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Pseudotumor Cerebri, Pediatric Perspective

William C Robertson Jr, MD, Professor, Departments of Neurology, Pediatrics, and Family Practice, Clinical Title Series, University of Kentucky
Maria-Carmen B Wilson, MD, Medical Director of Pain Management, Department of Neurology, Tampa General Hospital; Associate Professor, Department of Neurology, Assistant Professor, Department of Pediatrics, University of South Florida College of Medicine; Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital

Updated: Dec 4, 2008

Introduction

Background

Pseudotumor cerebri (PTC), or idiopathic intracranial hypertension, is characterized by signs and symptoms of increased intracranial pressure (ICP) in the absence of a space-occupying lesion. Pseudotumor cerebri should be divided into primary (idiopathic) and secondary forms. 

In primary pseudotumor cerebri, no cause can be found. In the secondary form, no mass or space-occupying lesion exists, but other potential causative factors can be identified such as venous sinus thrombosis, hypo- or hypervitaminosis A, and the use of certain medications. Treatment of ICP in both types is essentially the same. Primary pseudotumor cerebri typically occurs among obese women of childbearing age. Although prevalence among the pediatric population is not known, the condition is not uncommon among the young. In children younger than 6 years, a specific cause for intracranial hypertension can usually be identified (secondary pseudotumor cerebri). Primary or idiopathic cases are usually seen after age 11 years.

Children with pseudotumor cerebri usually complain of headaches and may have vomiting, blurred vision, and horizontal diplopia. The headaches are diffuse, worse at night, and often aggravated by sudden movement. Less common complaints include irritability, transitory visual loss (obscurations), dizziness, and tinnitus.

As in adults, treatment is designed to decrease intracranial pressure and preserve vision. (See eMedicine article Pseudotumor Cerebri.)

Pathophysiology

The precise mechanism of ICP in pseudotumor cerebri is unknown. Potential factors important in the pathogenesis include excessive CSF and extracellular edema, increased venous sinus pressure, and/or defective CSF absorption. Evidence for each has been documented in the literature and likely more than 1 mechanism is responsible. 

The importance of venous sinus pressure is seen in children who develop ICP following thrombosis of 1 or more dural sinuses usually secondary to otitis or mastoiditis. Studies in pseudotumor cerebri have shown elevated sagittal sinus pressure, which could lead to resistance to CSF absorption at the arachnoid villi. Using radioisotope cisternography, affected patients have also been found to have a 3- to 5-fold decrease in CSF absorption.

A number of studies in pseudotumor cerebri have suggested the presence of excessive "brain water" (an edematous brain).

Although controversial, histologic evidence of vasogenic brain edema has been observed in biopsy specimens from a small number of patients. Increased intracranial blood volume has also been shown in several studies and excessive white matter water has been reported on MRI.

Malm et al studied CSF dynamics in 17 patients older than 15 years using a constant pressure infusion method and found reduced conductance to CSF flow; however, it was insufficient to explain the increase in CSF pressure. No significant difference in rate of CSF production was noted between patients with pseudotumor cerebri and controls. They also noted that sagittal sinus pressure was elevated in more than half of their patients, which was attributed to increased brain water content causing compression of venous outflow.1

Using sophisticated MR venography Farb and coworkers found a high incidence of transverse and sigmoid sinus stenoses in patients with primary pseudotumor cerebri compared with controls.2  Other studies have indicated that ICP can cause a collapse of the walls of the transverse sinus, which suggests that venous sinus stenosis is a secondary phenomenon. 

Hormonal influences appear to play some role in the pathogenesis of pseudotumor cerebri. In postpuberal patients, the condition is distinctly more common among females. Obesity is a well-recognized risk factor.

Studies have suggested a relationship with nonspecific infections, minor head injury, withdrawal from cortical steroid therapy, vitamin A, acne treatment, and certain antibiotics (tetracyclines). On rare occasion, severe iron deficiency anemia, endocrinopathies, and CO2 retention have been implicated.

Frequency

United States

Epidemiologic studies in children are not available. The annual incidence among all adults in the United States appears to be 0.9 per 100,000; 3.5 per 100,000 females; and 13-19 per 100,000 obese females.

International

  • 1.7 per 100,000 incidence in Libya
  • 3.5 per 100,000 incidence in adult women

Mortality/Morbidity

  • Mortality rate associated with pseudotumor cerebri is no higher than in the general population.
  • The only major morbidity is visual loss. The incidence of visual loss among children with pseudotumor cerebri is unknown. Among all patients some degree of permanent visual loss occurs in approximately 10% of cases.

Race

Some studies have suggested that urban African Americans may be at increased risk.

Sex

A strong predilection exists for women after puberty.

Age

Pseudotumor cerebri is most common among women of childbearing age. However, it has been reported in early infancy. Typically, a specific cause can be identified among young children.

Clinical

History

Common signs and symptoms of pseudotumor cerebri in the young include headache, vomiting, blurred vision, and diplopia.

  • Headaches are intermittent, diffuse, worse at night, may awaken the child, and often aggravated by sudden movement. (For related information, see Medscape's Headache Resource Center.)
  • Visual disturbances include visual obscurations, blurred vision, double vision, and photophobia. Diplopia is almost always horizontal (side by side) and is secondary to paresis of the sixth cranial nerve. It has been estimated that up to 50% of children with pseudotumor cerebri have sixth cranial nerve dysfunction.
  • Other symptoms of increased intracranial pressure include lethargy, irritability, and vomiting.
  • Nonspecific associated symptoms include neck stiffness, tinnitus, dizziness, clumsiness, and paresthesias.

Physical

  • The neurologic examination is typically normal with the exception of papilledema and weakness of one or both of the abducens nerves.
  • Other cranial nerve palsies have been reported on rare occasion.
  • General medical examination may reveal signs of otitis media or mastoiditis, which raises the possibility of venous sinus thrombosis. The presence of acne vulgaris should prompt an inquiry about the possible use of retinoic acid or tetracyclines. Physical findings of adrenal or thyroid dysfunction may also be present.
  • Papilledema: Funduscopic examination reveals optic disk nerve swelling (papilledema). Diagnosis should not be made in the absence of papilledema unless the patient has optic atrophy. Papilledema is typically bilateral but may be asymmetrical or unilateral. Initially, visual acuity is usually preserved helping one to distinguish acute papilledema from optic neuritis.

Causes

Many conditions are associated with pseudotumor cerebri in children, none of which are convincingly causative, with the exception of medications.

  • The following medications may be associated with pseudotumor cerebri:
    • Retinoic acid
    • Antibiotics - Tetracycline, nitrofurantoin, fluoroquinolones
    • Hormones - Steroid use or withdrawal (even topical use), oral contraceptives, L-thyroxine
    • Vitamin A
    • Lithium
    • Immunizations - In one case report, development of pseudotumor cerebri in a 7-month-old following DTP immunization
  • Refeeding and weight gain in nutritionally deprived children (eg, cystic fibrosis)
  • Endocrine abnormalities: These include adrenal dysfunction and Addison disease, hypothyroidism or hyperthyroidism, hypocalcemia due to vitamin D deficiency or hypoparathyroidism, and panhypopituitarism.

Differential Diagnoses

Arteriovenous Malformations
Hydrocephalus
Aseptic Meningitis
Medulloblastoma
Cavernous Sinus Syndromes
Migraine Headache
Cerebral Aneurysms
Migraine Headache: Pediatric Perspective
Cerebral Venous Thrombosis
Childhood Migraine Variants
Headache: Pediatric Perspective

Other Problems to Be Considered

Abducens (CN VI) nerve palsy

Workup

Laboratory Studies

  • Diagnosis of pseudotumor cerebri is made after exclusion of other causes of increased intracranial pressure such as mass lesions, particularly involving the midline (medulloblastoma), and causes of recurrent or chronic headache such as migraine and hydrocephalus.
  • Laboratory studies should include evaluation for endocrine abnormalities if indicated by history and/or physical examination.

Imaging Studies

  • Brain imaging: MRI of the brain with MR venogram (MRV) is preferred. In children, CT scan of the head should be avoided when possible to minimize radiation exposure. The addition of MRV should enable one to exclude thrombosis of one of the major venous sinuses. Stenosis of the transverse sinus is a common finding in pseudotumor cerebri but is probably the result of increased intracranial pressure. Brain MRI is normal but may show relatively small ventricles.
  • Brain imaging should be obtained prior to performing a lumbar puncture. Careful measurements of opening and closing pressures should be obtained. CSF studies are normal except for an elevated opening pressure.

Other Tests

  • Assessment of the visual system
    • Visual field testing: Perimetry can be used to evaluate response to therapy. Common field defects include enlargement of the blind spot, loss of the inferonasal portion and generalized constriction. Other defects include a variety of scotomas and altitudinal patterns of visual loss.
    • Visual acuity assessment: Most patients will have some measurable visual loss that is often asymptomatic, usually occurs gradually, and improves with therapy. 
  • Serial photographs of the fundus may be taken for follow-up.

Procedures

  • Lumbar puncture
    • Performing lumbar puncture in children can be challenging and difficult; sedation may be required. CSF pressure may be elevated falsely in the crying child. Also, no consensus exists on what constitutes the upper limit of normal for different age groups. In their review, Soler et al3 gave the following values:
      • 0-2 years - 75 mm H2 O
      • 2-5 years - 135 mm H2 O
    • Other sources cite the upper limit of normal in children as 200 mm H2 O.
    • Diurnal variations in CSF pressure are seen; therefore, the pressure measured at any given time may not reflect the peaks. CSF pressure may be normal in patients with florid papilledema. If the diagnosis of pseudotumor cerebri is suspected, then repeat lumbar puncture or prolonged pressure monitoring (ie, Camino catheter or lumbar pressure catheter) should be considered.
    • The diagnosis of pseudotumor cerebri requires that the CSF be of normal composition (ie, cell count, protein, glucose).

Treatment

Medical Care

Sometimes, the symptoms of pseudotumor cerebri resolve with the initial diagnostic lumbar puncture. If this occurs, no further medical treatment is required. Acetazolamide and steroids are the mainstays of the medical treatment of pseudotumor cerebri.

  • Acetazolamide is administered in initial doses of 25 mg/kg/day and the dose titrated upward until clinical response is attained (maximum dose 100 mg/kg/day). Electrolytes must be monitored to evaluate for the development of hypokalemia and acidosis. If the patient remains on treatment for more than 6 months, renal ultrasound should be ordered to look for the presence of renal calculi.
  • If acetazolamide is ineffective then prednisone can be given at a dose of 2 mg/kg/day for 2 weeks followed by a 2-week taper.
  • A low-salt diet and weight reduction has been shown to be helpful in adult patients. If the child is obese, weight reduction may be beneficial.
  • Topiramate is now being widely used in the treatment of migraine and pseudotumor cerebri in adults. Topiramate functions as a carbonic anhydrase (CA) inhibitor and appears to be efficacious in the treatment of both conditions. This medication may prove to be useful in selected children with pseudotumor cerebri.
  • Repeat lumbar puncture may help in some patients but its invasiveness and difficulty in children make it a less than ideal medical therapy. Reduction in pressures is often only transient.

Surgical Care

Indications for surgical intervention in the treatment of pseudotumor cerebri are deterioration in vision and incapacitating headaches despite aggressive medical management. Two surgical procedures, lumboperitoneal shunting (LPS) and optic nerve sheath fenestration (ONSF), have a place in the treatment of pseudotumor cerebri.

  • Lumboperitoneal shunting
    • LPS may relieve headache and reduce ICP in patients with pseudotumor cerebri.
    • The long-term visual outcome of patients treated with LPS is unknown.
    • Complications of LPS include infection and shunt obstruction. Low-pressure headaches have also been reported to develop as a result of LPS.
  • Optic nerve sheath fenestration
    • ONSF has been shown to improve visual outcome.
    • ONSF has a better outcome in the patient with acutely decompensating vision and papilledema. The authors prefer optic nerve fenestration to a lumboperitoneal shunting.

Consultations

  • Neuro-ophthalmology
  • Pediatric neurology

Diet

Low-salt diet and weight loss may be beneficial.The authors' experience suggests that weight loss is difficult to achieve in the overweight adolescent.

Medication

Medications used in the treatment of pseudotumor cerebri include acetazolamide and steroids.

Carbonic anhydrase inhibitors

CA is an enzyme found in many tissues of the body, including the eye. These agents catalyze a reversible reaction in which carbon dioxide becomes hydrated and CA dehydrated.


Acetazolamide (Diamox, Diamox sequels)

First-line drug for treatment of pseudotumor cerebri.

Dosing

Adult

500-4000 mg/d PO bid/tid

Pediatric

25-100 mg/kg/d PO; not to exceed 2 g/d

Interactions

Can decrease therapeutic levels of lithium and alter excretion of drugs (eg, amphetamines, quinidine, phenobarbital, salicylates) by alkalinizing urine

Contraindications

Documented hypersensitivity; hepatic disease; severe renal disease; adrenocortical insufficiency; severe pulmonary obstruction; preexisting hypokalemia or hyponatremia; chronic noncongestive angle-closure glaucoma

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Patients with impaired hepatic function may go into coma; may cause substantial increase in blood glucose in some diabetic patients

Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.


Prednisone (Deltasone, Sterapred, Orasone)

If acetazolamide fails to relieve symptoms of pseudotumor cerebri, then steroids may be tried. Experience with this medication in treatment of pediatric Pseudotumor cerebri has shown that short-term use (1 month) is safe and effective.

Dosing

Adult

60-100 mg/d PO

Pediatric

2 mg/kg/d PO for 2 wk; follow by 2-wk taper

Interactions

Estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Contraindications

Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur

Follow-up

Further Outpatient Care

Neuro-ophthalmology follow-up with frequent assessment of visual fields is indicated.

Inpatient & Outpatient Medications

See Medication section.

Complications

As mentioned above, the most serious complication is permanent visual loss and blindness.

Prognosis

  • The natural history of pseudotumor cerebri in childhood is poorly understood.
  • Some children respond to initial lumbar puncture alone.
  • Visual loss can occur at any point in the disease and no reliable predictive factors are associated with this complication.

Patient Education

Patient and parental education as to the seriousness of permanent visual loss should be given. Early intervention in rapidly declining visual function is crucial to improve the long-term visual outcome.

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Keywords

benign intracranial hypertension, hypertensive meningeal hydrops, idiopathic intracranial hypertension, otitic hydrocephalus, serous meningitis, toxic hydrocephalus, PTC, pseudotumor cerebri

Contributor Information and Disclosures

Author

William C Robertson Jr, MD, Professor, Departments of Neurology, Pediatrics, and Family Practice, Clinical Title Series, University of Kentucky
William C Robertson Jr, MD is a member of the following medical societies: American Academy of Neurology and Child Neurology Society
Disclosure: Nothing to disclose.

Coauthor(s)

Maria-Carmen B Wilson, MD, Medical Director of Pain Management, Department of Neurology, Tampa General Hospital; Associate Professor, Department of Neurology, Assistant Professor, Department of Pediatrics, University of South Florida College of Medicine
Maria-Carmen B Wilson, MD is a member of the following medical societies: American Academy of Neurology, American Pain Society, American Society of Neuroimaging, Florida Medical Association, and International Association for the Study of Pain
Disclosure: Nothing to disclose.

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Medical Editor

Raj D Sheth, MD, Professor of Neurology, Mayo College of Medicine; Chief, Division of Pediatric Neurology, Nemours Children's Clinic
Raj D Sheth, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Neurological Association, and Child Neurology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Pediatrics, Division of Pediatric Neurology, Department of Neurology, Oregon Health and Science University; Consulting Staff, Shriners Hospital for Children
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Further Reading

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