Epicrania Fugax

Abstract

Letter to the Editor Dear Editor, Epicrania fugax has recently been defined as a paroxysmal headache localized in the posterior cranial area, which starts at a focal area and rapidly radiates ipsilaterally around the eye or the nose (1,2). Autonomic findings can accompany some cases (2,3). In this syndrome, the average onset age of which is 48 years, the pathogenesis has not been fully enlightened. Its treatment is similar to the treatment of neuralgiform pains. In this article, a case who was diagnosed with epicrania fugax, a newly defined primary headache, and who responded to gabapentin (1800 mg/day) treatment was presented. A 59-year-old female patient presented to our neurology polyclinic with a complaint of headache that had persisted for 2 years. The headache was moderate or severe, it lasted 5-10 s, it started from the right side of the back of the head and radiated to the eye, it was a stabbing type of pain, it was accompanied with lachrymation, and its attack frequency was 2-3 times a day. Neurological and other system examinations were normal. There was no risk factor other than hypertension. Contrast brain-diffusion magnetic resonance imaging and magnetic resonance angiography examinations were normal. No pathology was found in the laboratory examinations. The patient did not respond to indomethacin (150 mg/day) and carbamazepine (600 mg/day) treatments. The case whose headache was reconsidered was thought to have epicrania fugax. Our patient who began to have less headaches with the treatment of gabapentin 1800 mg/day is still being followed by our polyclinic. Epicrania fugax was first defined by Pareja et al. (1) in 2008. Although there were only 20 cases defined until 2009, this number has currently increased to 100 (1,2,3). Its characteristics that differentiate it from other epicranias and neuralgiform aches are the origin of the headache, its duration, and how it radiates (4). The type of headache that can be confused the most with epicrania fugax is the short-term, one-sided neuralgiform headache accompanied by autonomic findings; however, this type of headache does not radiate (5). Although its pathogenesis is not fully known, peripheral and central mechanisms are thought to play a role (1,2). Case reports show responses to the treatments of occipital nerve block, carbamazepine, lamotrigine, pregabalin, and gabapentin (1,2,3). In one-sided, short-term headaches that start from a focal region at the back of the head and quickly radiate to the nose and the eye, epicrania fugax, which is a newly defined type of headache, should be considered.

Epicranial headaches part 2: Nummular headache and epicrania fugax

“…EF is viewed as a rare headache disorder, although its incidence and prevalence in the general population are unknown. To date, 82 cases with a typical picture of EF have been published (29,36,(71)(72)(73)(74)103,104,(106)(107)(108)(109)(110)(111). This figure excludes cases with atypical features and cases attributed to an underlying disease, as well as repetitions of individual patients in more than one publication.…”

“…Depending on the direction of movement two main clinical forms have been distinguished: posteroanterior or forward EF, which is more frequent (two thirds of the cases described), and anteroposterior or backward EF (Figure 5). In the posteroanterior form the pain starts in a specific area of the occipital, parietal, or temporal region, and then radiates forward, reaching the ipsilateral forehead, temple, eye, or nose (29,36,71,72,74,103,(107)(108)(109)(110)(111); in the anteroposterior form the pain follows a reverse path: it originates in the frontal region, eye, or nose, and goes backwards, towards the parietal or occipital region (36,73,74,104,106,110). Normally the attacks are strictly unilateral with no side shift.…”

“…During painful paroxysms, 30% of patients may have cranial autonomic accompaniments ipsilateral to the pain (29,36,(71)(72)(73)(74)103,106,107,109,111). The most common autonomic symptom is lacrimation; other possible autonomic symptoms are conjunctival injection, a gritty sensation in the eye, eyelid edema, eyelid ptosis, and rhinorrhea.…”

“…The drugs most used in EF are antiseizure medications; satisfactory therapeutic responses have been reported for several drugs in this group, especially gabapentin (30,(71)(72)(73)(74)109,116,(120)(121)(122)124) and lamotrigine (71)(72)(73)(74)(75)116,120,121,124) but also pregabalin (74,121), carbamazepine (73,103,117,121), oxcarbazepine (120,121), eslicarbazepine (107,121,125), lacosamide (121), and levetiracetam (74). Amitriptyline (73,75,116,122), duloxetine (120), and indomethacin (74,75) have also been used with effect in a minority of patients.…”