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The complex interrelations between two paroxysmal disorders: headache and epilepsy

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Abstract

The interrelations between headache/migraine and epileptic seizures are an interesting topic, still lacking a systematization, which is the objective of the present revision. We organize the general setting on: (a) a distinction between pre-ictal, ictal, post-ictal and inter-ictal headaches, assuming “ictal” as epileptic seizure, and (b) the kind of headache, if it is of migraine type or not. Concerning pre-ictal migraine/headache, the necessity of its differentiation from an epileptic headache presenting as an aura of a seizure is stressed; this is connected with the indefiniteness of the term “migralepsy”. The term “migraine aura-triggered seizure” should be used only in front of a proven triggering effect of migraine. Epileptic headache (called also “ictal epileptic headache”) is a well-characterized entity, in which different types of head pain may occur and an ictal EEG is necessary for the diagnosis. It may present as an isolated event (“isolated epileptic headache”), requiring a differential diagnosis from other kinds of headache, or it may be uninterruptedly followed by other epileptic manifestations being in this case easily identifiable as an epileptic aura. Hemicrania epileptica is a very rare variant of epileptic headache, characterized by the ipsilaterality of head pain and EEG paroxysms. Ictal non-epileptic headache needs to be differentiated from epileptic headache. Post-ictal headaches are a frequent association of headache with seizures, particularly in patients suffering also from inter-ictal headache-migraine. The reported systematization of the topic led us to suggest a classification which is shown in Appendix.

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Authors and Affiliations

  1. Former Professor of Child and Adolescent Neurology and Psychiatry, University of Cagliari, Cagliari, Italy

    Carlo Cianchetti

  2. Department of Neurophysiology and Epilepsy Unit, Besta Neurological Institute, Milan, Italy

    Giuliano Avanzini

  3. Neurologic Unit, Epilepsy Center, SS.Giovanni e Paolo Hospital, Venice, Italy

    Filippo Dainese

  4. Child and Adolescent Neuropsychiatry, Sapienza University, Rome, Italy

    Vincenzo Guidetti

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  1. Carlo Cianchetti
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  2. Giuliano Avanzini
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  3. Filippo Dainese
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  4. Vincenzo Guidetti
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Correspondence to Carlo Cianchetti.

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Appendices

Appendix

Suggestions for a classification of the possible relationships between headache/migraine and epilepsy.

Headache and epileptic seizures

Headache may precede, be contextual, or follow an epileptic seizure, or may occur independent from it. Moreover, headache may be the expression of an epileptic seizure.

Headache preceding an epileptic seizure (pre-ictal headache)

Description An attack of headache or of migraine, with or without aura, during which, or in short time following which (conventionally within 1 h), an epileptic seizure occurs.

Diagnostic criteria Presence of all A, B, C

  1. (A)

    a headache attack of undetermined type, or a migraine attack with or without aura diagnosed according to ICHD-3β criteria.

  2. (B)

    a seizure, fulfilling diagnostic criteria for any type of epileptic attack, occurring during, or within (conventionally) 1 h after cessation of, the headache attack.

  3. (C)

    headache as an epileptic manifestation has been excluded.

Comment Headache and epilepsy are both paroxysmal brain disorders. Migraine with visual aura and occipital epileptic seizures share a common site of origin with probably partially similar mechanisms. Seizures beginning with visual symptoms and migraine-like attack, immediately followed by other epileptic manifestations, may occur in occipital epilepsy, including symptomatic cases. In this condition, the migraine-like manifestation is an epileptic phenomenon, part of the epileptic seizure as an initial pain phase of it, that is head pain as an epileptic aura (see “Epileptic headache (EH)”).

The definition “pre-ictal migraine” requires the absence of ictal-epileptic EEG features during aura and migraine in the cases where the phenomena occur in a direct sequence. A time interval following the aura/migraine before the epileptic seizure should exclude an epileptic origin of the sequence also in absence of an EEG record.

Pre-ictal migraine with aura is more frequent than without aura. Reported auras are visual, although rarely also paresthetic.

A triggering effect of migraine, rather than an occasional association, is suggested in cases lacking a history of epilepsy, or of repeated occurrence, although it is generally difficult to establish with certainty; therefore, in most cases the term pre-ictal migraine should be preferred, instead of “migraine aura-triggered seizure”.

The term “migralepsy” was originally used in reference to the condition of visual symptoms followed by migraine and subsequently an epileptic seizure; the term may be applied both to an epileptic migraine evolving with other types of epileptic manifestation, both to a true migraine followed by a seizure. Therefore, it is a term requiring a better and consensual definition.

Ictal headache

Epileptic headache (EH)

Description Headache is a possible manifestation of an epileptic seizure.

It is characterized by head pain (whether migraine-like or not) with onset, and cessation if isolated, coinciding with a scalp EEG pattern consistent with an epileptic seizure (cases are reported in which EEG alterations may only be detectable using intracranial electrodes).

It may present as an isolated manifestation (“pure” or “isolated epileptic headache”), or it may directly precede other manifestations of an epileptic seizure (in this condition, headache more clearly appears as an “aura” of the seizure, an “epileptic aura”). When isolated, it is not distinguishable from other types of headache, and only an EEG during the manifestation can lead to the correct diagnosis.

The immediate pain cessation at the infusion of an antiepileptic therapy helps to confirm the diagnosis.

“Hemicrania epileptica” is a very rare variant of epileptic headache, characterized by ipsilateral location of a hemicranial headache and ictal EEG paroxysms.

Diagnostic criteria:

  1. (A)

    Headache attack of any type fulfilling criterion B.

  2. (B)

    Onset, and cessation if isolated, coinciding with a scalp or intracranial EEG pattern of epileptic seizure.

Variants:

  1. (a)

    Pure or isolated (Isolated Epileptic Headache, IEH), when headache is the only manifestation of a seizure (minor additional manifestations, like nausea, vomit, phono-photophobia, agitation or irritability or hypersensibility to noises or dyspnea, may be present).

  2. (b)

    Epileptic headache followed without discontinuity by other epileptic manifestations, thus headache representing actually the initial manifestation of an epileptic seizure.

Ictal non-epileptic headache

Description Headache occurring during an epileptic seizure, but not being epileptic in nature, therefore not showing strict coincidence with epileptiform EEG features.

The presence of this kind of headache in epidemiological studies is reported with a frequency from 0.5 to 5%. In practice, it can be differentiated from the epileptic headache when ictal headache onset clearly precede and/or continues after cessation of the seizure.

Diagnostic criteria:

  1. (A)

    Headache attack of any type occurring during an epileptic seizure, fulfilling criterion B.

  2. (B)

    Onset and/or cessation not coinciding with the seizure, or not coincidence with the EEG pattern of epileptic seizure.

Post-ictal headache (PIH)

Description The occurrence of headache after an epileptic seizure is frequent, ranging between 12 and 52%.

PIH occurs (conventionally) within 3 h following a seizure, and may last up to 72 h. Headache may have migrainous or tension-type features and is usually of moderate to severe intensity. PIH is more frequent after generalized tonic–clonic, prolonged and repetitive seizures; moreover, it occurs more frequently in subjects suffering from inter-ictal headache.

Diagnostic criteria:

  1. (A)

    Headache of any type fulfilling criterion B.

  2. (B)

    Headache occurring within 3 h after any type of epileptic seizure.

Inter-ictal headache

Description Patients suffering from epilepsy may also suffer from any type of headache, having its occurrence independent of the epileptic seizures, although occasionally near it. This is a comorbid condition, to be kept separated from the above-reported peri-ictal and ictal headaches.

Diagnostic criteria:

  1. (A)

    Headache of any type in a patient suffering from epilepsy.

  2. (B)

    Headache attacks and epileptic seizures occur independently, although headache may be rarely pre-ictal and frequently post-ictal.

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Cianchetti, C., Avanzini, G., Dainese, F. et al. The complex interrelations between two paroxysmal disorders: headache and epilepsy. Neurol Sci 38, 941–948 (2017). https://doi.org/10.1007/s10072-017-2926-5

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