Epileptic headache: A rare form of painful seizure

Purpose To describe the concept, features and mechanisms of epileptic headache (EH). Methods Analysis of all published articles concerning EH and related subjects. Results There are more than 30 published case studies of patients with headache as the only manifestation of a seizure, a condition that has been variously called “EH” “ictal epileptic headache” “hemicrania epileptica” “cephalic pain seizure”. It is necessary to differentiate EH from “migralepsy” and “ictal non-epileptic headache”. EH may be an isolated event or the initial phase of a seizure followed by other manifestations. An isolated EH is clinically relevant because it is often symptomatic of structural brain disease; this underlines the importance of a differential diagnosis as the head pain of EH has no specific diagnostic characteristics. The described cases indicate that the location of the foci may vary, thus suggesting the involvement of different parts of the pain network. EH is a “focal aware” seizure, but there are a few reports of cases in which it was associated with generalised epileptiform activity. A correct diagnosis of EH requires an ictal EEG recording showing epilepsy-compatible discharges that coincide with the onset and cessation of the headache. A rapid response to the acute administration of an antiepileptic drug may support the diagnosis. Conclusions EH is a particular type of pain seizure that has a complex pathophysiology and, when isolated, requires differential diagnostic consideration. We believe that, although it is not frequent, pain as an ictal symptom should be highlighted in the operational classification of seizure types.