Idiopathic Intracranial Hypertension (IIH)

Introduction

Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri, is a condition characterized by increased pressure inside the skull without an obvious cause. This pressure can lead to headaches, vision problems, and other neurological symptoms. It primarily affects overweight women of childbearing age.

Causes and Risk Factors

While the exact cause of IIH remains unknown, several factors may contribute to its development: - Obesity – Strongly linked to IIH, especially in younger women. - Hormonal changes – Including pregnancy, polycystic ovary syndrome (PCOS), and endocrine disorders. - Certain medications – Such as tetracyclines, excessive vitamin A intake, and corticosteroid withdrawal. - Venous outflow obstruction – Some studies suggest impaired cerebrospinal fluid drainage may contribute.

Symptoms

• Persistent headaches (often worse when lying down or with movement).
• Visual disturbances (blurred vision, double vision, or temporary vision loss).
• Pulsatile tinnitus (hearing a rhythmic sound in the ears).
• Papilledema (swelling of the optic nerve due to increased intracranial pressure).
• Nausea and dizziness.

Diagnosis

Diagnosis involves ruling out other causes of increased intracranial pressure: 1. Brain imaging (MRI or CT scan) to exclude tumors or structural abnormalities. 2. Lumbar puncture (spinal tap) to measure cerebrospinal fluid pressure and analyze its composition. 3. Ophthalmologic examination to assess papilledema and visual field defects.

Treatment Options

Treatment focuses on reducing intracranial pressure and alleviating symptoms.

1. Lifestyle Modifications

• Weight loss – Even a modest reduction (5-10% of body weight) can significantly improve symptoms.
• Healthy diet and exercise – Maintain a balanced diet to prevent further weight gain.

2. Medications

• Acetazolamide – A diuretic that reduces cerebrospinal fluid production.
• Topiramate – Often used in patients who also need weight loss benefits.
• Pain relievers – For managing chronic headaches.

3. Surgical Interventions (For Severe Cases)

• Optic nerve sheath fenestration – To relieve pressure on the optic nerve and prevent vision loss.
• Shunt placement (lumbar or ventricular shunt) – To drain excess cerebrospinal fluid.
• Venous sinus stenting – In cases where venous obstruction is suspected as a contributing factor.

Prognosis

With appropriate treatment, most patients experience symptom relief. However, untreated IIH can lead to permanent vision loss.

Conclusion

Early detection and treatment of IIH are crucial to prevent complications, particularly vision loss. If you experience persistent headaches with visual symptoms, seek medical attention promptly.

Source recommendations

1. American Academy of Neurology Guidelines on Idiopathic Intracranial Hypertension

1. https://www.aan.com/practice/guidelines
2. https://pmc.ncbi.nlm.nih.gov/articles/PMC6166610/
3. https://www.aan.com/Guidelines/home/GuidelineDetail/725
4. https://pmc.ncbi.nlm.nih.gov/articles/PMC4676757/
5. https://www.neurology.org/doi/10.1212/WNL.0000000000207866

2. European Society of Neurology Guidelines on Idiopathic Intracranial Hypertension

1. https://thejournalofheadacheandpain.biomedcentral.com/articles/10.1186/s10194-018-0919-2
2. https://pmc.ncbi.nlm.nih.gov/articles/PMC6166610/
3. https://academic.oup.com/ejendo/article/187/2/323/6972055
4. https://journals.sagepub.com/doi/full/10.1177/03331024231197118
5. https://www.aan.com/practice/guidelines