A. Headache for a total of more than 2 months.
B. Obligatory features
1. Unilaterality without side shift.3
2. Absolute and protracted indomethacin effect.*
3. Long-lasting, repetitive attacks—hours/days/weeks, with a tendency to a fluctuating chronic pattern over time.c
4. Intensity of pain: mild, moderate or severe (not excruciatingly severe).
C. Other nonobligatory but frequent characteristics of the pain syndrome 1. Female sex.
D. Negative provisos
1. Relative shortage of "local" autonomic phenomena.d
2. Relative lack of "migraine" symptoms and signs.6
3. Relative lack of "cervicogenic" symptoms and signs.'
4. Lack of effect of migraine and cluster headache drugs (triptans and ergotamine).
3The pain is mostly in the "anterior" area, but not infrequently also in the auricular/occipital area. ^Provided the dosage is adequate: 150 mg per day for 3 days. In the doubtful case, the "indotest" should be carried out (see Headache 1998;38:122-128). This is particularly important in the remitting cases, because a betterment of pain in reality being because of a remission may falsely be ascribed to indomethacin.
There are two forms of hemicrania continua from a temporal point of view: a remitting and nonremitting (chronic) form. There may be transitions from the one temporal pattern to the other. The continuous pattern may eventually seem to dominate.
"Lacrimation, conjunctival injection, rhinorrhea and nasal obstruction; such signs are usually meager and on the symptomatic side, and, if present, they occur mostly during the more severe attack periods.
'Nausea, vomiting, photo- and phonophobia, pulsatile character of pain, and accentuation upon mild physical activity. 'Reduction of range of motion in the neck; ipsilateral upper extremity discomfort; mechanical precipitation of pain/attacks. (Adapted with permission from Pareja, JA, Vincent, M, Antonaci, F, Sjaastad, O. Hemicrania continua: diagnostic criteria and nosologic status. Cephalalgia 2001;21:874-877, and from Blackwell Publishing.)
investigated in the history. SUNCT syndrome (short-lasting unilateral neuralgiform headache with conjunctival injection and /earing) is a rare but distinct syndrome.
Secondary causes of paroxysmal hemicranias have been reported as results of various causes including collagen vascular diseases, intrinsic brain tumors, gangliocytoma of the sella turcica, and cavernous sinus meningioma.
An alternative set of diagnostic criteria has also been proposed based on the expansion of the clinical syndrome since the original description of diagnostic criteria presented above. These include some cases not responsive to indomethacin and others responsive to other nonsteroidal anti-inflammatories. Hemicrania continua may also exist in an episodic, as well as a chronic, form, suggesting the existence of clinical (if not biological) subtypes (see Tables 10 and 11).
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