eMedicine Specialties > Neurology > Pediatric Neurology

Pseudotumor Cerebri, Pediatric Perspective: Treatment & Medication

Author: William C Robertson Jr, MD, Professor, Departments of Neurology, Pediatrics, and Family Practice, Clinical Title Series, University of Kentucky
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; Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Contributor Information and Disclosures

Updated: Dec 4, 2008

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.

Adult

500-4000 mg/d PO bid/tid

Pediatric

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

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

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

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.

Adult

60-100 mg/d PO

Pediatric

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

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

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

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

More on Pseudotumor Cerebri, Pediatric Perspective

Overview: Pseudotumor Cerebri, Pediatric Perspective
Differential Diagnoses & Workup: Pseudotumor Cerebri, Pediatric Perspective
Treatment & Medication: Pseudotumor Cerebri, Pediatric Perspective
Follow-up: Pseudotumor Cerebri, Pediatric Perspective
References
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Further Reading

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Keywords

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

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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.

 
 
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