IDIOPATHIC INTRACRANIAL
HYPERTENSION
Introduction
• Idiopathic intracranial hypertension (IIH), also called pseudotumor cerebri, is a
• Clinical criteria that include symptoms and signs isolated to those produced by increased
intracranial pressure (headache, papilledema, vision loss)
• Elevated ICP with normal cerebrospinal fluid (CSF) composition
• No other cause of intracranial hypertension evident on neuroimaging or other evaluations
• Usually occurs in women in childbearing years(15-45years) who are 20% or more than ideal
body weight.
• Annual incidence 1-2/million.
primarily affects females of childbearing age who are overweight
also reported in children as young as four months and in older patients (up to 88 years)
in children younger than 17 years old, There is no female preponderance in young patients, prepubertal children with IIH
are not more likely to be obese than the general age-matched population
older patients (greater than 44 years), more often male and less often obese than patients with IIH in the more typical age
range
Recent weight gain may be a risk factor for IIH,
Pathogenesis
precise pathogenesis of IIH remains unknown.
Proposed etiologies include
Cerebral venous outflow abnormalities (eg, venous stenoses and venous hypertension)
Increased cerebrospinal fluid (CSF) outflow resistance at the level of either the arachnoid granulations or
CSF lymphatic drainage sites
Obesity-related increased abdominal and intracranial venous pressure
Altered sodium and water retention mechanisms
Abnormalities of vitamin A metabolism
CLINICAL PRESENTATION
Typical presentation of IIH is that of an overweight female of childbearing age who complains of headaches and is found to
have papilledema on funduscopic examination.
Symptoms
Headache (84 to 92 percent)
Transient visual obscurations (68 to 72 percent)
Intracranial noises (pulsatile tinnitus; 52 to 60 percent)
Photopsia (48 to 54 percent)
Back pain (53 percent)
Retrobulbar pain (44 percent)
Diplopia (18 to 38 percent), typically from sixth nerve palsy
Sustained visual loss (26 to 32 percent)
Neck pain (41 percent)
• Signs
1. Papilledema
2. Visual field loss
3. Sixth nerve palsy
Diagnosis : modified dandy criteria
Symptoms and signs of increased intracranial pressure (eg, headache, transient visual obscurations,
pulse synchronous tinnitus, papilledema, visual loss)
No other neurologic abnormalities or impaired level of consciousness
Elevated intracranial pressure with normal cerebrospinal fluid (CSF) composition
A neuroimaging study that shows no etiology for intracranial hypertension
Evaluation
Magnetic resonance imaging
primary purpose of neuroimaging is to exclude secondary causes of increased intracranial pressure.
brain parenchyma and ventricles appear normal on MRI or CT
MRI abnormalities may be seen and suggest IIH are,
Flattening of the posterior sclera (43 to 80 percent); this is the most specific
Distension of perioptic subarachnoid space (45 to 67 percent)
Enhancement (with gadolinium) of the prelaminar optic nerve (7 to 50 percent)
Empty sella (25 to 80 percent)
Intraocular protrusion of the prelaminar optic nerve (3 to 30 percent)
Vertical tortuosity of the orbital optic nerve (40 percent)
Lumbar puncture with csf opening pressure
200 mmH2O to be normal, greater than 250 mmH2O to be abnormal, and 200 to 250 mmH2O to be equivocal
Ophthalmologic evaluation
Visual field examination to assess the severity of optic nerve involvement and monitor response to treatment.
Most common findings were a partial arcuate defect (commonly with a nasal step defect) coupled with an
enlarged blind spot.
Dilated funds examination.
Treatment
Alleviation of symptoms (usually headache),preservation of vision.
INITIAL TREATMENT FOR MOST PATIENTS
Address risk factors and comorbid conditions
Weight loss
Carbonic anhydrase inhibitors
Dosing and administration : Acetazolamide 500 mg twice per day and advance the dose as required up to 2 to 4 grams
per day,
Potential contraindications : sulfa allergy,Pregnancy, particularly the first 20 weeks.
Adverse effects :electrolyte changes, mild metabolic acidosis, and kidney stones.
Despite maximizing treatment with acetazolamide, if persistent symptoms furosemide (20 to 40 mg per day) can be added
Topiramate is an antiseizure medication that inhibits carbonic anhydrase activity.
Other carbonic anhydrase inhibitors, such as methazolamide , can also be used in acetazolamide-intolerant patients.
Rapidly progressive vision loss
Medical therapy - Acetazolamide should be initiated promptly and can be rapidly titrated up to 4 grams per day divided into
two doses.
In addition,
Glucocorticoids - In the setting of acute visual loss, a short course of intravenous glucocorticoids may be useful as a temporizing
measure prior to surgical intervention in fulminant IIH.
Serial lumbar punctures - Serial lumbar punctures or lumbar drainage can be a useful temporizing measure as a prelude to
surgery
INTERVENTIONS FOR SEVERE OR REFRACTORY DISEASE
Indications for intervention : Worsening visual field defect despite medical therapy
Presence of visual acuity loss attributed to papilledema
Surgical Interventions
Optic nerve sheath fenestration (ONSF) : Temporary diplopia,Efferent pupillary dysfunction.
Cerebrospinal fluid (CS) shunting procedures : Shunt failure,shunt infection, overdrainage causing low pressure.
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