Idiopathic Intracranial

Hypertension
By Becky Dru – FY1 Stroke Team
Patient C
 18 years old, no PMHx
 6 months intermittent headache/blurred vision
 Presented to optometrist 3/8/18
 Bilateral papilloedema on slit lamp examination (worse on left)
 Intraocular pressure 18mmHg bilaterally (N)
 Differential diagnosis pre-admission?
 Space occupying lesion, cerebral venous thrombosis,
hypervitaminosis A, meningitis… IIH?
Patient C comes to YDH
 Admission to hospital 3/8/18
 MRI/MR venogram: no space occupying lesion, normal CSF/venous
structures
 Lumbar puncture: opening pressure 31.7cmH2O, closing pressure
20 cmH2O
 (Differential) diagnosis?
 Idiopathic intracranial hypertension
Management for Patient C
 LP was therapeutic
 Role for acetazolamide?
 Advice from Neurologist at Musgrove Park:
 Formal visual field testing – if normal, not for acetazolamide
 Routine Neurology outpatient clinic appointment
 Advised if Sx return, to call Musgrove Park Neurology dept
 Weight loss
 Idiopathic Intracranial Hypertension
 Definition:

 “clinical syndrome of raised intracranial pressure, in the absence of

space-occupying lesions or vascular lesions, without enlargement of
the cerebral ventricles, for which no causative factor can be
identified”

 ‘benign intracranial hypertension’ (1955)

 Symptoms
 Headache  Pain
 Visual disturbance  Nausea +/- vomiting
 Tinnitus  Photophobia

Ball, A. and Clarke, C. (2006). Idiopathic intracranial hypertension. The Lancet

Neurology, [online] 5(5), pp.433-442.
Epidemiology
 In USA:
 1-2/100,000
 Similar incidence to Guillain-Barre syndrome, pituitary tumours, cluster headache
 If confined to women 20-44, >20% above ideal BW:
 15-19/100,000
 Similar to MND, multiple sclerosis
 Most common:
 Women
 Obese
 20-44 years
 No proven associations with other disease
Associations
 Association with increased retinol (fat-soluble
vitamin A) in CSF not serum
 Medications
 Diseases
 ?HTN
 ?PCOS
 Pregnancy – disproved
 Menstrual dysfunction
Pathogenesis - Hypotheses
 Excess CSF production
 N: 500ml per 24hrs
 Disproved
 Brain water content
 Still disputed
 Reduced CSF absorption
 Most popular
 Little known about mechanism
 Increased cerebral venous pressure
 Functional outflow obstruction (narrowing) in absence of occlusion
 Endocrine dysfunction
 Women of childbearing age  hormonal involvement? Unproven.
 Obesity  leptin involvement? Unproven.
Investigations
 Diagnosis of exclusion
 MRI and MRV – exclude cerebral
venous thrombosis, space-
occupying lesion
 Lumbar puncture – opening
pressure measurement
(>20cmH2O), biochemistry
analysis (normal)
 Management
 Cochrane Review 2015: 2 RCTs
 Acetazolamide vs placebo
 Weight loss intervention in both groups
 Modest benefit for acetazolamide, but insufficient evidence
 Acetazolamide
 Analgesics
 Others: corticosteroids
 Weight reduction – dietary modification, surgical intervention
 Surgical

Piper, Rory J, et al. “Interventions for Idiopathic Intracranial Hypertension.” Cochrane Systematic Reviews,
Cochrane Library, 7 Aug. 2015
Monitoring
 No consensus
 Regular LPs not done
 Assessment of Sx, regular fundoscopy & visual acuity assessment,
formal visual field documentation
Prognosis
 Most people = benign
 Blindness in at least one eye 8-10%
 Sustained visual loss almost half of patients
 No predictors of severity, recurrence
 Can recur months – years after initial occurrence resolved
Overview

Patient: Presentation: Investigations:

- Obese - Headache - MRI/MRV
- Female - Visual disturbance - LP
- 20-44yrs - Papilloedema

No other reason found Treatment: Prognosis:

for raised ICP  - ?acetazolamide - 10% blindness one eye
Diagnosis of IIH - Weight loss - no predictors of
- Regular monitoring severity/recurrence
- Vigilance of Sx
Thank you
Any questions?