Pseudotumor Cerebri Idiopathic Intracranial Hypertension

Pseudotumor Cerebri Idiopathic Intracranial Hypertension: Excerpt from The 5-Minute Pediatric Consult

Sabrina E. Smith, MD, PhDDennis J. Dlugos, MD

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - BASICS

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - description

Diagnostic criteria of idiopathic intracranial hypertension (IIH) include:

• Signs and symptoms of increased intracranial pressure (e.g., headache, vomiting, ocular manifestations, and papilledema)
• Elevated cerebrocranial fluid pressure but otherwise normal CSF
• Normal neurologic examination except for papilledema (occasional abducens or other motor cranial neuropathy)
• Normal neuroimaging study (or incidental findings only)

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - epidemiology

• Boys and girls are affected equally in childhood; in adulthood, more women than men are affected.
• IIH has been reported in patients as young as 4 months of age, with a median age of 9 years.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - incidence

Incidence in children is unknown.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - risk-factors

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - genetics

Sporadic, no clear genetic predisposition, unless related to an underlying hormonal, toxic, or inflammatory condition; no data are available in children.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - pathophysiology

Pathogenesis may involve decreased CSF absorption owing to arachnoid villi dysfunction or elevated intracranial venous pressure. For example, obesity may lead to increased intra-abdominal, intrathoracic, and cardiac filling pressure, eventually leading to elevated intracranial venous pressure.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - etiology

• Numerous precipitants of pseudotumor have been reported. In adolescents, it is clearly associated with obesity and weight gain, but not clearly linked to obesity in children younger than 11 years. Many weaker associations may be due to chance.
• Pseudotumor is often linked to minocycline, tetracycline, sulfonamides, isotretinoin, and thyroid replacements, and to corticosteroid withdrawal. It is also linked to vitamin A deficiency or intoxication, chronic anemia, and hypothyroidism.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - associated-conditions

• Visual loss owing to optic nerve pressure
• Endocrinopathies, exogenous steroids, lead exposure, and therapy involving tetracycline and several other antibiotics may be associated with pseudotumor.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - DIAGNOSIS

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - signs & symptoms

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - history

• Headache
• Blurred vision
• Transient visual darkening
• Stiff neck
• Pulsatile tinnitus
• Dizziness
• Infants and young children may present with irritability, somnolence, or ataxia.
• IIH should be considered in any child with chronic headache or unexplained visual changes.
• Directed history for signs of associated endocrinopathy, exposure to antibiotics or steroids, sinus infection, abnormal clotting, or familial tendency to thrombosis or visual disturbance

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - physical exam

• Examination of the fundi is essential.
• Recording baseline visual acuity and visual fields in older children is essential.
• Papilledema is almost always present in older children with IIH.
• Most infants have some degree of papilledema, even with open fontanelles and split sutures.
• 6th cranial nerve (abducens) palsies are common in children with IIH; they were found in 29 of 68 patients in 1 series.
• Other cranial nerve deficits are rarely found.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - tests

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - lab

• CSF cell count, glucose, and protein are essential and should be normal in IIH.
• CBC and thyroid function tests should be obtained because anemia, hypothyroidism, and hyperthyroidism have rarely been associated with pseudotumor.
• The following may be useful in selected cases:
  • ANA test
  • ESR
  • Urine cortisol
  • Serum lead level
  • Serologic testing for Lyme disease

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - imaging

Cranial CT or MRI should be normal. MRI is recommended because of superior imaging of brainstem, posterior fossa, and venous sinuses. Magnetic resonance venography is strongly suggested to evaluate for venous sinus thrombosis, which can be difficult to distinguish from IIH.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - diag-proced-surgery

• Lumbar puncture manometry, with the patient in a relaxed lateral decubitus position, should show an opening pressure >250 mm H
• Goldmann perimeter visual field testing or computerized visual fields are useful in children older than 5 years to document field deficits and monitor response to therapy.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - differencial diagnosis

Some conditions may be confused with IIH, but the clinical picture and CSF analysis usually permit their distinction.

• Chronic meningitis (e.g., CNS, Lyme disease), encephalitis, or cerebral edema (may show minimal changes on neuroimaging with elevated CSF protein levels and little evidence of pleocytosis)
• Cerebral venous sinus thrombosis
• Pseudopapilledema (optic nerve disc drusen)

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - TREATMENT

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - initial-stabilization

• The urgency of diagnosis and treatment depends on the severity of visual loss. Recent reports suggest that severe visual loss may progress rapidly, warranting close initial (weekly) tracking of vision and prompt consideration of surgical treatment (see below).
• For patients with no visual loss, removal of possible causative agents may be the only intervention needed, along with treatment of associated conditions (e.g., obesity, anemia, thyroid disease). Consider treatment with acetazolamide (Diamox; see later comment). Headache can be treated symptomatically if needed.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - medication

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - first-line

• For patients with mild-to-moderate visual loss, acetazolamide, a carbonic anhydrase inhibitor that decreases CSF production, is the drug of choice:
  • The pediatric dosage is 60 mg/kg/d divided q.i.d. for the standard form and b.i.d. for the long-acting form (Diamox sequels).
  • The initial adult dose is 250 mg q.i.d. or 500 mg b.i.d., increased to 750 mg q.i.d. or 1,500 mg b.i.d. if tolerated.
• If visual loss, papilledema, and symptoms of pressure resolve, acetazolamide dosage can be tapered after 2 months of therapy.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - second-line

Furosemide can be used if acetazolamide is ineffective or has intolerable adverse effects.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - surgery

• Serial lumbar punctures are not recommended as standard therapy, although the initial puncture can be useful to relieve symptoms quickly.
• Surgical therapy (e.g., optic nerve sheath fenestration, lumboperitoneal shunt) is indicated for progressive visual loss despite medical therapy and may also be considered as an urgent intervention at presentation depending on degree of visual loss. Optic nerve sheath fenestration may be the preferred surgical treatment, especially in children, because of the high failure rates of lumboperitoneal shunting. High-dose IV steroids and acetazolamide therapy may be used while awaiting surgical therapy.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - FOLLOW UP

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - disposition

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - issues for referral

Follow-up and tapering of acetazolamide should be done in conjunction with a neurologist or neuro-ophthalmologist.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - patient-monitoring

• Initially, patients should have visual acuity, visual fields, and fundi evaluated weekly or biweekly.
• If vision is stable, monthly visits may be adequate for 3–6 months.
• More frequent follow-up is required for any signs of progressive visual loss.

Pitfalls:

• Children are not exempt from permanent visual loss as a consequence of IIH.
• Ophthalmologic follow-up is important.
• Occasional patients, especially adolescents, may experience headache weeks or months after resolution of objective signs of IIH (i.e., even though intracranial pressure has returned to normal).
• IIH may be diagnosed erroneously if:
  • Pseudopapilledema is mistaken for papilledema. (Pseudopapilledema is apparent optic disc swelling that simulates papilledema, but is usually secondary to an underlying benign process. It can be differentiated by an experienced ophthalmologist or neurologist.)
  • CSF abnormalities (i.e., isolated increase in protein) are overlooked.
  • Neuroimaging study fails to identify a cerebral venous sinus thrombosis.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - bibliography

1. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. 2002;59:1492–1495.
2. Jones JS, Nevai J, Freeman MP, et al. Emergency department presentation of idiopathic intracranial hypertension. Am J Emerg Med. 1999;17:517–521.
3. Lim M, Kurian M, Penn A, et al. Visual failure without headache in idiopathic intracranial hypertension. Arch Dis Child. 2005;90:206–210.

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - CODES

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - icd9

348.2 Benign intracranial hypertension pseudotumor cerebri

Pseudotumor Cerebri Idiopathic Intracranial Hypertension - FAQ

• Q: What are the side effects of acetazolamide?
• A: Side effects of acetazolamide include: GI upset, paresthesias, loss of appetite, drowsiness, metabolic acidosis, and renal stones. An alternative is furosemide.
• Q: If IIH occurs on tetracycline, can the patient take penicillin?
• A: Penicillins/cephalosporins have not been reported as a significant cause of IIH.
• Q: Are there any limitations on physical activity?
• A: Activity can be graded entirely according to the child’s symptoms.

Book Source Details

• Book Title: The 5-Minute Pediatric Consult
• Author(s): M. William Schwartz MD; et al.
• Year of Publication: 2008
• Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

More About Pseudotumor Cerebri

• Back to disease: Pseudotumor Cerebri: Introduction
• Next Book Extract About Pseudotumor Cerebri: Surveys relating to Pseudotumor Cerebri
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

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