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      Epicrania fugax combining forward and backward paroxysms in the same patient: the first four cases

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          The first description of epicrania fugax (EF) reported brief painful paroxysms that start in posterior regions of the scalp and move forward to reach the ipsilateral forehead, eye, or nose. A backward variation, wherein pain stems from frontal areas and radiates to the posterior scalp, has also been acknowledged. We report four patients with features reminiscent of EF and the coexistence of forward and backward pain paroxysms.


          We considered all patients attending the headache outpatient office at two tertiary hospitals from March 2008 to March 2016. We enrolled four patients with paroxysms fulfilling criteria for EF and a combination of forward and backward radiations.


          In all cases, pain paroxysms moved both in forward and backward directions with either a zigzag (n=2) or linear (n=2) trajectory. Three patients presented two stemming points, in the occipital scalp and forehead (n=2) or in the parietal area and eye (n=1), whereas the fourth patient only had a stemming point located in the parietal region. Pain quality was mainly stabbing, and its intensity was moderate (n=1) or severe (n=3). The duration of the paroxysms was highly variable (3–30 seconds), and two patients reported autonomic symptoms.


          The clinical picture presented by our patients does not fit with other types of known headache or neuralgia syndromes; we propose it corresponds to a bidirectional variant of EF.

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          Most cited references 12

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          Numular headache: a coin-shaped cephalgia.

          Numular headache is a chronic, mild to moderate, pressurelike pain in a circumscribed cranial area of approximately 2 to 6 cm in diameter. Pain usually is limited to the parietal region, although it may appear in any cranial site. It is a benign process of usually unknown origin.
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            Epicrania fugax: an ultrabrief paroxysmal epicranial pain.

            Ten patients (one man and nine women, mean age 48.8 +/- 20.1) presented with a stereotypical and undescribed type of head pain. They complained of strictly unilateral, shooting pain paroxysms starting in a focal area of the posterior parietal or temporal region and rapidly spreading forward to the ipsilateral eye (n = 7) or nose (n = 3) along a lineal or zigzag trajectory, the complete sequence lasting 1-10 s. Two patients had ipsilateral lacrimation, and one had rhinorrhoea at the end of the attacks. The attacks could be either spontaneous or triggered by touch on the stemming area (n = 2), which could otherwise remain tender or slightly painful between the paroxysms (n = 5). The frequency ranged from two attacks per month to countless attacks per day, and the temporal pattern was either remitting (n = 5) or chronic (n = 5). This clinical picture might be a variant of an established headache or represent a novel syndrome.
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              Epicrania fugax: ten new cases and therapeutic results.

              We aimed to report 10 new cases of epicrania fugax (EF), showing their clinical features and therapeutic responses. Epicrania fugax has been recently described as a paroxysmal head pain starting in a focal area located at a posterior cranial region and rapidly spreading forward to the ipsilateral eye or nose along a linear or zigzag trajectory. In some patients the pain is followed by ocular or nasal autonomic features. In the prior series, 1 patient got pain relief with anesthetic blockades, while another patient improved with carbamazepine. Since the first description of EF, we have assessed 10 patients with the same clinical picture (8 women and 2 men) at the Neurology outpatient offices of our 2 centers. The mean age at onset was 48.5 years (SD: 19.8, range: 23-83). All the patients complained of strictly unilateral pain paroxysms starting at parietal (n = 5), occipital (n = 4), or parieto-occipital locations (n = 1), and immediately spreading forward through a linear pathway toward the ipsilateral forehead (n = 3) or the ipsilateral eye (n = 7), the complete sequence lasting 1-10 seconds. No trigger was identified in any of our patients, while 5 of them suffered mild pain in the stemming area between the paroxysms. Three patients had ipsilateral lacrimation, and 2 had conjunctival injection at the end of the attacks. The frequency ranged from 1 attack per week to multiple attacks per day. Neuroimaging and laboratory tests were consistently normal. Interictal pain was responsive to acetaminophen. In 3 cases a preventive was considered in order to avoid the paroxysms. Gabapentin led to significant improvement in 2 cases. The third patient did not obtain any benefit from gabapentin or amitriptyline, but improved slightly with lamotrigine. This description reinforces the proposal of EF as a new headache variant or a new headache syndrome. Anesthetic blockades, carbamazepine, gabapentin, and lamotrigine have been apparently effective in individual patients. Further observations and therapeutic trials are needed.

                Author and article information

                J Pain Res
                J Pain Res
                Journal of Pain Research
                Journal of Pain Research
                Dove Medical Press
                23 June 2017
                : 10
                : 1453-1456
                [1 ]Headache Unit, Department of Neurology, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
                [2 ]Department of Neurology, Hospital Clínico San Carlos, Madrid, Spain
                [3 ]Department of Medicine, School of Medicine, Universidad de Valladolid, Valladolid, Spain
                [4 ]Department of Medicine, School of Medicine, Universidad Complutense de Madrid, Madrid, Spain
                Author notes
                Correspondence: Ángel Luis Guerrero, Headache Unit, Department of Neurology, Hospital Clínico Universitario de Valladolid, Avda Ramón y Cajal 3, 47005 Valladolid, Spain, Tel +34 9 8342 0000, Email gueneurol@ 123456gmail.com
                © 2017 Barón-Sánchez et al. This work is published and licensed by Dove Medical Press Limited

                The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.

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