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Hey, it’s been a couple years since I’ve done this sort of thing. Let’s get back to it.

black-woman-with-headache-600x300A 32 y/o African-American female presented with a headache x3 days. The pain was described as being the worst around and behind the eyes. Pt said she preferred to sit in the dark. Regular Motrin provided temporary relief. Pt said she thought it might be a sinus infection. She denied any fevers, stuffy nose, trouble breathing, runny nose, postnasal drip, cough. The pain is not changed by looking up or down. Pt describes the pain as being “funny.” She denied any past medical history of migraines. Her history does include asthma, and “swollen optic nerves” found a couple years ago on routine eye exam. Pt says she saw a neuro-opthalmologist for the latter; scans of her head and a spinal tap were reportedly normal and no other interventions were performed. Pt has not had this kind of headache before.

Vitals were normal, besides pt being overweight. Her lungs were clear. She had no particular tenderness on her face or forehead. Her neck was non-stiff. Her cranial nerves showed no obvious problems. No muscle weakness or numbness were present.

WHAT IS THE MOST LIKELY DIAGNOSIS?

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Pseudotumor cerebri, aka idiopathic intracranial hypertension

Although the pt was unsure of the cause of her “swollen optic nerves,” upon further questioning she has indeed heard the term “pseudotumor” before, and she was told her condition was related to her weight.

Pseudotumor cerebri, or increased pressure of the fluid inside the brain for no known cause, almost exclusively strikes overweight women. For some reason, this cerebrospinal fluid accumulates, and the resulting pressure is most observable in the back of the eye, as swelling occurs around the optic nerve — a sign called “papilledema.” While sometimes asymptomatic, this pressure obviously can produce massive headaches as a result. And pseudotumor is usually a chronic condition, with no permanent solution other than, sometimes, losing weight.

Papill2That’s the bad news. The good news: if you are forced to pick a reason to have papilledema, pseudotumor is damn well your best option. Better a pseudotumor then, you know… an actual freaking tumor. In fact, that’s how the condition got its name: physicians found patients with a classic sign of brain tumors, papilledema; yet no tumors were ever found. While the headaches can be intense, they can be relieved by simply draining the fluid out of the spinal cord. In other words, this condition leads to the interesting solution known as a therapeutic lumbar puncture. 

Other options include the addition of an unusual kind of diuretic that reduces production of cerebrospinal fluid. Patients and physicians can agree this is a better option than getting a spinal tap a few times a year. Brain surgery is a more extreme option if all other options fail.

And while weight as well as female gender is usually related to pseudotumor, there is no known specific cause — hence the “idiopathic” in its other name. This also means that other causes of increased intracranial pressure (ICP; yes, the pressure in your head has the same initials as a certain notorious hip-hop duo) have to be ruled out first by CT and MRI. Unlike in a related condition that strikes older people, in younger pts, the brain holds firm against all this increased fluid, and brain scans only show the most subtle of changes. The main way to truly diagnose pseudotumor is by measuring an elevated ICP, generally via lumbar puncture, with the absence of markers of other conditions.

Anyway, the patient was advised she needed to go to the ER for brain imaging and an LP. Pt was in tears over this but I told her, with proper treatment, it usually leaves no lasting damage at all. She will need to follow up with a neurologist, probably get put on that diuretic, and be told to look into weight loss.