What are the causes and management of benign raised Intracranial Pressure (ICP)?

From the Guidelines

Benign raised intracranial tension (ICT) should be managed with weight loss, acetazolamide, and monitoring of visual fields to prevent permanent vision loss. The primary causes of benign raised ICT include obesity, certain medications, endocrine disorders, and systemic conditions.

• Obesity is a major risk factor, especially in women of childbearing age, and weight loss is a crucial part of management, as even a 5-10% reduction can significantly improve symptoms 1.
• Medications such as tetracyclines, vitamin A derivatives, steroids, and lithium can contribute to the development of benign raised ICT.
• Endocrine disorders like Addison's disease and hypoparathyroidism, as well as systemic conditions such as lupus or kidney failure, can also increase the risk of developing this condition. Management of benign raised ICT involves:
• Weight loss for obese patients
• Medication therapy with acetazolamide or topiramate to reduce CSF production
• Monitoring of visual fields and fundoscopy to assess for papilledema
• Discontinuation of contributing medications and management of underlying conditions
• Regular lumbar punctures can provide temporary relief by directly removing CSF, but this is not a long-term solution and should be used with caution, as recommended by guidelines for managing elevated intracranial pressure 1. It is essential to note that treatment with steroids has yielded mixed results and is not recommended for patients with HIV disease, and its use in HIV-negative patients is also not well-established 1. Acetazolamide and mannitol have not been shown to provide any clear benefit in the management of elevated intracranial pressure resulting from cryptococcal meningitis, but acetazolamide is effective in reducing CSF production in benign raised ICT 1. In severe cases with visual deterioration, surgical interventions like optic nerve sheath fenestration or CSF diversion procedures may be necessary. Overall, the goal of management is to reduce intracranial pressure, prevent permanent vision loss, and improve quality of life.

From the Research

Definition and Causes of Benign Raised Intracranial Pressure

• Benign raised intracranial pressure, also known as idiopathic intracranial hypertension (IIH), is a condition characterized by increased intracranial pressure without any identifiable cause 2.
• The exact causes of IIH are unknown, but it is often associated with obesity, female sex, and certain medical conditions 2.

Symptoms of Benign Raised Intracranial Pressure

• Common symptoms of IIH include headache, visual disturbances, papilledema, and sixth nerve palsy 2.
• Headaches are often non-specific and can be severe, while visual disturbances can range from blurred vision to loss of peripheral vision 2.

Management of Benign Raised Intracranial Pressure

• Treatment of IIH aims to reduce intracranial pressure, prevent visual loss, and manage symptoms such as headaches 2.
• Weight loss is a key component of treatment, as it can help reduce intracranial pressure and improve symptoms 3, 2.
• Medications such as acetazolamide can also be used to reduce intracranial pressure and improve symptoms 4, 5, 3, 6.
• Acetazolamide works by inhibiting the production of cerebrospinal fluid, which can help reduce intracranial pressure 5.
• In some cases, surgical intervention may be necessary to relieve pressure and prevent permanent visual loss 2.

Safety and Tolerability of Acetazolamide

• Acetazolamide is generally well-tolerated, but can cause side effects such as paresthesia, dysgeusia, nausea, and fatigue 6.
• The majority of patients can tolerate acetazolamide at dosages up to 4g/d for 6 months, although some may experience adverse events 6.
• The safety profile of acetazolamide is acceptable, making it a viable treatment option for IIH 6.