1. Absolute response to indomethacin or

2. One of the following autonomic features with severe pain exacerbation:

a. Conjunctival injection.

b. Lacrimation.

c. Nasal congestion.

d. Rhinorrhea.

e. Ptosis.

f. Eyelid edema.

E. At least one of the following:

1. No suggestion of a disorder, such as trigeminal neuralgia, idiopathic stabbing headache, cough headache, benign exertional headache, headache associated with sexual activity, or hypnic headache.

2. Such a disorder is suggested but excluded by appropriate investigations.

3. Such a disorder is present, but first headache attacks do not occur in close temporal relation to the disorder. Comment: Hemicrania continua is usually continuous (hemicrania continua-chronic), but rare cases of remission have been reported. Also, an episodic pattern with pain-free remissions lasting weeks to months may precede the continuous headache phase in up to a third of patients, and in some cases the pattern may remain episodic (hemicrania continua-episodic). Painful exacerbations may be associated with migraine-related symptoms of photophobia, phonophobia, nausea, vomiting, and even aura.

Adapted with permission from Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias SUNCT syndrome and other short-lasting headache with autonomic features, including new cases. Brain 1997;120:193-209.

Headaches caused by CSF fistulas and low CSF volume and pressure may be difficult to diagnose easily, and finding the source of the CSF leak can be quite challenging, often requiring imaging of the entire neuraxis, and sometimes involving radionuclide tracer studies to localize the leak. They may resolve spontaneously with simple measures, such as bed rest, or sometimes require surgical repair. Subdural hematoma can occur secondary to low CSF pressure.

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