Child Neurology Net

Pseudotumor Cerebri

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Definition

Pseudotumor cerebri is a syndrome of cerebral hypertension, increased intracranial pressure, normal cerebral spinal fluid, normal brain with normal or small ventricles on imaging.

Etiology/ Pathogenesis

The pathogenesis is unknown. The condition may be idiopathic, or related to certain drugs (for example tetracycline, and oral contraceptives), systemic conditions (such as Guillain-Barre, Polycythemia Vera), head trauma, infections (otitis media) or metabolic abnormalities (diabetic ketoacidosis, and adrenal insufficiency, for example).
Elevated venous sinus pressure is seen in most patients, which blocks drainage of the CSF into the arachnoid granulation.

Age of Onset

<6 years if a specific cause, usually, >11 if idiopathic

Classic Presentation

Male:female equally affected.

The presentation of pseudotumor cerebri is highly varied.
In infants, clinical signs of increased intracranial pressure are often present. These include bulging fontanel, macrocephaly, and the "sunset sign" (an inability to look up because of pressure on the dorsal midbrain).
Young children may have only nonspecific symptoms such as irritability, somnolence and apathy. Symptoms that are seen more often are related to elevated intracranial pressure and the resulting papilledema. Headaches are nonspecific, fluctuating in type, frequency and location. Vomiting, diplopia, pulsatile tinnitus, visual disturbances and progressive loss of peripheral and color vision.
In teenagers, headaches are common, but the VIth nerve is often involved. The headaches are usually daily and increase with coughing, bending over, and lying down, all things that increase the intracranial pressure.
Note that papilledema is not present in every case!

Rarer Symptoms

Neck stiffness, paresthesias, ataxia, radicular pain in the arms
Visual changes (such as blurring and transient obsscurations of vision), tinnitus, and vertigo.

Usual Course

If not treated, some children have progressive papilledema and optic atrophy due to compression. This, leads to progressive enlargment of the blind spot, decreased acuity, or generalized constriction of fields. Loss of vision may be rapid and severe.

Genetics

Groups at high risk

Teenage girls seem more at risk than boys, but boys and girls are equally affected at younger ages. Obesity is a risk factor. Use of certain medications (e.g., corticosteroid use or withdrawal, tetracyclines, oral contraceptives, vitamin A), certain systemic disease and conditions(e.g., pregnancy or menarche, Guillain-Barre, polycythemia, leukemia, iron-deficiency anemia, Lyme disease), and some metabolic disorders (e.g., hyperthyroidism or initiation of treatment of hypothyroidism, hypoparathyroidism, adrenal insufficiency or hyperaldrenalism) all are risk factors.

Diagnostic Testing

Diagnosis of exclusion

Need imaging study, usually normal

Lumbar puncture with opening pressure after a negative imaging study. MRI with MR venogram is recommended. A good ophthalmologic examination, including fundal photography and formal field testing is recommended.

Differential

Arteriovenous Malformations

Aseptic Meningitis

Hydrocephalus, both communicating and non-communicating.

Intracranial Epidural Abscess

Intracranial Hemorrhage

Lyme Disease

Meningioma

Migraine Headache

Subarachnoid Hemorrhage

Systemic Lupus Erythematosus

Abducens (CN VI) nerve palsy

Tumors and other masses

Venous sinus thrombosis (although some do not separate these two disorders).

Myasthenia (this can lead to diplopia)

Chronic daily headaches,

Depression.

Treatment

Want to relieve headache and preserve vision.

Therapeutic lumbar puncture may be performed. The goal of puncture is to make closing pressure 1/2 of opening pressure by taking off fluid). It is unknown how the lumbar puncture helps, it is possible that CSF fluid dynamics are changed. The lumbar puncture is repeated if symptoms return.

Acetazolamide, 10 mg/kg/day after lumbar puncture. Acetazolamide decreases CSF production; it is not clear if furosemide adds any additional benefit.

Eliminate underlying precipitant if possible. Weight loss if applicable.

Paradoxically, a brief course of steroids may be of benefit.

Ventriculoperitoneal shunting is preferred to lumboperitoneal shunting despite what some of the textbooks and older articles say; the latter can lead to herniation of the cerebellar tonsils.

Optic nerve sheath fenestration.

Misc

Did you localize this well? The VIth nerve or lateral rectus muscle on the left is involved. For a wonderful web site that teaches about eye movements and their abnormalities, see the Eye Movement Simulator: http://cim.ucdavis.edu/EyeBeta/Interface/eSim.htm

Websites with additional info

Family Village http://www.familyvillage.wisc.edu/lib_pc.html

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Definition	Pseudotumor cerebri is a syndrome of cerebral hypertension, increased intracranial pressure, normal cerebral spinal fluid, normal brain with normal or small ventricles on imaging.
Etiology/ Pathogenesis	The pathogenesis is unknown. The condition may be idiopathic, or related to certain drugs (for example tetracycline, and oral contraceptives), systemic conditions (such as Guillain-Barre, Polycythemia Vera), head trauma, infections (otitis media) or metabolic abnormalities (diabetic ketoacidosis, and adrenal insufficiency, for example). Elevated venous sinus pressure is seen in most patients, which blocks drainage of the CSF into the arachnoid granulation.
Age of Onset	<6 years if a specific cause, usually, >11 if idiopathic
Classic Presentation	Male:female equally affected. The presentation of pseudotumor cerebri is highly varied. In infants, clinical signs of increased intracranial pressure are often present. These include bulging fontanel, macrocephaly, and the "sunset sign" (an inability to look up because of pressure on the dorsal midbrain). Young children may have only nonspecific symptoms such as irritability, somnolence and apathy. Symptoms that are seen more often are related to elevated intracranial pressure and the resulting papilledema. Headaches are nonspecific, fluctuating in type, frequency and location. Vomiting, diplopia, pulsatile tinnitus, visual disturbances and progressive loss of peripheral and color vision. In teenagers, headaches are common, but the VIth nerve is often involved. The headaches are usually daily and increase with coughing, bending over, and lying down, all things that increase the intracranial pressure. Note that papilledema is not present in every case!
Rarer Symptoms	Neck stiffness, paresthesias, ataxia, radicular pain in the arms Visual changes (such as blurring and transient obsscurations of vision), tinnitus, and vertigo.
Usual Course	If not treated, some children have progressive papilledema and optic atrophy due to compression. This, leads to progressive enlargment of the blind spot, decreased acuity, or generalized constriction of fields. Loss of vision may be rapid and severe.
Genetics
Groups at high risk	Teenage girls seem more at risk than boys, but boys and girls are equally affected at younger ages. Obesity is a risk factor. Use of certain medications (e.g., corticosteroid use or withdrawal, tetracyclines, oral contraceptives, vitamin A), certain systemic disease and conditions(e.g., pregnancy or menarche, Guillain-Barre, polycythemia, leukemia, iron-deficiency anemia, Lyme disease), and some metabolic disorders (e.g., hyperthyroidism or initiation of treatment of hypothyroidism, hypoparathyroidism, adrenal insufficiency or hyperaldrenalism) all are risk factors.
Diagnostic Testing	Diagnosis of exclusion Need imaging study, usually normal Lumbar puncture with opening pressure after a negative imaging study. MRI with MR venogram is recommended. A good ophthalmologic examination, including fundal photography and formal field testing is recommended.
Differential	Arteriovenous Malformations Aseptic Meningitis Hydrocephalus, both communicating and non-communicating. Intracranial Epidural Abscess Intracranial Hemorrhage Lyme Disease Meningioma Migraine Headache Subarachnoid Hemorrhage Systemic Lupus Erythematosus Abducens (CN VI) nerve palsy Tumors and other masses Venous sinus thrombosis (although some do not separate these two disorders). Myasthenia (this can lead to diplopia) Chronic daily headaches, Depression.
Treatment	Want to relieve headache and preserve vision. Therapeutic lumbar puncture may be performed. The goal of puncture is to make closing pressure 1/2 of opening pressure by taking off fluid). It is unknown how the lumbar puncture helps, it is possible that CSF fluid dynamics are changed. The lumbar puncture is repeated if symptoms return. Acetazolamide, 10 mg/kg/day after lumbar puncture. Acetazolamide decreases CSF production; it is not clear if furosemide adds any additional benefit. Eliminate underlying precipitant if possible. Weight loss if applicable. Paradoxically, a brief course of steroids may be of benefit. Ventriculoperitoneal shunting is preferred to lumboperitoneal shunting despite what some of the textbooks and older articles say; the latter can lead to herniation of the cerebellar tonsils. Optic nerve sheath fenestration.
Misc	Did you localize this well? The VIth nerve or lateral rectus muscle on the left is involved. For a wonderful web site that teaches about eye movements and their abnormalities, see the Eye Movement Simulator: http://cim.ucdavis.edu/EyeBeta/Interface/eSim.htm
Websites with additional info	Family Village http://www.familyvillage.wisc.edu/lib_pc.html