Ictal headache: Headache as first ictal symptom in focal epilepsy

Headache may be associated with seizures as a preictal, ictal, or postictal phenomenon, but it is often neglected because of the dramatic neurological manifestations of the seizure. Headache can also be the sole or predominant clinical manifestation of epileptic seizures, although this is a relatively rare condition. We describe two cases of focal symptomatic drug-resistant epilepsy with headache as the first ictal symptom. In both cases, the headache, which lasted a few seconds, was contralateral to the ictal discharge and did not have the clinical features of migraine. Ictal headache is a rare epilepsy symptom that can help to localize ictal EEG discharges. Recently, the term ictal epileptic headache has been proposed in cases in which headache is the sole ictal epileptic manifestation Diagnosis requires the simultaneous onset of headache with EEG-demonstrated ictal discharges.     

Epileptic seizures and headache/migraine: A review of types of association and terminology

PurposeThere are different possible temporal associations between epileptic seizures and headache attacks which have given rise to unclear or controversial terminologies. The classification of the International League Against Epilepsy does not refer to this type of disorder, while the International Classification of Headache Disorders (ICHD-2) defines three kinds of association: 1. migraine-triggered seizure (“migralepsy”), 2. hemicrania epileptica, and 3. post-ictal headache.MethodsWe performed an extensive review of the literature, not including “post-ictal” and “inter-ictal” headaches.ResultsOn the basis of well-documented reports, the following clinical entities may be identified: (A) “epileptic headache (EH)” or “ictal epileptic headache (IEH)”: in this condition headache (with or without migrainous features) is an epileptic manifestation per se, with onset, and cessation if isolated, coinciding with the scalp or deep EEG pattern of an epileptic seizure. EH maybe followed by other epileptic manifestations (motor/sensory/autonomic); this condition should be differentiated from “pure” or “isolated” EH, in which headache/migraine is the sole epileptic manifestation (requiring differential diagnosis from other headache forms). “Hemicrania epileptica” (if confirmed) is a very rare variant of EH, characterized by ipsilateral location of headache and ictal EEG paroxysms. (B) “Pre-ictal migraine” and “pre-ictal headache”: when a headache attack is followed during, or shortly after, by a typical epileptic seizure. The migraine attack may be with or without aura, and its seizure-triggering role (“migraine-triggered seizure”) is still a subject of debate. A differentiation from occipital epilepsy is mandatory. The term “migralepsy” has not been used uniformly, and may therefore led to misinterpretation.ConclusionsOn the basis of this review we suggest definitions and a terminology which may become the basis of a forthcoming classification of headaches associated with epileptic seizures.     

Ictal epileptic headache as "subtle" symptom in generalized idiopathic epilepsy

Epilepsy and migraine are common neurologic chronic disorders with episodic manifestations characterized by recurrent attacks and a return to baseline conditions between attacks. Epilepsy and migraine are frequently observed in comorbidity, with the occurrence of one disorder increasing the probability of the other: Migraine occurs in about one-fourth of patients with epilepsy, whereas epilepsy is present in 8-15% of patients with migraine. The link between headache and seizures is controversial and multifactorial. In epilepsy, headache can be seen as a preictal, ictal, or postictal phenomenon. In this report, we describe a case of a 37-year-old patient, affected by both drug-resistant generalized idiopathic epilepsy and headache, who displayed the sudden onset of a headache attack referred during a 24-h electroencephalography (EEG). The EEG tracing during this event revealed the activation of subcontinuous epileptic activity consisting of generalized spike-wave discharges (GSWDs) and generalized polyspike and wave discharges (GPSWDs) that persisted for 60 min, that is, until the disappearance of the headache. The case we describe appears to be original in that it represents one of the few EEG-documented ictal epileptic headaches in generalized idiopathic epilepsy.     

From migralepsy to ictal epileptic headache: the story so far

In the last few years several cases of headache as sole manifestation of an epileptic seizure have been reported and the term “ictal epileptic headache” has been recently proposed to identify an EEG-recorded epileptic seizure with migraine/headache-like features. Among the potential practical implications arising from these clinical observations, there is the urgent need for a revision of both International Classifications of Epilepsy and Headache disorders. We discuss these topics and provide additional comments about the physiopathological links between epilepsy and migraine.     

Seizure-associated headache in epilepsy

PURPOSE: Headache is often ignored as a symptom of epileptic seizures. The purpose of this prospective study was to analyze frequency, classification, and characteristics of seizure-associated headache (SH) according to the criteria of the International Headache Society. METHODS: Over a period of 15 months, 341 patients with epilepsy, consecutively evaluated at our outpatient clinic for SH, completed a standardized questionnaire. RESULTS: Of the 341 epilepsy patients, 115 (34%) experienced SH with a pain intensity of 6.1 +/- 1.6 (SD) on the visual analogue scale and a duration of 12.8 +/- 15.7 (SD) h. Seizures were always accompanied by headache in 69 (60%) of these 115 patients. SH occurred in four (3%) of 115 patients only preictally, in 31 (27%) of 115 patients periictally, and in 80 (70%) of 115 patients only postictally. In the majority of the 115 patients (55.7%), SH could be classified as migraine headache, whereas in 36.5%, as tension-type headache. The type of SH was not correlated with sex, an epilepsy syndrome, or a seizure type. Migraine-like SH was significantly associated with a history of migraine (p < 0.001). In 20 (77%) of the 26 patients experiencing migraine-like SH with a history of migraine, the phenomenology of migraine-like SH and migraine attacks was identical. CONCLUSIONS: SH is a frequent, long-lasting, and severe symptom of epileptic seizures, causing major impairment of daily living. A history of migraine significantly increases the risk for developing migraine-like SH.     

Relationships between migraine and epilepsy: Pathophysiological mechanisms and clinical implications

Migraine and epilepsy are distinct neurological diseases with specific clinical features and underlying pathophysiological mechanisms. However, numerous studies have highlighted the complex and multifaceted relationships between the two conditions. The relationships between headache and epilepsy manifest themselves in different ways. Firstly, the clinical diagnosis of these disorders may be challenging in view of possible overlapping. While post-ictal headache is a frequent condition, ictal epileptic headache is a rare but challenging diagnosis. Both situations raise the question of the pathophysiological mechanism of headache triggered by seizures. Migraine aura and epilepsy can also exhibit overlapping symptoms leading to their misdiagnosis, in particular in the case of visual aura. Secondly, migraine with aura and epilepsy can occur as a co-morbid condition, particularly in familial hemiplegic migraine (FHM). From a pathophysiological perspective, the identification of genetic mutations in FHM has brought significant advances in the understanding of dysfunctions of neuronal networks leading to hyperexcitability. The purpose of this review is to present clinical situations encompassing headache and epilepsy that can be challenging in neurological practice and to discuss the underlying pathophysiological mechanism of such interactions.     

Should "migralepsy" be considered an obsolete concept? A multicenter retrospective clinical/EEG study and review of the literature

The few reports that have been published on the current International Classification of Headache Disorders, Second Edition (ICHD-II), criteria for migralepsy and hemicrania epileptica have highlighted the considerable confusion regarding this "hot topic" within both headache and epilepsy classifications (ICHD-II and International League Against Epilepsy [ILAE]). Indeed, the ICHD-II describes a migraine-triggered seizure as a rare event in which a seizure occurs during migraine aura; on the other hand, hemicrania epileptica is described as an "ictal headache" that occurs "synchronously" with a partial seizure. To confuse matters even further, neither the term migralepsy nor the term hemicrania epileptica is included in the currently used ILAE classification. On the basis of both a review of "migralepsy" cases in the literature and 16 additional retrospective multicenter cases, we suggest that the term migraine-triggered seizure or migralepsy be deleted from the ICHD-II classification until unequivocal evidence is provided of its existence, and that the term ictal epileptic headache be introduced into the ILAE classification.     

Ictal yawning in a patient with drug-resistant focal epilepsy: video/EEG documentation and review of literature reports

Yawning is an involuntary sequence of mouth opening, deep inspiration, brief apnea, and slow expiration. Few cases of yawning as a clinical sign of epileptic seizures, ictally or postictally, have been reported. We report the video/EEG documentation of yawning as an ictal sign in a 31-year-old patient affected by drug-resistant focal epilepsy symptomatic of bilateral perisylvian polymicrogyria. Since the age of 10 she has had seizures characterized by yawning, staring, and eye blinking. Bilateral rhythmic frontotemporal spikes and waves characterized her EEG. We reviewed all reported cases and compared clinical and EEG features. We believe that yawning as part of an epileptic seizure might be considered a rare automatic behavior, like other automatisms frequently reported in epileptic seizures. Automatisms are more frequently described in patients with temporal lobe epilepsy, and involvement of the temporal lobe in most of the published cases may have led to this explanation. It is possible that yawning within epileptic seizures could be considered activation of distinct symptomatogenic cortex rather than a release phenomenon. This rare ictal manifestation should be recognized as epileptic to avoid misdiagnosis and treatment failure.     

Ictal epileptic headache in adult life: Electroclinical patterns and spectrum of related syndromes

ObjectivesBoth headache and epilepsy are frequent paroxysmal disorders that often co-occur or are related in numerous ways. Although ictal epileptic headache has become the focus of several studies, this remains a very rare and not well-known phenomenon. Electroclinical features, pathophysiology, and syndromic context are heterogeneous. We investigated the electroclinical and neuroimaging findings in a population of adult patients with ictal epileptic headache.MethodsWe retrospectively examined 8800 EEG recordings of almost 4800 patients admitted to our video-EEG laboratory from 2010 to 2013 with a history of well-documented epilepsy. We selected patients who reported headache closely related to a seizure documented by video-EEG or 24-hour ambulatory EEG. We analyzed ictal electroclinical features of headache, and we defined the related epileptic syndromes.ResultsWe identified five patients with ictal epileptic headache. Two patients described tension headache during an epileptic seizure. In three patients, the headache was accompanied by other “minor” neurological symptoms mimicking a migrainous aura. In all cases, the headache stopped with the end of the epileptic activity. Three patients had a history of partial symptomatic epilepsy with cerebral lesions (low grade glioma, astrocytoma, porencephalic cyst) in the left posterior regions, whereas two patients were affected by idiopathic generalized epilepsy.ConclusionThis study confirms the rarity of ictal epileptic headache. To date, well-documented video-EEG cases remain as exceptional reports, especially in cases of idiopathic generalized epilepsies. Moreover, we confirm the main involvement of posterior regions in patients with ictal epileptic headache affected by partial symptomatic epilepsies.     

Hyperorality in epileptic seizures: periictal incomplete Klüver-Bucy syndrome

PURPOSE: To analyze systematically hyperorality associated with epileptic seizures and its relation to the localization of epileptic activity. METHODS: To identify patients with periictal hyperorality, we reviewed video-recordings of 269 patients (aged 6-59 years) who had consecutively undergone presurgical evaluations including ictal video-EEG recordings and high-resolution magnetic resonance imaging (MRI) and had had epilepsy surgery because of intractable frontal (FLE) or temporal lobe epilepsy (TLE). Periictal hyperorality was defined if patients put or unambiguously intended to put nonfood items into their mouths during or after at least one of the reviewed seizures. For the further analysis, we included only patients with periictal hyperorality. We reviewed their medical records and reexamined their ictal video-EEG recordings. RESULTS: We identified eight patients (six women) aged 8-59 years who had hyperorality during or after seizures. Seven patients had TLE, and one patient had frontal lobe epilepsy (FLE). Three of these patients underwent right-sided surgery, whereas five patients had surgery on the left. Three patients exhibited ictal and five showed postictal hyperorality. Interictal EEG suggested bilateral interictal epileptiform discharges (IEDs) in three patients; in two other patients, no IEDs were detected. Ictal EEG suggested bilateral involvement in six cases. Patients with unilateral epileptiform activity had left TLE. CONCLUSIONS: Periictal hyperorality is a rare phenomenon occurring in 3% of the investigated epilepsy population. We suggest that periictal hyperorality is an ictal-postictal mental disturbance, an incomplete Klüver-Bucy syndrome. In most patients, bilateral seizure activity plays an important role in the pathomechanism, but it would appear that left-sided epileptic activity without contralateral involvement also can cause periictal hyperorality.     

Ictal electroencephalographic findings of neonatal seizures in preterm infants

The aim of this study is to clarify the characteristics of ictal EEG findings of neonatal seizures in preterm infants. Seizures associated with ictal EEG changes were recognized in nine infants with gestational age of less than 37 weeks. Propagation, migration, shifting, changes in morphology of ictal EEG discharges were evaluated. Seizure manifestation was divided into the following categories; motor seizure, apneic seizure, automatic seizure and seizure without clinical symptoms. The types of the seizures were motor seizures in five infants, apneic in two, automatic in one and those without clinical symptoms in five. All seizures were of focal onset. The foci of seizures were temporal in six infants, occipital in two, central in one, and frontal in one. The morphology of ictal discharges was low voltage spikes or sharp waves in six infants, spikes in two, theta waves in one and high-voltage spiky theta in one. The propagation of ictal discharges was focal in five infants and regional in five. The migration of ictal discharges was observed in two infants and a shift in two. There was no clear relation between seizure manifestation and ictal EEG foci, duration of seizures and morphology or propagation of ictal discharges.     

A case of infantile spasms: Epileptic apnea as partial seizures at onset

We report a 2-month-old boy who presented with apneic attacks as a manifestation of epileptic seizures at onset and eventually progressed to infantile spasms. At onset, at 2 months of age, apneic attacks were the sole symptom of epileptic fits. Although these seizures were accompanied by cyanosis, bradycardia was not noted. An ictal electroencephalogram showed focal paroxysmal discharges in the temporal area. Treatment with sodium valproate was not effective to control his seizures. By 6 months of age, he progressed to infantile spasms. Although his seizures could be completely controlled with the use of zonisamide, vitamin B6 or high-dose immunoglobulin, his mental and behavioral development was retarded severely. There have been no previously published cases with infantile spasms that evolved from epileptic apnea as partial seizures.     

儿童头痛性癫痫诊断问题的探讨

目的探讨头痛性癫痫的诊断及与偏头痛的鉴别诊断。方法分析由院外诊断为头痛性癫痫的１０２例患者的临床资料,详细询问病史,进行仔细的体格检查,复查ＥＥＧ１～３次后,重新确定诊断,实施治疗并进行临床随访。结果１０２例患者中,６８例为偏头痛,３４例为神经性头痛。复查ＥＥＧ结果显示大部分在正常范围,部分异常ＥＥＧ中以弥散性慢波增多为主,仅有３例ＥＥＧ显示有散在癫痫样波,但临床表现却符合典型偏头痛的诊断。结论头痛性癫痫临床少见,单纯以头痛诊断为癫痫是不可靠的,必须依靠临床表现、ＥＥＧ检查结果综合判断,并和偏头痛进行鉴别。以头痛为主诉的儿童不应轻易诊断为癫痫。     

The complex interrelations between two paroxysmal disorders: headache and epilepsy

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.     

Clinical factors associated with postictal headache in Chinese patients with partial epilepsy

PurposeTo investigate the incidence of postictal headache (PIH) and the factors potentially related to the occurrence of PIH in a Chinese epileptic center.MethodsConsecutive adult patients with epilepsy, referred to the outpatient clinic of the Epilepsy Center of the PLA General Hospital between February 01, 2012, and May 10, 2013, were recruited to this study. 854 patients with partial epilepsy completed a questionnaire regarding headache, 466 patients with temporal lobe epilepsy (TLE), 82 patients with occipital lobe epilepsy (OLE) and 306 patients with frontal lobe epilepsy (FLE). A semi-structured interview was performed in those who confirmed headache.ResultsPIH occurred in 328 (38.41%) of the subjects. By type of epilepsy, PIH was found in 164 (35.19%) of the patients with TLE, 46 (56.01%) of the patients with OLE, and 118 (38.56%) of the patients with FLE. The incidence of PIH in OLE was significantly higher than in TLE and FLE (P < 0.05). It occurs more frequently after generalized tonic–clonic seizures than other seizure types. Logistic regression analysis revealed that age at onset, type of seizure and classification of epilepsy were each significantly related to the occurrence of PIH.ConclusionThe results of our study revealed possible relationships between PIH and the region of epileptic focus and area of spread of epileptic discharges.     

儿童非癫痫性发作

目的 :探讨儿童非癫痫性发作的各种临床表现形式。方法 :对 35例非癫痫性发作患儿的临床结合脑电图监测进行分析。结果 :35例儿童非癫痫发作中 ,生理性发作占 5 1% ,且平均年龄最小 ,全部患儿经 2 4小时脑电图或录相监测脑电图检查发作间期和 /或发作期均无异常放电 ;本组误诊 2 1例 ,其中癫痫 10例。结论 :儿童的非癫痫性发作表现形式多样 ,以生理性发作最多。儿童非癫痫性发作易误诊为癫痫及其它疾病 ,应用动态脑电图监测对其诊断及鉴别诊断有重要意义。     

Prolonged ictal aphasia: A diagnosis to consider

Aphasia is a common symptom encountered by clinical neurologists. It is usually caused by strokes or lesions involving language regions of the brain, yet prolonged aphasia is rarely the sole manifestation of a simple partial status epilepticus. We report six patients, who suffered from prolonged ictal aphasia. All but one patient had a structural lesion in the left hemisphere, only three suffered from clinical seizures during or shortly prior to the aphasic episode. All patients had ictal patterns on the electroencephalogram (EEG), four of whom had periodic lateralized epileptiform discharges, and five showed frequent recurrent electrographic seizures during the aphasic state. The aphasia lasted several days in all patients, and it resolved after administration of antiepileptic drug treatment. In conclusion, prolonged ictal aphasia is a rare but important treatable cause of aphasia. Surface EEG recordings should be obtained in all patients with unexplained prolonged aphasia to diagnose this rare but treatable entity.     

Five pediatric cases of ictal fear with variable outcomes

Purpose: Ictal fear is an uncommon condition in which fear manifests as the main feature of epileptic seizures. The literature has suggested that ictal fear is generally associated with poor seizure outcomes. We wanted to clarify the variability in seizure outcome of children with ictal fear. Subjects and methods: We identified five pediatric patients with ictal fear who were followed up on at Okayama University Hospital between January 2003 and December 2012. We retrospectively reviewed their clinical records and EEG findings. Results: The onset age of epilepsy ranged from 8 months to 9 years and 10 months. The common ictal symptoms were sudden fright, clinging to someone nearby, and subsequent impairment of consciousness, which were often accompanied by complex visual hallucinations and psychosis-like complaints. Ictal fear, in four patients, was perceived as a nonepileptic disorder by their parents. Ictal electroencephalograms (EEG) of ictal fear were obtained in all patients. Three showed frontal onset, while the other two showed centrotemporal or occipital onsets. Two patients were seizure free at last follow-up, while seizures persisted in the other three. A patient with seizure onset during infancy had a favorable outcome, which was considered to be compatible with benign partial epilepsy with affective symptoms. Conclusion: Ictal fear is not always associated with a symptomatic cause or a poor seizure outcome. It is quite important to make a correct diagnosis of ictal fear as early as possible to optimize treatment.     

Characteristics of headache in children with epilepsy

PurposeThe association of headache with seizures is well known to neurologists but poorly understood. The purpose of this prospective study was to evaluate the types and frequency of seizure-associated headaches among pediatric patients with epilepsy, and to identify their risk factors with special attention to the anatomic localization of the epileptogenic focus and seizure classification.MethodsPatients with focal and generalized active seizures and on treatment at the time of questionnaire administration were included. Patients were prospectively interviewed by questionnaire as to whether or not they suffered from headaches associated with epileptic seizures.ResultsOf 98 patients (age range: 5–18 years), 34 (34.7%) complained of seizure-associated headaches. In patients with seizure-associated headaches, headache was significantly more frequent (31/74, 41.9%) with partial seizures than with generalized seizures (3/24, 12.5%; p = 0.012). The frequency of seizure attacks was 4.1 times per year in patients with seizure-associated headache and 1.3 times per year in those with non-seizure-associated headache. Of the 34 patients, 20 (58.8%) complained of headache at the frontal region.ConclusionsThe location of headache was not always in agreement with electroencephalographic focus. Headache was more frequent in patients with partial epilepsy and frequent seizures.     

Interictal and ictal EEG activity in the basal ganglia: an SEEG study in patients with temporal lobe epilepsy

PURPOSE: The interictal and ictal EEG activity in the basal ganglia in patients with temporal lobe epilepsy were studied during invasive EEG monitoring. METHODS: Eight epilepsy surgery candidates, each with a proven mesiotemporal seizure-onset zone, participated in the study. We used two invasive EEG methods to determine the seizure-onset zone. In both methods, diagonal electrodes were targeted into the amygdalohippocampal complex via a frontal approach and were passed through the basal ganglia with several leads. We analyzed 16 partial epileptic seizures, four of which became secondarily generalized. RESULTS: No epileptic interictal or ictal discharges were noticed in the basal ganglia. The interictal activity in the basal ganglia was a mixture of low-voltage beta activity and medium-voltage alpha-theta activity. When the ictal paroxysmal activity remained localized to the seizure-onset zone, the activity of the basal ganglia did not change. The spread of epileptic activity to other cortical structures was associated with the basal ganglia EEG slowing to a theta-delta range of 3-7 Hz. This slowing was dependent on the spread of ictal discharge within the ipsilateral temporal lobe (related to the investigated basal ganglia structures); alternatively, the slowing occurred in association with the regional spread of ictal activity from the mesiotemporal region to the temporal neocortex contralaterally to the investigated basal ganglia. Secondary generalization was associated with a further slowing of basal ganglia activity. CONCLUSIONS: The basal ganglia do not generate specific epileptic EEG activity. Despite the absence of spikes, the basal ganglia participate in changing or reflect changes in the distribution of the ictal epileptic activity.