Idiopathic intracranial hypertension: Year of the Zebra
Introduction 0:00–0:24
Idiopathic intracranial hypertension, also known as pseudotumor cerebri, is a rare neurological condition that leads to increased intracranial pressure, or ICP, due to an unknown cause.
Risk factors include young individuals who are assigned female at birth, particularly those who have obesity or a history of recent weight gain.
Idiopathic intracranial hypertension can cause headaches that often get worse in the morning and during a change in position, such as bending over or lying down, as well as when coughing or sneezing.
Clinical Manifestations 0:24–1:31
Another common feature is pulsatile tinnitus, which is when an individual hears a “whooshing” noise, like rushing water, caused by turbulent blood flow through blood vessels.If left untreated, increased ICP can lead to papilledema, or swelling of the optic disc, which is the point where the optic nerve passes through the retina in the eye.
This can cause visual disturbances, such as blurry vision, light flashes, and intermittent loss of vision. Over time, there may be progressive loss of vision.
Individuals may also experience double vision when looking in a certain direction due to damage to one of the nerves responsible for eye movement.Although rare, some individuals may experience a severe decline in visual acuity that requires urgent treatment to preserve visual function.
Now, diagnosis of idiopathic intracranial hypertension is based on a history of headaches and evidence of papilledema on a fundoscopic eye exam.
Diagnosis 1:31–2:19
Next, a CT scan or MRI of the brain is performed to rule out secondary causes of increased ICP, such as a tumor. If brain imaging has been confirmed as normal, a lumbar puncture, or spinal tap, should be performed.
This is where a needle is placed into the spinal canal with the individual lying on their side, to measure pressure of the cerebrospinal fluid, or CSF, and to remove CSF for analysis.
With idiopathic intracranial hypertension, the opening pressure of CSF is elevated due to an increase in ICP, and CSF analysis does not show signs of inflammation or infection.Treatment consists of lifestyle modifications, medications, and procedures to help minimize the increased intracranial pressure and its effects.
Treatment 2:19–3:21
Lifestyle modifications include weight loss, particularly in cases where the individual has obesity or a recent weight gain.
Weight loss between 5 to 10% of the individual’s total body weight has been shown to cause remission.Medications, like acetazolamide, can also be given, which help lower the ICP by decreasing production of CSF.
In some cases, during lumbar puncture, small amounts of CSF may be drained to help with the pressure from excess CSF. In cases with rapid and severe visual disturbances, a procedure called optic nerve sheath fenestration can be done emergently.
This procedure creates a small window in the optic nerve sheath to allow the surrounding CSF to be released into the space surrounding the eye.
Alternatively, a ventriculoperitoneal shunt, which connects the ventricular system in the brain to the peritoneal cavity in the abdomen, may be placed in order to allow CSF to drain.Alright, as a quick recap, idiopathic intracranial hypertension is a rare neurological condition of unknown cause that results in headaches, nausea and vomiting, and visual disturbances.
Review3:21–3:43
It usually affects individuals who are assigned female at birth and have obesity or a recent weight gain. Treatment focuses on weight loss and medications like acetazolamide to lower intracranial pressure, as well as surgery.
- "European Headache Federation guideline on idiopathic intracranial hypertension. " J Headache Pain (2018;19(1))
- "Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. " Lancet Neurol (2016;15(1):78-91. )
- "Idiopathic intracranial hypertension: consensus guidelines on management. " J Neurol Neurosurg Psychiatry (2018;89(10):1088-1100. )
- "Cerebrospinal fluid removal for idiopathic intracranial hypertension: less cerebrospinal fluid is best. " J Neuroophthalmol (2019;39(3):330-332)
- "An update on idiopathic intracranial hypertension. " Acta Neurochir (Wien) (2017;159(3):491-499. )
- "Idiopathic intracranial hypertension: update on diagnosis and management. " Clin Med. (2020;20(4):384-388. )
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