Epileptic headache: A rare form of painful seizure

- The features of the 32 published cases are detailed and the characteristics of EH delineated.
- The isolated form of EH is clinically relevant for a differential diagnosis.
- The particular pathophysiology of these painful seizures is evaluated.
- Related terms, including “Ictal non-epileptic headache”, are discussed.
- Stringent new criteria for the diagnosis of EH and its variants are proposed.

PurposeTo describe the concept, features and mechanisms of epileptic headache (EH).MethodsAnalysis of all published articles concerning EH and related subjects.ResultsThere 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.ConclusionsEH 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.