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Atypical Headache: A Case Study

An atypical headache may or may not be a migraine. If migraine symptoms are not present this may be a headache type that is an emergency headache. Unfortunately, even emergency headaches may start slowly and gather steam so to speak.


  • Case Study:

A 26 year old overweight female with a 4 month history of new atypical headache came to our office. Mom has migraines; however the patient's are different..no throbbing, no light sensitivity, no nausea.

The headaches start about 10AM and are relieved by sleeping. They are also worse if she coughs or has a bowel movement.

She says her vision is normal, she has no weakness, no other symptoms.

She works in a doctor's office so he put her on Topamax which had little to no effect.

None of the triptans (Imitrex,etc) affected the headache. She was also put on a one week course of steriods which DID help the headache.

Her examination was normal except for some pulsating veins on her retina.

Diagnosis? Intracranial Hypertension vs Chiari I malformation.

Unusual headaches should be investigated further by a headache specialist. Even primary care physicians may misdiagnose migraines and other atypical headache. This could have easily become an emergency, and probably should have had an MRI of the brain done LONG before 4 months went by.

We put her on a higher dose of steriods to relieve the pressure in the brain and did the MRI of the brain. Fortunately this was a very mild case. While the diagnosis of intracranial hypertension is rare, any atypical headache that is new and "weird" should be investigated and an MRI ordered. Don't ignore any atypical headache symptoms.

Chiari I malformation is when the back of the brain (the cerebellum) starts slipping down and pressing on the spinal cord. This tends to worsen throughout the day due to gravity, and is relieved by lying down. It can run in families and unless serious, where surgery is needed, usually is asymptomatic and left alone.

When it comes to intracranial hypertention or pseudotumor cerebri as it is also called, many times the patient will start to have blurred vision with the headache. This type of headache typically worsens with cough or bending over. These are valsalva maneuvers which temporarily increase pressure and make the headache worse. The reason for the blurry vision is that the ventricles in the brain begin to enlarge and press on the optic nerves causing visual distortion. As the problem gets worse, peripheral blood pressure will also start to rise. The condition is associated with young morbidly obese females.

A course of steroid helps reduce the pressure in the brain and takes the headache away. If patients are severely symptomatic with visual distortions, we will do a lumbar puncture in the office to remove some of the cerebrospinal fluid and take the pressure off the brain. This is both therapeutic and diagnostic as we can measure the pressure of the fluid as it comes out. Elevated CSF pressure is diagnostic.

Topamax did not work in this case as when pseudotumor occurs, topamax is used after the condition stablizes and is more of a preventative. More often than not, patients are on Diamox also, which prevents reoccurance of this atypical headache.

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