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Occipital lobe epilepsies (OLE) comprise 5–10% of focal epilepsies in surgical and paediatric series; with little data from adult medical cohorts. This longitudinal study examined OLE patients, to characterise prevalence, semiology, co-morbidity and prognosis in a neurology outpatient setting. 24 adult OLE patients identified over 12 months from 1548 patients in a neurologist’s service were followed over 12 years. 92% of these OLE patients had simple visual hallucinations, misdiagnosed in 40% of cases. 75% had co-morbid interictal migraine and 38% had visual field defects. Only 33% achieved long-term remission, and only 2 /10 (20%) of OLE patients with a structural aetiology were seizure-free. The two patients with migralepsy achieved remission. Adult OLE accounted for 7.7% of focal epilepsies in this cohort, misdiagnosed or misclassified in 40%. Most patients had co-existing migraine. A minority had migralepsy characterised by a longer aura and good prognosis. Remission rates were lower than that of childhood OLE and general adult epilepsy populations, strengthening the argument for considering epilepsy surgery in drug-resistant OLE patients with a structural cause. Precision medicine will potentially refine diagnosis and management in those OLE patients without an identified cause but is predicated on accurate clinical phenotyping.

Migraine, epilepsy and stroke are highly prevalent neurological disorders, often comorbid. They share diverse pathophysiological mechanisms that explain the use of similar drugs on certain occasions (i.e., the use of antiepileptic drugs in migraine prevention). Migraine with aura represents a risk for ischemic stroke, and avoiding contraceptives, tobacco use, and ergot alkaloids should be advised in those patients. Epilepsy bears a bidirectional relationship with headache. Only three entities are considered as seizure-related headaches: migraine-triggered seizure (migralepsy), hemicrania epileptica, and post-ictal headache. Topiramate (100-200 mg daily) and valproic acid (500-1,000 mg daily) are first-line drugs in migraine prevention, while older antiepileptics have no use in this setting. Stroke is the most common cause of symptomatic epilepsy in the adult. Therapy with lamotrigine, gabapentine, and levetiracetam is advised in late-onset (2 weeks after stroke) stroke-seizures, while early-onset seizures usually do not require therapy.

The term "ictal epileptic headache" has been recently proposed to classify the clinical picture in which headache is the isolated ictal symptom of a seizure. There is emerging evidence from both basic and clinical neurosciences that cortical spreading depression and an epileptic focus may facilitate each other, although with a different degree of efficiency. This review address the long history which lead to the 'migralepsy' concept to the new emerging pathophysiological aspects, and clinical and electroencephalography evidences of ictal epileptic headache. Here, we review and discuss the common physiopathology mechanisms and the historical aspects underlying the link between headache and epilepsy. Either experimental or clinical measures are required to better understand this latter relationship: the development of animal models, molecular studies defining more precise genotype/phenotype correlations as well as multicenter clinical studies with revision of clinical criteria for headache-/epilepsy-related disorders represent the start of future research. Therefore, the definition of ictal epileptic headache should be used to classify the rare events in which headache is the only manifestation of a seizure. Finally, using our recently published criteria, we will be able to clarify if ictal epileptic headache represents an underestimated phenomenon or not.

Background Headache and epilepsy are two paroxysmal disorders with epidemiological and clinical comorbidity. Among the primary headaches, migraine, and in particular the subtype with aura (MA) has a significant correlation with epilepsy. Like epilepsy MA is a chronic condition characterized by recurrent focal neurological attacks that are variously accompanied by headache and inconstant autonomic features. Interictal electroencephalogram (EEG) findings are usually normal although several abnormalities, especially diffuse slowings, have been reported in migraineurs. EEG is not useful in MA with the exception of situations where seizure disorders are to be excluded. The relationship between headaches and epilepsy is covered in the International Classification of Headache Disorders in chapters 1 and 7 where migraine-triggered seizure (1.4.4), hemicrania epileptica (7.6.1) and post-ictal headache (7.6.2) are collocated. An interictal headache was defined as headache starting not earlier than three hours after a seizure or a headache never proceeding directly into an epileptic fit. Headache caused by an epileptic seizure, occurring during and/or after the seizure and remitting spontaneously within hours or up to 3 days [1,2]. The classification of the International League against Epilepsy does not refer to this type of disorder. In the last decade other terms for this association have been proposed (epileptic headache or ictal epileptic headache) and some Authors have proposed to abandon or modify some terms (hemicrania epileptica and migralepsy) [3-5].

The special relationship between migraine and epilepsy has been recognized for centuries and was formally acknowledged by Gowers in his 1906 lecture "Borderland of Epilepsy." The term migralepsy was introduced by Lennox and Lennox in 1960, with multiple cases described in the literature since that time. In the ensuing years, the relationship between migraine and epilepsy has proven complex. The 2 conditions have been found to be comorbid with each other, suggesting a common underlying mechanism or genetic tendency. Specific diseases with both phenotypes provide further evidence of a common pathophysiology, and as the mechanism of migraine has been further elucidated, commonalities with seizure have been recognized. The terms "hemicrania epileptica" and "migraine triggered seizure" were defined by the International Headache Society, formalizing the concept that one can lead to the other. However, case reports and case series in the literature reveal that distinguishing between the 2 entities can be challenging. The concept of migralepsy is likely to evolve as greater understanding of both conditions is gained.