Idiopathic intracranial hypertension
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Idiopathic intracranial hypertension, also known as pseudotumor cerebri, is characterized by an unexplained increase in pressure surrounding the brain. Its symptoms mimic those of a brain tumor, and the elevated intracranial pressure may lead to optic nerve swelling and subsequent vision impairment.
Idiopathic intracranial hypertension happens when there is an accumulation of cerebrospinal fluid (CSF), which surrounds and cushions the spinal cord and brain. Pressure accumulates within your cranial cavity, exerting force on the optic nerve located at the rear of your eye, responsible for visual perception.
While pseudotumor cerebri can occur in individuals of various age groups, it primarily impacts women who are in their reproductive years, especially those with excess weight. Management strategies for this condition may encompass pharmaceutical therapies and surgical procedures.
Symptoms
An abrupt, severe headache is the most typical symptom of idiopathic intracranial hypertension. Occasionally, the intensity of the headache is so excruciating that it rouses you from slumber.
Other signs and symptoms may include:
- Loss of vision
- Loss of side or peripheral vision
- Double vision
- Temporary episodes of blindness that impact one or both eyes and last only a few seconds
- Seeing bursts of light
- A pulsating, whooshing sound inside your head that synchronizes with your heartbeat.
- Tinnitus, or ear ringing sensation
- Lightheadedness, vomiting or nausea
- Pain in the neck, shoulder, or back
- Fatigue
Causes
The brain and spinal cord are enveloped by cerebrospinal fluid, providing a protective cushion against potential injuries to essential tissues. This fluid is produced in the brain and subsequently absorbed into the bloodstream at a rate that typically maintains a constant pressure within the brain.
In the case of idiopathic intracranial hypertension, cerebrospinal fluid accumulates without any identifiable cause. The increased intracranial pressure associated with pseudotumor cerebri may stem from an issue in the absorption mechanism.
Certain cases of intracranial hypertension are linked to identifiable factors. Individuals may experience chronic intracranial hypertension due to health conditions such as brain tumors or blood clots. When a specific cause is identified, the condition is termed secondary intracranial hypertension, as opposed to idiopathic.
Risk factors
The likelihood of developing idiopathic intracranial hypertension is higher in women compared to men. The majority of cases occur in individuals aged between 20 and 50 years.
Other risk factors for pseudotumor cerebri include:
- Being overweight: The likelihood of developing this condition increases in women of reproductive age who are obese, or with a body mass index (BMI) exceeding 30.
- Certain drugs: Secondary intracranial hypertension can be associated with certain medications, including growth hormone, tetracycline, and excessive intake of vitamin A.
- Underlying health issues: Secondary intracranial hypertension is associated with various health problems, including Addison’s disease, anemia, blood-clotting disorders, kidney disease, lupus, polycystic ovary syndrome, sleep apnea, and underactive parathyroid glands.
Diagnosis
The diagnosis of idiopathic intracranial hypertension can be challenging given that its symptoms may resemble those of a brain tumor, thus formerly known as pseudotumor cerebri.
To rule out a tumor and determine the cause of the symptoms, the healthcare provider will need to analyze one’s medical history and symptoms, perform a physical exam, and require some tests.
The tests that may be ordered include:
- Eye exams: A visual field examination will be conducted to detect any potential blind spots in the vision. Generally, the back of the eye is examined for a unique swelling that affects the optic nerve when there is a suspicion of pseudotumor cerebri. Photographs of the eyes will likely be taken, and an imaging test, such as optical coherence tomography, will be performed to assess the thickness of retinal layers.
- Brain imaging: Imaging tests may be recommended to help rule out conditions like brain tumors and blood clots that can present with identical symptoms. These may include a computed tomography (CT) scan or magnetic resonance imaging (MRI).
- Spinal tap (lumbar puncture): In this procedure, a needle is inserted between two vertebrae in the lower back, extracting a small sample of cerebrospinal fluid for laboratory testing. This test may be requested to assess the spinal fluid and gauge the pressure inside the skull.
Treatment
Treatment typically leads to an improvement in symptoms associated with intracranial hypertension for the majority of patients. The primary objective of treatment is to alleviate symptoms and safeguard vision from deterioration.
After receiving treatment, it is crucial to maintain regular follow–up appointments with a healthcare provider to oversee one’s vision. In the absence of appropriate treatment, intracranial hypertension has the potential to cause permanent vision loss.
Treatment options for idiopathic intracranial hypertension include:
- Weight loss: For individuals with a high BMI, symptom relief can be achieved through weight loss. The healthcare provider might suggest shedding 5% to 10% of the body weight as a recommended approach. A weight–loss diet that is low in sodium can also alleviate symptoms. Certain individuals may find relief through weight–loss programs or gastric surgery.
- Medications
- Glaucoma medications: Acetazolamide may be prescribed to lessen symptoms and the amount of cerebrospinal fluid produced. Stomach upset, kidney stones, weariness, tingling in the fingers, toes, and mouth are among possible adverse effects.
- Diuretics: Another diuretic may be prescribed to increase urine production and decrease fluid retention. This is advised when acetazolamide by itself is ineffective.
- Migraine medications: Excruciating headaches that frequently accompany pseudotumor cerebri may be eased by migraine drugs.
- Surgery: Severe cases of intracranial hypertension may necessitate surgery, especially in cases where the vision deteriorates.
- Optic nerve sheath fenestration: In most cases, vision either stabilizes or shows improvement with this treatment. During the procedure, an opening is created in the membrane encasing the optic nerve to enable the release of excess cerebrospinal fluid. When this treatment is performed on one eye, most patients report benefits to both eyes. However, this procedure may contribute to increased vision issues and success is not always guaranteed.
- Spinal fluid shunt: Shunt placement is typically considered when other treatments have not provided relief. However, shunts are prone to blockages, often necessitating additional surgeries to maintain functionality.
In this procedure, the shunt is placed into the lower part of the spine or brain to assist in removing extra cerebrospinal fluid. The extra fluid is released via the shunt in the abdomen, where the tubing is buried beneath the skin. Infections and low–pressure headaches are the possible complications.
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- Venous sinus stenting: Further research is still required to ascertain the advantages and drawbacks of this procedure. To enhance blood flow, a stent is placed in one of the larger veins in the head.
