (weight loss + acetazolamide + topiramate + surgical rescue), and visual-field-driven severity stratification (HVF perimetric mean deviation, not visual acuity). PubMed-MCP verification 2026-05-26 — all 9 evidence PMIDs live-verified (Friedman 2013 PMID 23966248, Mollan consensus 2018 PMID 29903905, Mollan infographic 2018 PMID 30154235, Wall IIHTT 2014 PMID 24756514, Mollan IIH:WT 2021 PMID 33900360, Mollan IIH:WT substudy 2022 PMID 35790425, Abbott 2023 PMID 37813577, Markey/Mollan 2015 PMID 26700907, Gurney 2020 PMID 32021528). The orchestrator-suggested Yiangou venous-sinus-stenting PMID candidate did not resolve on PubMed and was substituted with the verified Gurney 2020 review (PMID 32021528). RxCUI live-verification (RxNav curl reverse-check 2026-05-26): acetazolamide 167 ✓, furosemide 4603 ✓, topiramate 38404 ✓, methazolamide 6826 ✓ (orchestrator-supplied candidate 6735 was INVALID — empty record), octreotide 7617 ✓ (orchestrator-supplied candidate 7651 was INVALID — empty record), liraglutide 475968 ✓ (GLP-1RA emerging adjunct anchored to Mitchell 2019 PMID 30865008). Registry-id resolution — used `workup.acute_headache` for ENTRY (headache-red-flag triage including IIH), `workup.weight_loss` for TREATMENT (disease-modifying weight loss anchored to Abbott 2023 PMID 37813577); `calc.ckd_epi_2021` for acetazolamide renal-adjusted dosing; panels `panel.cbc / panel.renal / panel.lft / panel.inflammation` all resolve. No IIH-specific workup or protocol exists in clinical-tools-registry.ts at this build. Schema-blocked: no `workup.iih` / `workup.papilledema` / `workup.elevated_icp` / `workup.lumbar_puncture` / `protocol.iih_acetazolamide_titration` in clinical-tools-registry.ts at this build — flagged for future depth-pass registry expansion. Settings shipped: ed / inpatient / outpatient / transition (4). Top-level dossier.settings uses `acute / outpatient / inpatient / transition` per EngineDossier schema. Severity triggers: 10 (fulminant / severe-papilledema / rapid-field-progression / pregnancy-with-vision-loss / drug-induced / transverse-sinus-stenosis-predominant / recurrence / ACZ-intolerance / atypical / IIH-WOP). §5.5.2 Bayesian depth-pass NOT performed at this build — Friedman 2013 LR derivations for individual criteria (papilledema sens/spec, opening-pressure cut-off), HVF perimetric-mean-deviation prognostic banding, and OCT RNFL prognostic threshold flagged as NEEDS_SOURCE_REVIEW for future depth-pass-2.

Entry points (6)

  • symptom
    Progressive headache in young woman with elevated BMI (≈90% of IIH; Friedman 2013 PMID 23966248)
    progressive_headache_obese_young_woman
  • symptom
    Transient visual obscurations (≈70% in IIH; seconds-long greyout with postural change / Valsalva; Mollan 2018 PMID 29903905)
    transient_visual_obscurations
  • symptom
    Pulsatile tinnitus (≈60% in IIH; turbulent flow at transverse-sigmoid stenosis; Mollan 2018 PMID 29903905; Gurney 2020 PMID 32021528)
    pulsatile_tinnitus
  • symptom
    Horizontal diplopia from CN VI palsy (non-localising sign of raised ICP; Friedman 2013 PMID 23966248)
    diplopia_cn_vi_palsy
  • symptom
    Papilledema on fundoscopy (REQUIRED for diagnosis except IIH-WOP; Friedman 2013 PMID 23966248)
    papilledema_on_fundoscopy
  • imaging
    MRI signs of raised ICP — empty sella, optic-nerve-sheath dilation/tortuosity, posterior-globe flattening, transverse-sinus stenosis (Friedman 2013 PMID 23966248)
    iih_mri_signs

Required inputs (15)

  • agerequired
    demographic • used at CONTEXT
    Adult IIH peaks in third/fourth decade; pediatric IIH has lower opening-pressure cut-off (≥28 cmH2O obese, ≥25 lean) and different sex distribution (Friedman 2013 PMID 23966248)
  • sexrequired
    demographic • used at CONTEXT
    IIH ~90% female of reproductive age; male IIH is atypical and lower prevalence but more likely to lose vision (Markey 2015 PMID 26700907)
  • bmirequired
    demographic • used at CONTEXT
    BMI ≥30 is the dominant modifiable risk factor; BMI ≥35 is the IIH:WT enrolment threshold and the strongest bariatric-surgery indication (Mollan IIH:WT 2021 PMID 33900360; Abbott 2023 PMID 37813577)
  • papilledema_grade_frisenrequired
    symptom • used at INITIAL_WORKUP
    Papilledema is a Friedman 2013 required criterion; Frisén grade 0-5 and OCT RNFL drive severity stratification — grade ≥3 + RNFL >300 µm marks severe (Friedman 2013 PMID 23966248; Wall IIHTT 2014 PMID 24756514)
  • opening_pressure_cmh2orequired
    lab • used at INITIAL_WORKUP
    CSF opening pressure (cmH2O) measured laterally with legs extended; Friedman 2013 ≥25 adult, ≥28 in obese, ≥28 in sedated/obese children, ≥25 in lean children — REQUIRED for diagnosis (Friedman 2013 PMID 23966248)
  • csf_compositionrequired
    lab • used at INITIAL_WORKUP
    Normal CSF cell count, protein, glucose REQUIRED for IIH diagnosis; pleocytosis or elevated protein excludes IIH and points to meningitis / inflammatory / neoplastic cause (Friedman 2013 PMID 23966248)
  • mri_brain_with_mrvrequired
    imaging • used at INITIAL_WORKUP
    MRI brain + MRV REQUIRED to exclude mass / hydrocephalus / CSF leak / CVST; positive IIH signs include empty sella, ONS dilation, transverse-sinus stenosis (Friedman 2013 PMID 23966248; Mollan 2018 PMID 29903905)
  • humphrey_visual_field_24_2required
    imaging • used at INITIAL_WORKUP
    Humphrey visual field 24-2 perimetric mean deviation is the IIHTT operative outcome measure (NOT visual acuity) — quantifies field loss and drives severity / surgical-rescue decisions (Wall IIHTT 2014 PMID 24756514)
  • oct_rnflrequired
    imaging • used at INITIAL_WORKUP
    OCT RNFL thickness quantifies papilledema severity beyond Frisén grading; RNFL >300 µm marks severe papilledema; trend correlates with treatment response (Wall IIHTT 2014 PMID 24756514; Mitchell 2019 PMID 30865008)
  • creatininerequired
    lab • used at TREATMENT
    eGFR drives acetazolamide renal-adjusted dosing (avoid if CrCl<10; reduce in CKD) and topiramate adjustment (Mollan 2018 PMID 29903905)
  • bicarbonaterequired
    lab • used at TREATMENT
    Baseline + serial HCO3 monitoring under acetazolamide (carbonic-anhydrase inhibition causes metabolic acidosis; symptomatic acidosis or HCO3 <18 → dose-reduce; Wall IIHTT 2014 PMID 24756514)
  • bhcg_pregnancy_testrequired
    lab • used at CONTEXT
    Pregnancy contraindicates acetazolamide (FDA category C / animal teratogenic; pragmatic avoidance) and topiramate (cleft palate); pregnancy IIH managed with serial LP + ophthalmology shared-decision (Mollan 2018 PMID 29903905)
  • iih_drug_triggersrequired
    medication • used at CONTEXT
    IIH-trigger medications (tetracyclines, vitamin A / retinoids, cyclosporine, lithium, growth hormone, all-trans retinoic acid) — drug-induced secondary intracranial hypertension is reversible on withdrawal; Friedman 2013 mandates exclusion + de-prescription (Friedman 2013 PMID 23966248; Mollan 2018 PMID 29903905)
  • recent_weight_gain
    history • used at CONTEXT
    Recent weight gain (5-15% over months) is a major precipitant in adult IIH and a recurrence trigger after remission (Markey 2015 PMID 26700907; Abbott 2023 PMID 37813577)
  • pcos_obstructive_sleep_apnea
    history • used at CONTEXT
    PCOS and OSA are over-represented in IIH cohorts and share an obesity-hyperandrogenism axis; OSA worsens nocturnal ICP and may co-treat (Markey 2015 PMID 26700907; Markey 2016 PMID 27186074)

12-phase flow (12)

  1. 1FRAME
    Vision-threatening intracranial hypertension in a young woman with elevated BMI; pivot on headache + papilledema + normal neuro exam + normal imaging + raised LP opening pressure + normal CSF (Friedman 2013 PMID 23966248)
    advance: IIH suspected on syndromic grounds (papilledema + headache phenotype)
  2. 2ENTRY
    Headache (≈90%), transient visual obscurations (≈70%), pulsatile tinnitus (≈60%), CN VI palsy diplopia, and incidental papilledema on routine fundoscopy (Mollan 2018 PMID 29903905)
    inputs: age, sex, bmi
    actions: workup.acute_headache
    advance: Headache-and-papilledema phenotype documented
  3. 3CONTEXT
    Obesity (BMI ≥30), recent weight gain, PCOS, OSA, drug triggers (tetracyclines / retinoids / cyclosporine / lithium / GH), pregnancy / postpartum (Friedman 2013 PMID 23966248; Mollan 2018 PMID 29903905)
    inputs: bmi, iih_drug_triggers, bhcg_pregnancy_test, recent_weight_gain, pcos_obstructive_sleep_apnea
    advance: Risk substrate + drug history captured
  4. 4RED_FLAGS
    Fulminant IIH (vision loss <4 wk), severe papilledema (Frisén 4-5), rapid field-loss trajectory, pregnancy with worsening vision, GCS decline, headache with new focal deficit → STAT ophthalmology + neurosurgery (Mollan 2018 PMID 29903905)
    inputs: papilledema_grade_frisen, humphrey_visual_field_24_2
    advance: Fulminant phenotype either confirmed or excluded
  5. 5INITIAL_WORKUP
    MRI brain + MRV (exclude CVST + structural cause + map IIH-positive signs); LP with opening pressure (lateral decubitus, legs extended); CSF cell count, protein, glucose; HVF 24-2 + OCT RNFL; CBC, CMP, HCO3 baseline (Friedman 2013 PMID 23966248; Mollan 2018 PMID 29903905)
    inputs: mri_brain_with_mrv, opening_pressure_cmh2o, csf_composition, humphrey_visual_field_24_2, oct_rnfl, papilledema_grade_frisen, creatinine, bicarbonate
    actions: panel.cbc, panel.renal, panel.lft, panel.inflammation, workup.weight_loss
    advance: Friedman 2013 criteria met OR alternative diagnosis confirmed
  6. 6BRANCHING_WORKUP
    If MRV shows transverse-sinus stenosis with pressure gradient → endovascular consult for stenting candidate; if pregnancy → MFM + ophthalmology co-management; if drug-induced → de-prescribe and reassess at 4-8 wk; if atypical (male, lean, child) → expand secondary-cause workup (Gurney 2020 PMID 32021528; Mollan 2018 PMID 29903905)
    advance: Phenotype subtype mapped (typical / atypical / drug-induced / pregnancy / fulminant / venous-stenosis-predominant)
  7. 7DIFFERENTIAL
    CVST (separate engine; MRV-positive), CSF leak (orthostatic headache, low-pressure phenotype), pituitary mass, malignant hypertension, dural AVF, IIH-without-papilledema (rare), chronic migraine without papilledema (Friedman 2013 PMID 23966248; Mollan 2018 PMID 29903905)
    advance: IIH confirmed by Friedman 2013 criteria + secondary causes excluded
  8. 8RISK_STRATIFICATION
    Severity is VISUAL-FIELD-driven (HVF perimetric mean deviation), NOT symptom-driven: mild = preserved fields + Frisén 1-2 (acetazolamide + weight loss); moderate = mild field loss + Frisén 3 (escalate ACZ to 4 g/day); severe = rapid field loss / Frisén 4-5 / RNFL >300 µm (surgical candidate); fulminant = vision loss <4 wk (urgent ONSF / CSF shunt / serial LP) (Wall IIHTT 2014 PMID 24756514; Mollan 2018 PMID 29903905)
    inputs: humphrey_visual_field_24_2, papilledema_grade_frisen, oct_rnfl
    advance: Severity tier assigned: mild / moderate / severe / fulminant
  9. 9TREATMENT
    Pillar 1 — weight loss 5-15% (lifestyle for BMI<35; bariatric surgery for BMI≥35 per Mollan IIH:WT 2021 / Abbott 2023); Pillar 2 — acetazolamide PO BID titrated to 1-4 g/day in divided doses (IIHTT); Pillar 3 — topiramate 25-100 mg/day (weight-loss bonus, teratogenic — counsel); Pillar 4 — surgery for severe / fulminant (ONSF for vision-failure-rescue; CSF shunt for headache-dominant; venous-sinus stenting for venous-stenosis-predominant); furosemide adjunct; methazolamide if ACZ intolerant (Wall IIHTT 2014 PMID 24756514; Mollan IIH:WT 2021 PMID 33900360; Mollan 2018 PMID 29903905; Gurney 2020 PMID 32021528)
    inputs: bmi, creatinine, bicarbonate, bhcg_pregnancy_test
    advance: Treatment pathway selected + ophthalmology surveillance scheduled
  10. 10DISPOSITION
    Outpatient for typical mild-moderate IIH; admit for fulminant IIH / surgical candidate / acute vision loss / status migrainosus; obstetric admission for pregnancy-IIH with worsening vision (Mollan 2018 PMID 29903905)
    advance: Disposition decided + follow-up booked
  11. 11MONITORING
    Serial ophthalmology (HVF + OCT) at 1-2 wk acute → monthly until stable → 3-6 monthly long-term; serial weight + BMI; serial HCO3 / K on acetazolamide; pregnancy-specific monthly visits (Mollan 2018 PMID 29903905; Wall IIHTT 2014 PMID 24756514)
    inputs: humphrey_visual_field_24_2, oct_rnfl, bicarbonate, bmi
    advance: Monitoring cadence + thresholds documented
  12. 12FOLLOWUP
    Lifelong relapse surveillance — recurrence with weight regain is common; reinforce weight-maintenance + bariatric-surgery referral if BMI ≥35 sustained; PCOS / OSA co-management; contraception counselling (avoid GH, retinoids, tetracyclines lifelong); pregnancy planning + monitoring algorithm (Abbott 2023 PMID 37813577; Mollan 2018 PMID 29903905)
    advance: Long-term plan + recurrence-trigger education delivered