Management of Idiopathic Intracranial Hypertension

Clinical Presentation and Diagnosis

IIH predominantly affects women with BMI >30 kg/m², with symptoms including progressively worsening headache, transient visual obscurations, pulsatile tinnitus, visual blurring, and horizontal diplopia 1, 2
Papilledema is the hallmark finding, though IIH without papilledema is a rare subtype 2
Neuroimaging (MRI brain preferred, or CT if MRI unavailable) should be performed within 24 hours to exclude secondary causes 2
CT or MR venography is mandatory to exclude cerebral sinus thrombosis 2
Typical neuroimaging findings include empty or partially empty sella, increased optic nerve tortuosity, enlarged optic nerve sheath, flattened posterior globe/sclera, intraocular protrusion of optic nerve head, and transverse sinus stenosis 3

Weight loss is the primary disease-modifying treatment for IIH 3
When there is evidence of declining visual function, acute management to preserve vision is surgical 3
A temporizing lumbar drain may protect vision while planning urgent surgical treatment 3
Surgical options include CSF diversion procedures, optic nerve sheath fenestration (ONSF), and venous sinus stenting 3, 4
Patients must be informed early about the risks of medication overuse headache 4
Topiramate may have a role in IIH management with weekly dose escalation from 25 mg to 50 mg twice daily 4
Women prescribed topiramate must be informed about reduced contraceptive efficacy and potential side effects including depression, cognitive slowing, and teratogenic risks 4

VP shunts should be the preferred CSF diversion procedure for visual deterioration in IIH 3
Neuronavigation should be used to place VP shunts 3
Adjustable valves with antigravity or antisiphon devices should be considered to reduce the risk of low-pressure headaches 3
ONSF has fewer complications than CSF diversion but is performed more frequently in Europe and the USA than in the UK 4
Neurovascular stenting shows promise but its role is not yet fully established; long-term antithrombotic therapy is required for longer than 6 months following this procedure 4

Treatment failure rates for surgical interventions include worsening vision after initial stabilization in 34% of patients at 1 year and 45% at 3 years 3
Failure to improve headache occurs in one-third to one-half of surgically treated patients 3
Patients with IIH in ocular remission may still have ongoing headache morbidity requiring neurological management 5
Fulminant IIH requires emergency referral and management 5
Patients in the UK should inform the Driver and Vehicle Licensing Agency following VP shunt placement 3

Patients with IIH may experience additional symptoms such as dizziness, neck pain, back pain, cognitive disturbances, and radicular pain, according to the American Academy of Neurology 6
Short-term analgesics, including NSAIDs or paracetamol, with indomethacin potentially advantageous due to its ICP-reducing effects, may be used for symptom management, as recommended by the American Headache Society 6

The American Academy of Neurology recommends short-term analgesics, such as NSAIDs or paracetamol, for pain management in IIH patients 6
Serial lumbar punctures provide only temporary relief, lasting hours, and are not recommended for long-term management, as stated by the American Academy of Neurology 6