What is the preferred term for Idiopathic (IIH) Intracranial Hypertension?

Preferred Term for Idiopathic Intracranial Hypertension (IIH)

The preferred term for this condition is "Idiopathic Intracranial Hypertension (IIH)" as established by consensus guidelines from the Journal of Neurology, Neurosurgery and Psychiatry. 1

Rationale for Current Terminology

The term "Idiopathic Intracranial Hypertension" has been formally established through:

• Consensus guidelines developed by a specialist interest group including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives 1
• Endorsement by multiple professional bodies including the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists 1
• Consistent use in contemporary medical literature and diagnostic criteria 2

Historical Context and Terminology Evolution

The condition has been known by various names over time:

• "Pseudotumor cerebri" (older term still occasionally used) 3
• "Benign intracranial hypertension" (outdated term)

The shift away from these older terms reflects improved understanding of the condition's potential serious consequences, particularly regarding vision loss. The term "benign" was abandoned as it incorrectly suggested the condition was harmless, when in fact it can lead to permanent visual impairment if not properly managed 1, 2.

Subtypes and Classification

Current guidelines recognize several subtypes of IIH that should be specified when discussing the condition 1, 2:

• Typical IIH: Female, reproductive age, BMI >30 kg/m²
• Atypical IIH: Not meeting typical criteria (not female, not of reproductive age, or BMI <30 kg/m²)
• Fulminant IIH: Rapid visual decline within 4 weeks of diagnosis
• IIH without papilledema (IIHWOP): Meets all criteria except for papilledema
• IIH in ocular remission: Previously diagnosed IIH where papilledema has resolved

Diagnostic Criteria

The diagnosis of IIH requires all of the following 1, 2:

• Papilledema
• Normal neurological examination (except for possible sixth nerve palsy)
• Normal brain parenchyma on neuroimaging
• Normal CSF composition
• Elevated lumbar puncture opening pressure (>250 mm H₂O in adults)

Clinical Implications of Terminology

Using the correct terminology is important for:

1. Accurate communication between healthcare providers
2. Proper documentation in medical records
3. Appropriate patient education about the potential seriousness of the condition
4. Consistency in research and clinical trials

Conclusion

The term "Idiopathic Intracranial Hypertension (IIH)" should be used when referring to this condition, with appropriate subtype specification when applicable. This terminology accurately reflects the pathophysiology (increased intracranial pressure) while acknowledging that the underlying cause remains unknown (idiopathic) in most cases.