Neurology · Year 3 · from Neurology
Case 3: Secondary Headache - Idiopathic Intracranial Hypertension
Patient Demographics
- Age: 32 years
- Sex: Female
- Occupation: Administrative assistant
- BMI: 38 kg/m²
Chief Complaint
"My headaches are getting worse and sometimes my vision goes gray."
History of Present Illness
The patient reports progressive daily headaches over the past 4 months. The headache is generalized, pressure-like, worse in the morning and with bending over, coughing, or straining. She hears a "whooshing" sound in her ears that pulses with her heartbeat (pulsatile tinnitus). She has transient visual obscurations - brief episodes lasting seconds where her vision "grays out," especially when standing up or bending. She has noticed peripheral vision loss and intermittent horizontal diplopia. She gained 30 lbs over the past year. Recent medications include oral contraceptive pills.
Neurological Examination Findings
Mental Status:
- Alert, oriented, appropriate
Cranial Nerves:
- Visual acuity: 20/25 right, 20/30 left
- Fundoscopy: Bilateral papilledema (Grade II) - blurred disc margins, obscuration of vessels
- Visual fields: Enlarged blind spots bilaterally, inferonasal field constriction
- Pupils: Normal reactivity
- Left CN VI palsy - esotropia in primary gaze, cannot abduct left eye (false localizing sign of increased ICP)
- Other CNs intact
Motor Examination:
- 5/5 strength throughout
Sensory Examination:
- Intact
Reflexes:
- 2+ symmetric
Coordination and Gait:
- Normal
Diagnostic Criteria Applied
Modified Dandy Criteria for IIH:
- Signs/symptoms of increased ICP (YES - headache, papilledema, pulsatile tinnitus, visual obscurations, VI palsy)
- No localizing neurological signs except VI palsy (YES)
- CSF opening pressure ≥25 cm H2O (YES - see below)
- Normal CSF composition (YES)
- No other identifiable cause of intracranial hypertension (YES - imaging negative)
Neuro Workup
- MRI Brain with and without contrast: Normal brain parenchyma; findings of IIH: flattening of posterior sclera, empty sella, distension of optic nerve sheath, stenosis of transverse venous sinuses
- MRV: Patent venous sinuses, no thrombosis
- Lumbar puncture (lateral decubitus):
- Opening pressure: 32 cm H2O (elevated; normal <25)
- CSF clear, colorless
- WBC 1, protein 22, glucose 65 (normal composition)
- Patient reports headache improvement after LP
- Formal perimetry (Humphrey visual field): Enlarged blind spots, inferior nasal field defects
Diagnosis
Idiopathic Intracranial Hypertension (IIH) / Pseudotumor cerebri
Management
Acute Management:
- Large-volume LP (therapeutic) performed - 20 mL removed
Medical Treatment:
- Acetazolamide 500 mg BID, titrate to 1-2 g/day (max 4 g/day)
- Monitor electrolytes for metabolic acidosis and hypokalemia
- Potassium supplementation as needed
- Topiramate 25-100 mg BID (also promotes weight loss)
Weight Management (most important long-term):
- Target weight loss of 5-10% body weight
- Dietary counseling, low-sodium diet
- Consider bariatric surgery referral if medical weight loss fails
Ophthalmological Follow-up:
- Serial visual field testing every 4-6 weeks initially
- OCT for retinal nerve fiber layer thickness
- Monthly fundoscopic examinations
Interventional Options (if failing medical therapy):
- Serial therapeutic LPs (bridge until medications work)
- Optic nerve sheath fenestration (if vision threatened)
- VP or LP shunt (for intractable headache)
- Venous sinus stenting (if significant stenosis)
Medication Adjustments:
- Discontinue oral contraceptives (possible contributor)
Clinical Image
Image Description: Fundoscopic photograph showing bilateral papilledema with blurred optic disc margins, elevated disc, and obscuration of vessels at the disc margin.
Attribution: Image from Wikipedia Commons, by Jonathan Trobe, M.D. Licensed under CC BY 3.0. Source: https://commons.wikimedia.org/wiki/File:Papilledema.jpg