Epicrania fugax: ten new cases and therapeutic results

Abstract

Objective: We aimed to report 10 new cases of epicrania fugax (EF), showing their clinical features and therapeutic responses.

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.