ISSN 0017-8748 doi: 10.1111/head.12542 Published by Wiley Periodicals, Inc.
Headache © 2015 American Headache Society
Comment Observation and Rebuttals Epileptic Headache: Need for a Better Knowledge
In their article, Saitowitz et al1 attempt to characterize headaches of an epileptic nature. It is not entirely possible, however, to derive from their paper a correct definition of the disorder or indeed ascertain its clinical characterization. For example, the “cardinal manifestations” (shown in Table 1 of their paper) do not fit with the characteristics of the roughly 15 patients as presently reported in other reviews.2,3 From these patients, it appears clearly that “confusion, incontinence and nocturnal occurrence” as well as “visual aura,” as defined by Saitowitz et al as cardinal manifestations, are very rare and absolutely not characterizing signs. Apart from the use of somewhat redundant terminology “ictal epileptic headache” (an “epileptic headache” is necessarily “ictal,” as is every epileptic phenomenon; therefore the term “ictal” is redundant), Saitowitz and colleagues do not delineate this cephalic pain manifestation due to an epileptic event in their main characteristics. These main characteristics are simple and clear: “a headache of any type, with onset and (if not followed by other epileptic manifestations) with cessation coinciding with a (scalp or deep) EEG pattern of epileptic seizure”2 (a video-EEG example has been published4). The confused conceptualization of epileptic headache is also evident in Table 2, where the authors cite Panayiotopoulos syndrome (PS) and frontal lobe epilepsy as conditions in which ictal headache is present. Yet there is no report in the literature of an epileptic headache in these conditions, both in PS5 and in the different expressions of epilepsies originating in the frontal lobe, although a peri-ictal (pre- or post-ictal) headache may be present. Moreover, we believe Saitowski and colleagues may not be suffi-
ciently aware of the mechanisms of these kind of manifestations when, in the section of their paper labeled Pathophysiology, they state: “. . . headache . . . as a reaction to focal discharges . . . CSD, leading to trigeminovascular activation and subsequent headache.” Epileptic headache is not a “reaction,” and does not depend on trigeminovascular activation. Like every epileptic phenomenon it depends on the cortical activation, that in this specific condition is one of the areas involved in pain, which are present in multiple cortical sites. Non-epileptic headaches have a different mechanism, such as migraines being triggered by cortical spreading depression. The multiple cortical pain sites explain the variability of the ictal EEG foci in the cases of epileptic headache, and are connected to the different etiologies:2 malformations, gliotic scars, flogoses, etc, as well as genetic and/or epigenetic mechanisms, as in every other epileptic event. Saitowitz et al1 take their cue to treat the topic from the cases of two patients, the first of which is a 7-year-old girl with recurrent episodes of “awakening at night, associated with pallor, nausea, vomiting, and headache,” without vigilance impairment, gaze deviation or motor symptoms. Their diagnosis of epilepsy has been entertained on the basis of an EEG “abnormal with L occipital high amplitude spike and slow wave discharges,” without any recording of an ictal EEG discharge (they should probably take into account that epileptiform EEG abnormalities are detectable in 10% of children without epilepsy). It is difficult to support their assertion, therefore, that this headache is epileptic and not post-ictal. Only the occurrence of headache during EEG abnormalities compatible with an epileptic seizure could determine 580
Headache if the headache is epileptic. As already noted, this occurrence has never been reported in ictal EEG registrations of patients having PS.5 It is unclear why Saitowitz et al1 discuss the “benign occipital epilepsy-Gastaut type” (incidentally, the ILAE’s correct terminology is “Late onset childhood occipital epilepsy [Gastaut-type]”). This is a disorder characterized by the presence of visual symptoms that, however, they do not report in either of their two patients highlighted. In this disorder ictal headache may be present, although very rare; meanwhile post-ictal headache is frequent (about 1/3 of cases). On the other hand, their second patient appears to really represent a case of epileptic headache: in fact he had an EEG record during a headache attack, showing “manifestations of an L frontal lobe seizure, with buildup of spike and slow waves to rhythmic fast beta activity overlying the L frontal region, lasting for 1 minute, and followed by L frontal delta slowing.” This is what is required and sufficient for a diagnosis of epileptic headache, although the authors do not stress this, instead choosing to overstate the importance of a differential diagnosis with primary stabbing headache, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA), disorders that instead can be immediately excluded since they not include epileptic abnormalities on the ictal EEG. From the clinical point of view, the interest of their case 2 is that it belongs to the isolated form of epileptic headache (“Pure epileptic headache”4). In fact, diagnosis was made only after an ictal EEG, and this led to the correct therapy. There is obviously no diagnostic uncertainty when epileptic headaches are accompanied by other epileptic manifestations.2,4 Epileptic headache is not included in the proposed International Classification of Headache Disorders, 3rd edition beta version,6 and this omission needs to be rectified since the disorder is probably not
581 as rare as it seems from the few cases reported in the literature. In fact, it is surprising that the majority of cases have been published in the last 5 years, moreover all by Italian epileptologist. This suggests the need for greater awareness of the disorder by headache experts. Carlo Cianchetti, MD1; Federico Vigevano, MD2; Vincenzo Guidetti, MD3 1 University of Cagliari, Cagliari, Italy 2 Neuroscience Department, Bambino Gesù Children’s Hospital, Rome, Italy 3 Child and Adolescent Neuropsychiatry, Sapienza University of Rome, Rome, Italy email: [email protected]
REFERENCES 1. Saitowitz Z, Flamini R, Berenson F. Ictal epileptic headache: A review of current literature and differentiation from migralepsy and other epilepsies. Headache. 2014;54:1534-1540. 2. Cianchetti C, Pruna D, Ledda M. Epileptic seizures and headache/migraine: A review of types of association and terminology. Seizure. 2013;22:679-685. 3. Parisi P, Striano P, Verrotti A, Villa MP, Belcastro V. What have we learned about ictal epileptic headache? A review of well-documented cases. Seizure. 2013;22:253-258. 4. Cianchetti C, Pruna D, Porcu L, Peltz MT, Ledda MG. Pure epileptic headache and related manifestations: A video-EEG report and discussion of terminology. Epileptic Disord. 2013;15:84-92. 5. Specchio N, Trivisano M, Claps D, Battaglia D, Fusco L, Vigevano F. Documentation of autonomic seizures and autonomic status epilepticus with ictal EEG in Panayiotopoulos syndrome. Epilepsy Behav. 2010;19: 383-393. 6. Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013;33:629-808.
Copyright of Headache: The Journal of Head & Face Pain is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Headache © 2015 American Headache Society
Comment Observation and Rebuttals Epileptic Headache: Need for a Better Knowledge
In their article, Saitowitz et al1 attempt to characterize headaches of an epileptic nature. It is not entirely possible, however, to derive from their paper a correct definition of the disorder or indeed ascertain its clinical characterization. For example, the “cardinal manifestations” (shown in Table 1 of their paper) do not fit with the characteristics of the roughly 15 patients as presently reported in other reviews.2,3 From these patients, it appears clearly that “confusion, incontinence and nocturnal occurrence” as well as “visual aura,” as defined by Saitowitz et al as cardinal manifestations, are very rare and absolutely not characterizing signs. Apart from the use of somewhat redundant terminology “ictal epileptic headache” (an “epileptic headache” is necessarily “ictal,” as is every epileptic phenomenon; therefore the term “ictal” is redundant), Saitowitz and colleagues do not delineate this cephalic pain manifestation due to an epileptic event in their main characteristics. These main characteristics are simple and clear: “a headache of any type, with onset and (if not followed by other epileptic manifestations) with cessation coinciding with a (scalp or deep) EEG pattern of epileptic seizure”2 (a video-EEG example has been published4). The confused conceptualization of epileptic headache is also evident in Table 2, where the authors cite Panayiotopoulos syndrome (PS) and frontal lobe epilepsy as conditions in which ictal headache is present. Yet there is no report in the literature of an epileptic headache in these conditions, both in PS5 and in the different expressions of epilepsies originating in the frontal lobe, although a peri-ictal (pre- or post-ictal) headache may be present. Moreover, we believe Saitowski and colleagues may not be suffi-
ciently aware of the mechanisms of these kind of manifestations when, in the section of their paper labeled Pathophysiology, they state: “. . . headache . . . as a reaction to focal discharges . . . CSD, leading to trigeminovascular activation and subsequent headache.” Epileptic headache is not a “reaction,” and does not depend on trigeminovascular activation. Like every epileptic phenomenon it depends on the cortical activation, that in this specific condition is one of the areas involved in pain, which are present in multiple cortical sites. Non-epileptic headaches have a different mechanism, such as migraines being triggered by cortical spreading depression. The multiple cortical pain sites explain the variability of the ictal EEG foci in the cases of epileptic headache, and are connected to the different etiologies:2 malformations, gliotic scars, flogoses, etc, as well as genetic and/or epigenetic mechanisms, as in every other epileptic event. Saitowitz et al1 take their cue to treat the topic from the cases of two patients, the first of which is a 7-year-old girl with recurrent episodes of “awakening at night, associated with pallor, nausea, vomiting, and headache,” without vigilance impairment, gaze deviation or motor symptoms. Their diagnosis of epilepsy has been entertained on the basis of an EEG “abnormal with L occipital high amplitude spike and slow wave discharges,” without any recording of an ictal EEG discharge (they should probably take into account that epileptiform EEG abnormalities are detectable in 10% of children without epilepsy). It is difficult to support their assertion, therefore, that this headache is epileptic and not post-ictal. Only the occurrence of headache during EEG abnormalities compatible with an epileptic seizure could determine 580
Headache if the headache is epileptic. As already noted, this occurrence has never been reported in ictal EEG registrations of patients having PS.5 It is unclear why Saitowitz et al1 discuss the “benign occipital epilepsy-Gastaut type” (incidentally, the ILAE’s correct terminology is “Late onset childhood occipital epilepsy [Gastaut-type]”). This is a disorder characterized by the presence of visual symptoms that, however, they do not report in either of their two patients highlighted. In this disorder ictal headache may be present, although very rare; meanwhile post-ictal headache is frequent (about 1/3 of cases). On the other hand, their second patient appears to really represent a case of epileptic headache: in fact he had an EEG record during a headache attack, showing “manifestations of an L frontal lobe seizure, with buildup of spike and slow waves to rhythmic fast beta activity overlying the L frontal region, lasting for 1 minute, and followed by L frontal delta slowing.” This is what is required and sufficient for a diagnosis of epileptic headache, although the authors do not stress this, instead choosing to overstate the importance of a differential diagnosis with primary stabbing headache, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA), disorders that instead can be immediately excluded since they not include epileptic abnormalities on the ictal EEG. From the clinical point of view, the interest of their case 2 is that it belongs to the isolated form of epileptic headache (“Pure epileptic headache”4). In fact, diagnosis was made only after an ictal EEG, and this led to the correct therapy. There is obviously no diagnostic uncertainty when epileptic headaches are accompanied by other epileptic manifestations.2,4 Epileptic headache is not included in the proposed International Classification of Headache Disorders, 3rd edition beta version,6 and this omission needs to be rectified since the disorder is probably not
581 as rare as it seems from the few cases reported in the literature. In fact, it is surprising that the majority of cases have been published in the last 5 years, moreover all by Italian epileptologist. This suggests the need for greater awareness of the disorder by headache experts. Carlo Cianchetti, MD1; Federico Vigevano, MD2; Vincenzo Guidetti, MD3 1 University of Cagliari, Cagliari, Italy 2 Neuroscience Department, Bambino Gesù Children’s Hospital, Rome, Italy 3 Child and Adolescent Neuropsychiatry, Sapienza University of Rome, Rome, Italy email: [email protected]
REFERENCES 1. Saitowitz Z, Flamini R, Berenson F. Ictal epileptic headache: A review of current literature and differentiation from migralepsy and other epilepsies. Headache. 2014;54:1534-1540. 2. Cianchetti C, Pruna D, Ledda M. Epileptic seizures and headache/migraine: A review of types of association and terminology. Seizure. 2013;22:679-685. 3. Parisi P, Striano P, Verrotti A, Villa MP, Belcastro V. What have we learned about ictal epileptic headache? A review of well-documented cases. Seizure. 2013;22:253-258. 4. Cianchetti C, Pruna D, Porcu L, Peltz MT, Ledda MG. Pure epileptic headache and related manifestations: A video-EEG report and discussion of terminology. Epileptic Disord. 2013;15:84-92. 5. Specchio N, Trivisano M, Claps D, Battaglia D, Fusco L, Vigevano F. Documentation of autonomic seizures and autonomic status epilepticus with ictal EEG in Panayiotopoulos syndrome. Epilepsy Behav. 2010;19: 383-393. 6. Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013;33:629-808.
Copyright of Headache: The Journal of Head & Face Pain is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
