Summary about Disease
Pseudotumor cerebri, also known as idiopathic intracranial hypertension (IIH), is a condition where there is increased pressure around the brain (intracranial pressure) without any detectable tumor or other apparent cause on imaging. This elevated pressure can mimic the symptoms of a brain tumor, hence the name "pseudotumor cerebri," which translates to "false brain tumor." While not life-threatening in itself, the increased pressure can lead to vision loss if left untreated. It is more common in women of childbearing age, particularly those who are overweight or obese.
Symptoms
Persistent headache (often daily, throbbing, and worse with straining)
Vision changes (blurred vision, double vision, temporary dimming or loss of vision (transient visual obscurations), blind spots)
Ringing in the ears (tinnitus) - often described as pulsatile, matching heartbeat
Neck, shoulder, or back pain
Nausea and vomiting
Dizziness
Stiffness
Causes
The exact cause of pseudotumor cerebri is unknown (idiopathic). However, several factors are associated with an increased risk:
Obesity: Especially in women of childbearing age.
Certain Medications: Such as tetracycline antibiotics, high doses of vitamin A, growth hormones, and some birth control pills.
Underlying Medical Conditions: Such as sleep apnea, polycystic ovary syndrome (PCOS), and Addison's disease.
Venous Sinus Stenosis: Narrowing of the large veins that drain blood from the brain.
Changes in hormone levels
Medicine Used
Acetazolamide (Diamox): A diuretic that reduces cerebrospinal fluid (CSF) production. This is often the first-line treatment.
Topiramate (Topamax): An anticonvulsant that can also reduce CSF production and may promote weight loss.
Furosemide (Lasix): Another diuretic, sometimes used in combination with acetazolamide.
Pain relievers: Over-the-counter or prescription medications to manage headaches.
Is Communicable
No, pseudotumor cerebri is not communicable. It is not an infectious disease and cannot be spread from person to person.
Precautions
Weight management: If overweight or obese, losing weight can significantly reduce symptoms and pressure.
Medication review: Review all medications with a doctor to identify and potentially discontinue any that could be contributing to the condition.
Regular eye exams: To monitor vision and optic nerve health.
Low sodium diet: In some cases, reducing sodium intake may help.
Sleep apnea treatment: If sleep apnea is present, seek treatment.
How long does an outbreak last?
Pseudotumor cerebri isn't an "outbreak" condition. It's a chronic condition that can last for months or years. Symptoms can fluctuate in severity over time, and some people may experience periods of remission followed by relapses. The duration of symptoms and the course of the condition vary from person to person.
How is it diagnosed?
Neurological Exam: To assess vision, reflexes, and other neurological functions.
Eye Exam (including fundoscopy): To look for swelling of the optic disc (papilledema), a hallmark of increased intracranial pressure. Visual field testing is performed to look for blind spots.
Brain Imaging (MRI or CT Scan): To rule out other causes of increased intracranial pressure, such as a brain tumor, blood clot, or hydrocephalus. A MRV or CTV of the head can be done to evaluate the venous sinuses.
Lumbar Puncture (Spinal Tap): To measure the opening pressure of the cerebrospinal fluid (CSF) and to analyze the fluid for infection or other abnormalities. Elevated CSF pressure is a key diagnostic criterion.
Timeline of Symptoms
The onset and progression of symptoms can vary.
Early Stages: Initial symptoms may be mild and intermittent, such as occasional headaches or brief episodes of blurred vision.
Progression: As the condition progresses, headaches may become more frequent and severe. Vision changes may become more noticeable and persistent, with development of blind spots or double vision. Tinnitus and other symptoms may also appear.
Long-Term: Without treatment, vision loss can become permanent.
Important Considerations
Vision Loss: The primary risk of pseudotumor cerebri is permanent vision loss. Early diagnosis and treatment are crucial to protect vision.
Monitoring: Regular follow-up with a neurologist and ophthalmologist are essential to monitor the condition and adjust treatment as needed.
Treatment Adherence: It's important to take medications as prescribed and follow lifestyle recommendations, even if symptoms improve.
Surgical Options: In severe cases or if medical treatment is not effective, surgical procedures such as optic nerve sheath fenestration (to relieve pressure on the optic nerve) or CSF shunting (to drain excess fluid) may be considered.
Pregnancy: Pseudotumor cerebri can be more challenging to manage during pregnancy. Close monitoring and coordination between specialists are necessary.