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Key Features

Essentials of Diagnosis

  • Headache, worse on straining

  • Visual obscurations or diplopia may occur

  • Examination reveals papilledema

  • Abducens palsy is commonly present

General Considerations

  • Thrombosis of the transverse venous sinus as a complication of otitis media or chronic mastoiditis is one cause, and sagittal sinus thrombosis may lead to a clinically similar picture

  • Other causes

    • Chronic pulmonary disease

    • Systemic lupus erythematosus

    • Uremia

    • Endocrine disturbances such as hypoparathyroidism, hypothyroidism, or Addison disease

    • Vitamin A toxicity

    • Use of tetracycline or oral contraceptives

  • Cases have also followed withdrawal of corticosteroids after long-term use

  • Idiopathic intracranial hypertension

    • No specific cause can be found

    • Occurs most commonly among overweight women aged 20–44

    • Screening for space-occupying lesion of brain is important

Clinical Findings

Symptoms and Signs

  • Symptoms

    • Headache

    • Diplopia

    • Other visual disturbances due to papilledema and abducens nerve dysfunction

  • Examination reveals

    • Papilledema

    • Some enlargement of the blind spots

    • Patients otherwise look well

Differential Diagnosis

  • Venous sinus thrombosis

  • Dural arteriovenous malformation

  • Space-occupying lesion, eg, brain tumor

  • Meningitis

  • Systemic hypertension

  • Migraine

  • Glaucoma

  • Associated conditions

    • Hypoparathyroidism

    • Addison disease

    • Hypothyroidism

    • Chronic pulmonary disease

    • Systemic lupus erythematosus

    • Uremia

  • Associated drugs

    • Vitamin A

    • Tetracycline

    • Minocycline

    • Oral contraceptives

    • Corticosteroid withdrawal

    • Isotretinoin

    • Danazol

Diagnosis

Laboratory Tests

  • Lumbar puncture confirms intracranial hypertension, but the cerebrospinal fluid is normal

Imaging Studies

  • CT or MRI shows small or normal ventricles and an empty sella turcica

  • MR venography is important in screening for thrombosis of the intracranial venous sinuses

Treatment

Medications

  • Acetazolamide (250–500 mg three times daily orally increasing slowly to a maintenance dose of up to 4000 mg daily, divided two to four times daily) reduces formation of cerebrospinal fluid

  • Topiramate

    • See Table 24–2

    • Has been shown to be effective

    • Has added benefit of causing weight loss

  • Furosemide (20–40 mg/day) may be helpful as adjunct therapy

  • Corticosteroids (eg, prednisone 60–80 mg/day) are sometimes prescribed but side effects and the risk of relapse on withdrawal have discouraged their use

  • Any specific cause of intracranial hypertension requires appropriate treatment

    • Hormone therapy should be initiated if there is an underlying endocrine disturbance

    • Discontinue the use of tetracycline, oral contraceptives, or vitamin A

    • If corticosteroid withdrawal is responsible, the medication should be reintroduced and then tapered more gradually

Table 24–2.Medication treatment for seizures in adults (in alphabetical order within classes).

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