Ideopathic intercranial hypertension

Idiopathic intercranial hypertension (IIH), also known as a pseudotumor cerebri, is a condition characterised by high pressure in the cerebrospinal fluid (CSF) without any identifiable cause revealed by imaging examinations. It is more common in women of childbearing age or who are overweight.

Symptoms

The symptoms of IIH include:

• Severe and persistent headaches, often worse when lying down.
• Blurred or double vision.
• Temporary loss of vision.
• Ringing in the ears (pulsatile tinnitus).
• Nausea and vomiting.
• Swelling of the optic nerve   (papillary oedema) visible during an ophthalmological examination.

Imaging examinations

To identify IIH and exclude other causes a number of imaging examinations are carried out:  

• Brain MRI: to visualise the brain structures and  exclude anomalies such as tumours or malformations
• Magnetic Resonance Angiography (MRA): to evaluate the cerebral veins and rule out a cerebrovascular thrombosis.  
• A CT scan of the brain: sometimes used in an emergency to rule out other causes of high intracranial pressure.

Medical pathway

Patients with IIL need to consult  number of specialists for comprehensive treatment as a matter of urgency to avoid any deterioration of  the visual function: 

• GP: for an initial assessment of symptoms and to direct the patient to the appropriate specialists.  
• Neurologist: principal specialist in diagnosing and managing IIH.  
• Ophthalmologist: to evaluate the effects of the IIH on vision and to monitor the papillary oedema.  
• Neuroradiologist: to interpret the brain imaging examinations.
• Neurosurgeon or interventional neuroradiologist: if surgery or endovascular intervention is necessary.

Treatment

IIH treatment seeks to reduce the intracranial pressure and prevent any loss of vision:  

• Weight loss: recommended for overweight patients, this is often effective in alleviating symptoms.

Medicines:  

• Acetazolamide: A diuretic that reduces the production of CSF and is a first line treatment with a high level of evidence.  
• Repeated lumbar punctures: to drain off excess CSF and used in the case of severe symptoms.  

Surgery:  

• Optic nerve decompression: to prevent irreversible loss of vision in the case of severe papillary oedema.  
• Cerebrospinal shunt: to drain off the CSF if other treatments fail.  
• Interventional neuroradiology: venous sinus stenting to improve drainage of venous blood and reduce intracranial pressure when these veins narrow.  

Multidisciplinary discussion  

IIH management requires a multidisciplinary approach that includes meetings between neurologists, ophthalmologists, interventional neuroradiologists, neurosurgeons and other health professionals to discuss complex cases and coordinate treatment  

Follow up

The monitoring of IIH patients is crucial and includes:  

• Regular vision monitoring: by an ophthalmologist to detect any deterioration.  
• Neurological monitoring: to assess the effectiveness of treatment and make adjustments when necessary.  
• Regular weight evaluations: plus dietary advice for overweight patients.
• Regular imaging examinations: to monitor intracranial pressure and the condition of brain structures.

The follow up is carried out by a multidisciplinary team to optimise the clinical results and improve the quality of life of patients.