Paroxysmal hemicrania: a prospective clinical study of 31 cases

Abstract

Paroxysmal hemicrania is a rare syndrome characterized by repeated attacks of strictly unilateral, severe, short-lasting pain occurring with cranial autonomic features. The hallmarks of this syndrome are the relatively short attacks and the exquisite response to indometacin. We describe the phenotype of this condition in a series of 31 patients. The mean duration of attack was 17 min. The mean attack frequency was 11. The distribution of the pain was orbital and temporal in 77% of the patients, retro-orbital in 61%, frontal in 55%, occipital in 42%; although pain was also reported in the vertex, second division of trigeminal nerve, neck, nose, jaw, parietal region, ear, teeth, eyebrow, shoulder (ipsilateral and bilateral), arm and third division of trigeminal nerve. Of the cohort, 87% had lacrimation, 68% had conjunctival injection, 58% rhinorrhoea, 54% nasal congestion, ptosis and facial flushing. Other cranial autonomic features include eyelid oedema, forehead/facial sweating, sense of aural fullness and periaural swelling, miosis, mydriasis and swelling of the cheek. The majority of the patients (80%) were agitated or restless, or both, with the pain and 26% were aggressive. All patients had positive placebo control indometacin test (100-200 mg intramuscularly), or a positive oral indometacin trial or both. We suggest the International Headache Society criteria be revised to remove specification of attack site, and to include the full range of cranial autonomic features. Currently, the sine qua non for paroxysmal hemicrania is a response to indometacin. Since there is no reliable clinical marker of that response we recommend an indometacin test, either orally or by injection for any patient with lateralized discrete attacks of head pain with associated cranial autonomic symptoms.

Clinical features of unilateral headaches beyond migraine and cluster headache and their response to indomethacin

“…Th us, as already pointed out in previous studies on unilateral headaches [16,19], an abnormal neurological examination was highly predictive of a secondary headache in our sample.…”

“…In 8 patients (13%) the lesion was causally related to headache according to ICHD-II. In a case series of patients with paroxysmal hemicrania by Cittadini and colleagues, causative structural abnormalities were found in 36% of all patients who underwent imaging [16]. In our study, the most common extracranial pathologies were dental aff ections.…”

“…In the general population, prevalence of primary stabbing headache is about 10%. Th e prevalence of paroxysmal hemicrania and hemicrania continua may lie around 5 per 10000 [12,16,17]. In hospital-based studies, primary stabbing headache was diagnosed in 13% of patients with short-duration headache [5] and unilateral tension-type headache was found in 4% and 18%, respectively [18].…”

Trigeminal autonomic cephalgias

“…Описан случай эпидермоидной карциномы околоушной слюнной железы с метастазами в головной мозг, которая была причиной развития парок-сизмальной гемикрании. Пароксизмальная гемикрания встречается при системных заболеваниях соединительной ткани [50].…”

Cluster headache: what has changed since 1999?

“…In a large study on CH patients, 48 % reported the headache outside the trigeminal distribution [16]. In PH pain can occur in various nontrigeminal areas, occipital (42 %), neck (32 %), ear (13 %), ipsilateral shoulder (10 %), and arm (10 %) [17]. In SUNCT pain can occur in the back of the head and neck in 31 % and in the ear in 6 % [18].…”