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PSEUDOTUMOR CEREBRI/
INTRACRANIAL HYPERTENSION

Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), is a neurological condition characterized by increased intracranial pressure (ICP) in the absence of any identifiable cause, such as a brain tumor or structural abnormality. The term "pseudotumor" refers to the fact that the condition mimics the symptoms of a brain tumor (e.g., headaches, visual disturbances, and increased ICP) but without an actual tumor being present.

The condition primarily affects young women of childbearing age, especially those who are overweight or obese, though it can also occur in other populations, including men and children.

Key Features

  1. Increased Intracranial Pressure (ICP):

    • Patients with pseudotumor cerebri have elevated intracranial pressure, which can cause brain swelling, leading to the clinical symptoms of the condition.

    • The pressure is typically not caused by a mass, like a tumor, but instead is thought to be related to an imbalance in the production and absorption of cerebrospinal fluid (CSF), or increased resistance to CSF drainage.

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  2. No Structural Abnormality:

    • Unlike other causes of increased ICP (e.g., brain tumors, hydrocephalus, or hemorrhage), pseudotumor cerebri does not involve any structural abnormalities of the brain.

    • Brain imaging (CT or MRI) is usually normal, ruling out other causes of increased pressure.

SYMPTOMS

The symptoms of pseudotumor cerebri can vary in severity but typically include:

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  1. Headaches:

    • The most common symptom, typically described as throbbing, persistent, and worse in the morning. The headache may be associated with nausea and vomiting, which can often be worsened by coughing, sneezing, or straining.

  2. Papilledema:

    • Swelling of the optic disc (the head of the optic nerve), which can be seen during a fundoscopic (eye) exam. Papilledema is a hallmark of increased ICP and can lead to visual disturbances such as blurred vision, double vision, or transient loss of vision.

    • Optic nerve atrophy can develop if the condition is not treated promptly, potentially leading to permanent vision loss.

  3. Visual Symptoms:

    • Blurred vision, temporary vision loss, or blind spots are common due to the elevated pressure affecting the optic nerves.

    • In more severe cases, permanent vision impairment can result from prolonged pressure on the optic nerve.

  4. Tinnitus (Ringing in the Ears):

    • A high-pitched ringing sound in the ears may occur, which is related to increased pressure on the structures within the brain.

  5. Neck and Shoulder Pain:

    • Some patients report neck or shoulder discomfort, possibly related to the increased pressure and muscle tension associated with the condition.

  6. Nausea and Vomiting:

    • Due to the elevated ICP, nausea and vomiting, particularly in the morning, are common.

CAUSES

There are two types of intracranial hypertension

  • Primary / Idiopathic (IIH): Raised pressure with no detectable structural causeIdiopathic Intracranial Hypertension

  • Secondary IH: Due to an identifiable cause that increases intracranial volume or impairs drainageTumor, venous thrombosis, trauma, etc.

 

Secondary Causes 

 

Increased Intracranial Volume

  • Brain tumors or masses (e.g., glioma, abscess, hematoma)

  • Hydrocephalus (excess CSF due to obstruction or overproduction)

  • Cerebral edema (swelling after trauma, stroke, infection, or hypoxia)

  • Intracerebral hemorrhage (bleeding inside the brain)

Mechanism: Added volume or swelling → increases total pressure in the rigid skull.

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Impaired CSF Absorption or Flow

  • Venous sinus thrombosis (clot in major brain veins)

  • Subarachnoid hemorrhage or meningitis (scarring blocks CSF reabsorption)

  • Chiari malformation or CCI (distorts CSF flow at skull base)

  • Spinal CSF leak (low pressure that paradoxically triggers compensatory venous engorgement)

 Mechanism: CSF can’t drain properly → accumulation → raised intracranial pressure

DIAGNOSIS

The diagnosis of pseudotumor cerebri is made based on a combination of clinical signs, symptoms, and exclusion of other causes of increased intracranial pressure. Key diagnostic steps include:

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  1. Neurological Examination:

    • A thorough neurological exam may reveal papilledema, visual field defects, and other signs of increased intracranial pressure.

  2. Brain Imaging (CT or MRI):

    • Brain imaging is typically normal in pseudotumor cerebri, ruling out brain tumors, structural abnormalities, or other causes of elevated ICP.

    • In some cases, imaging may reveal empty sella syndrome, a condition where the pituitary gland appears flattened, but this is not specific to pseudotumor cerebri.

  3. Lumbar Puncture (Spinal Tap):

    • The most definitive test for diagnosing pseudotumor cerebri is measuring the opening pressure during a lumbar puncture (spinal tap). Elevated opening pressure (typically > 20-25 cm Hâ‚‚O) confirms increased intracranial pressure.

    • The cerebrospinal fluid (CSF) analysis is typically normal, which helps differentiate pseudotumor cerebri from other causes of increased ICP like infection or hemorrhage.

  4. Visual Field Testing:

    • Visual field testing (e.g., perimetry) is done to assess for any visual impairments related to papilledema. This can help determine the extent of optic nerve involvement.

  5. Magnetic Resonance Venography (MRV):
     

    • This imaging test is used to rule out venous sinus thrombosis, a condition where blood clots form in the veins of the brain, which can also cause increased ICP.

TREATMENT

Treatment of pseudotumor cerebri aims to reduce intracranial pressure, relieve symptoms, and prevent complications like vision loss. The management plan often includes:

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  1. Weight Loss: For overweight or obese patients, weight loss is one of the most effective treatments for reducing intracranial pressure. Even modest weight loss can lead to significant improvements in symptoms.

  2. Medications:

    • Acetazolamide (Diamox): A carbonic anhydrase inhibitor that reduces CSF production and helps lower ICP. This is the first-line medication for managing pseudotumor cerebri.

    • Topiramate: A medication sometimes used in combination with acetazolamide to treat the condition, as it may also help with weight loss and reduce symptoms.

    • Furosemide: A diuretic may be used in conjunction with acetazolamide to help reduce fluid buildup.

    • Corticosteroids: In some cases, short courses of steroids may be used to control severe symptoms or acute episodes of increased ICP.

  3. Optic Nerve Sheath Fenestration: A surgical procedure in which the optic nerve sheath is opened to relieve pressure on the optic nerve. This may be considered if there is significant visual impairment or risk of vision loss.

  4. Shunt: In cases of severe or refractory pseudotumor cerebri, a shunt may be implanted to drain excess cerebrospinal fluid (CSF) and lower ICP. This is more commonly used in patients who fail medical management.

  5. Regular Monitoring: Regular follow-up visits with ophthalmology are crucial to monitor the progression of papilledema and visual field defects.

  6. Periodic lumbar punctures may be performed to measure ICP and assess the effectiveness of treatment.

PROGNOSIS

The prognosis for pseudotumor cerebri varies based on the severity of symptoms and how quickly the condition is diagnosed and treated. With early intervention and appropriate treatment, the long-term outlook is generally good, especially with weight loss and medical management. However, if left untreated, the condition can lead to permanent vision loss due to optic nerve damage from increased pressure. Therefore, timely management is critical to prevent complications.

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