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Epileptic Disorders - Editor's Choice Archive

The following are past Editor's Choice articles. Free access may not be available.

Disconnective surgery in posterior quadrantic epilepsy: a series of 12 paediatric patients

Epileptic Disorders September 2014

Peng-Fan Yang, Zhen Mei, Qiao Lin, Jia-Sheng Pei, Hui-Jian Zhang, Zhong-Hui Zhong, Jun Tian, Yan-Zeng Jia, Zi-Qian Chen, Zhi-Yong Zheng
Epileptic Disorders. 2014 Sep;16(3):296-304

Commentary by Pr. Douglas Nordli Jr, Associate Editor

From my perspective as a pediatric epileptologist involved in single-stage ECoG-guided resections for the past 25 years one of the most challenging aspects of pediatric temporal lobe surgery for intractable epilepsy due to cortical dysplasia is making sure all of the posterior extent of the lesion has been identified so that the surgeon can achieve a complete resection.

This is complicated because the lesion may sometime extend posteriorly and inferiorly a considerable distance beyond the usual margins of a typical temporal lobe resection necessitating a multilobar resection with encroachment into the parietal and occipital lobes. In the past, with the limited surgical exposures for a standard temporal lobectomy and no navigational guidance systems this was very challenging. Nowadays, in centers all around the world we have learned to expect this extent of the lesion and to anticipate the need for more extensive surgery in these cases.

A residual challenge is the best approach for children who have total or near-total involvement of the temporal parietal and occipital lobes with lesions which by themselves, do not need to be resected. Here, multilobar resections will result in a removal of a large amount of substrate and leave large defects, each with their attendant complications. A functional disconnection of these areas may be an elegant alternative approach, particularly if the patient already has a contralateral visual field defect.

P-F Yang and colleagues describe their experience with 12 children who underwent disconnection of the posterior quadrant for intractable epilepsy and highlight the feasibility of this approach. For the most part, these children had concordant clinical neurophysiological, MR and PET pre-operative results implicating the posterior quadrant. None had markedly discordant findings and none had lesions that would require resection for proper management, such as tumors or vascular malformations. Their surgical approach allowed for small incisions with minimal blood loss. Once they mastered the technique of sparing bridging vessels there were no significant unanticipated complications. All children had expected contralateral field defects, as expected. The outcomes at an average of 34 months were favorable with 75% of patients being seizure-free. Comparison of pre- and post-operative neuropsychological testing showed significant improvement (Global IQ mean of 58 versus 47, p < 0.04).;

This very useful contribution to the surgical literature supports the use of a disconnection strategy for patients with extensive involvement of the posterior quadrant with lesions that by themselves do not require resection.

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Persistent frequent subclinical seizures and memory impairment after clinical remission in smoldering limbic encephalitis

Kyoko Kanazawa, Riki Matsumoto, Akihiro Shimotake, Masako Kinoshita, Akiko Otsuka, Osamu Watanabe, Keiko Tanaka, Ryosuke Takahashi, Akio Ikeda.
Epileptic Disorders. 2014 Sep;16(3):312-7

Commentary by Pr. Yushi Inoue, Associate Editor

Not only the seizures but also impairment of cognition such as memory are important sequelae of limbic encephalitis in both acute and chronic phases. This manuscript described two patients with immune-mediated limbic encephalitis who had clinical seizures and memory impairment. Although clinical seizures remitted, memory impairment persisted over one to two years. The authors then gave the patients immunotherapy, which resulted in memory improvement. The clue was the EEG. The authors suggest a residual active chronic process of inflammation years after acute phase in a "smoldering" limbic encephalitis, and the importance of Video-EEG recording in diagnosing and treating such cases.

This contribution offers an important clinical message, as it seems that patients with limbic encephalitis need long term follow-up and assessment; the insult from the encephalitis may be not solely due to initial damage but also a lingering auto immune pathology that may need reinvestigation and treatment.

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Time to relapse after epilepsy surgery in children: AED withdrawal policies are a contributing factor.

Boshuisen et al.
Epileptic Disorders. 2014 Sep;16(3):305-11

Commentary by Pr. Michael Duchowny, Associate Editor
Director, Neurology Training and the Comprehensive Epilepsy Center
Miami Children's Hospital, Professor of Neurology and Pediatrics, University of Miami Miller School of Medicine
Florida, USA

Can the time course of seizure recurrence after epilepsy surgery in childhood be explained by operative or post-operative factors? This question has practical relevance as many children with intractable focal epilepsy who fail to become seizure-free after an excisional procedure return for further surgical consideration. Early postoperative seizure recurrence (generally regarded as occurring within the first six months) is traditionally believed to indicate a failure to fully excise the entire epileptogenic zone and structural lesion if found on MR imaging. Re-emergence of a postoperative seizure semiology that resembles the pre-operative pattern is believed to further implicate residual epileptic tissue in seizure persistence rather than a new independent seizure focus.

Until now there has been little interest in the specific role of post-operative AED withdrawal in promoting seizure recurrence. Indeed, studies of post-operative medical treatment have mainly focused on how long to continue treatment rather than examining any cause and effect relationship on seizure recurrence. Boshuisen and colleagues (1) set out to examine this issue in the Paediatric Epilepsy Surgery cohort of 95 patients from the “Time to Stop” study (2). Using multivariate analyses, they found that a shorter interval to AED reduction was the only independent predictor of a shorter time to relapse, revealing more rapidly whether surgery had the intended curative effect, independently of the other factors involved. It was also noted that univariate analysis identified incompleteness of resection of the epileptogenic zone was also associated with a shorter time to recurrence.

These observations extend the traditional view of surgical failure and in doing so raise many interesting questions. Does medication withdrawal “promote epileptogenesis after surgery and if so, how does this situation differ from the contribution of AED withdrawal in medical epilepsy patients? Should postoperative AED treatment be prolonged for a longer period of time, and are these findings unique to the pediatric patient? Further studies are clearly in order to address the questions raised by this important and provocative study.

Importantly, surgery was finally offered in more than 3/4 of the most complicated cases (25/32), with results that remained very good (64% of Engel class I). Even patients whose pathology finally proved normal or inconclusive - a finding, which remains challenging in the field of epilepsy surgery - could do well after surgery, although the rate of seizure freedom proved much less satisfactory (36% of Engel class I).

Overall, the merit of this study is to exist, and to serve as a reference in the field of invasive evaluation in children older than 2 years of age. The advent of 3D multimodality imaging methods should help in the future to better target the regions suspected to be epileptogenic, in order to avoid a second SEEG investigation (which represented almost 10% of the cases) and improve surgical results (17% of the children were classified as Engel class IV after surgery).

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1) Boshuisen K, Schmidt D, Uiterwaal CS, Arzimanoglou A, Braun KP, TimeToStop Study Group. Time to relapse after epilepsy surgery in children: AED withdrawal policies are a contributing factor. Epileptic Disord. 2014 Sep;16(3):305-11.

(2) Boshuisen K, Arzimanoglou A, Cross JH, Uiterwaal CS, Polster T, van Nieuwenhuizen O, Braun KP; TimeToStop study group. Timing of antiepileptic drug withdrawal and long-term seizure outcome after paediatric epilepsy surgery (TimeToStop): a retrospective observational study. Lancet Neurol. 2012

Stereo-electroencephalography (SEEG) in 65 children: an effective and safe diagnostic method for pre-surgical diagnosis, independent of age

Taussig D, Chipaux M, Lebas A, Fohlen M, Bulteau C, Ternier J, Ferrand-Sorbets S, Delalande O, Dorfmüller G.
Epileptic Disorders. 2014 Sep;16(3):280-95

Commentary by Pr. Philippe Kahane, Associate Editor
Director Epilepsy Unit, Neurology & Psychiatry Department Grenoble University Hospital, France

More than half a century after its development at Sainte Anne Hospital in Paris (1), the SEEG methodology, both in adults and children, has gained an increasing interest worldwide. For many years, the method has remained challenging in the young paediatric population, both for technical reasons (thickness and rigidity of the skull, tolerance to long-term monitoring) and for age-related semiological and neurophysiological peculiarities, which make localizing hypotheses more complex and difficult to elaborate than in adults.

Only a few studies have dealt specifically with SEEG in children (2, 3), thus making the present publication from the Rothschild Foundation team in Paris particularly interesting. Delphine Taussig and colleagues report their experience on the largest published series of SEEG in children (n=65), putting emphasis on the safety of the procedure (no morbidity, even in children as young as 20 months) and efficiency (78% of the patients eventually received surgery).

Interestingly, a large majority of the surgical procedures were monolobar, even in young children, which contrasts with the common idea that large resections are often mandatory at this age group. Such results confirm and underscore the fact that the main aim of SEEG investigations is to tailor the resections. Overall, 67% of the children became seizure-free (Engel class I), with results that appear better in young patients (n=21; age at SEEG < 5 years; seizure-free: 79%) as compared with the older children (n=44; age at SEEG > 5 years; seizure-free: 59%).

Importantly, surgery was finally offered in more than 3/4 of the most complicated cases (25/32), with results that remained very good (64% of Engel class I). Even patients whose pathology finally proved normal or inconclusive - a finding, which remains challenging in the field of epilepsy surgery - could do well after surgery, although the rate of seizure freedom proved much less satisfactory (36% of Engel class I).

Overall, the merit of this study is to exist, and to serve as a reference in the field of invasive evaluation in children older than 2 years of age. The advent of 3D multimodality imaging methods should help in the future to better target the regions suspected to be epileptogenic, in order to avoid a second SEEG investigation (which represented almost 10% of the cases) and improve surgical results (17% of the children were classified as Engel class IV after surgery).

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  1. Bancaud. Apport de l’exploration fonctionnelle par voie stéréotaxique à la chirurgie de l’épilepsie. Neurochirurgie 1959;5:5–112.
  2. Cossu et al. Stereo-EEG in children. Childs Nerv Syst 2006;22:766-778.
  3. Cossu et al. Stereoelectroencephalography in the presurgical evaluation of focal epilepsy in infancy and early childhood. J Neurosurg Pediatr 2012;9:290-300.

Can ACTH therapy improve the long-term outcome of drug-resistant frontal lobe epilepsy?

Epileptic Disorders June 2014

Giuseppe Gobbi, Giulia Loiacono, Antonella Boni, Lucia Marangio, Alberto Verrotti
Epileptic Disorders. Volume 16, Number 2, 185-190, June 2014

Commentary by Aristea S. Galanopoulou MD PhD, Epileptic Disorders Associate Editor
Professor, Saul R. Korey Department of Neurology
Associate Professor, Dominick P. Purpura Department of Neuroscience
Albert Einstein College of Medicine, Bronx NY, USA

Adrenocorticotropic hormone (ACTH) has been extensively used in the treatment of West syndrome (WS) but also for other early life epileptic encephalopathies, such as Landau Kleffner syndrome (LKS), and continuous spike wave in slow wave sleep syndrome (CSWS). These are epileptic syndromes that manifest in infancy or early childhood, do not necessarily have the same types of epileptic seizures, but have prominent interictal epileptic abnormalities with neurodevelopmental sequelae. Gobbi et al (Epileptic Disorders, June issue) report here beneficial effects of ACTH treatment in 5/6 young patients with drug-resistant frontal lobe epilepsy. Unlike patients with the classical ACTH-responsive epileptic encephalopathies (WS, LKS, CSWS), this small cohort included teenagers as well as children (range 4 to 17 years old). Their seizures were atypical absences, tonic or tonic versive or drop attacks, and all had secondary bilateral synchrony with focal abnormalities on their EEGs. Seizures stopped within 6 weeks in 83% of them. Among the responders, 60% maintained seizure freedom at 6 months of follow up while eventually all initial responders achieved seizure freedom at 12 months of follow up. No serious adverse effects were reported in this cohort.

The findings of Gobbi et al (Gobbi et al. 2014) indicate that ACTH may not be strictly the medical treatment for WS or LKS and CSWS but could potentially be considered in other drug resistant epileptic syndromes, as in frontal lobe epilepsy. Precedents do exist to support this concept. Snead et al (Snead et al. 1983) reported that 12/18 (66.7%) of children with drug-resistant epilepsies without infantile spasms responded to high dose ACTH, although the relapse rate was 40%. Okumura et al (Okumura et al. 2006) reported seizure freedom in 13/15 patients (86.7%) with generalized seizures (atypical absences, tonic, drop attacks or myoclonic but not infantile spasms) although again 46% relapsed. Kalra et al (Kalra et al. 2009) also reported therapeutic effects of ACTH in two patients (3 and 7 year old) with drug resistant epilepsies. In a Cochrane type review performed by Gayatri et al (Gayatri et al. 2007) only one double blind randomized prospective study was found that intended to evaluate the efficacy of steroids or ACTH4-9 analogue in children with intractable epilepsy without infantile spasms (Pentella et al. 1982). The study showed mild to moderate reduction in seizures in 3 / 4 children.

The existing small cohort studies are certainly valuable and maintain our interest in further exploring the possibilities of ACTH therapy in the pediatric epilepsy population, raising important questions. Which epilepsy syndromes might benefit from ACTH beyond WS / LKS / CSWS? Is ACTH beneficial only for very young patients or also for older age groups as the Gobbi study suggests? What are the long-term outcomes of ACTH treatment? The need for randomized, blinded, controlled, well powered studies to address these questions is evident, although such studies are not easy to be done.

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  • Gayatri NA, Ferrie CD, & Cross H. Corticosteroids including ACTH for childhood epilepsy other than epileptic spasms. Cochrane Database Syst Rev 2007: CD005222.
  • Gobbi G, Loiacono G, Boni A, Marangio L, & Verrotti A. Can ACTH therapy improve the long-term outcome of drug-resistant frontal lobe epilepsy? Epileptic Disord 2014; 16: 185-190.
  • Kalra V, Sharma S, & Arya R. ACTH therapy in refractory generalized epilepsy. Indian J Pediatr 2009; 76: 91-93.
  • Okumura A, Tsuji T, Kato T, Natsume J, Negoro T, & Watanabe K. ACTH therapy for generalized seizures other than spasms. Seizure 2006; 15: 469-475.
  • Pentella K, Bachman DS, & Sandman CA. Trial of an ACTH4-9 Analogue (ORG 2766) in children with intractable seizures. Neuropediatrics 1982; 13: 59-62.
  • Snead OC, 3rd, Benton JW, & Myers GJ. ACTH and prednisone in childhood seizure disorders. Neurology 1983; 33: 966-970.

Early add-on treatment vs alternative monotherapy in patients with partial epilepsy

Franck Semah, Pierre Thomas, Safia Coulbaut, Philippe Derambure
Epileptic Disorders. Volume 16, Number 2, 165-174, June 2014

Commentary by Graeme J Sills PhD, Epileptic Disorders Associate Editor
Department of Molecular & Clinical Pharmacology, University of Liverpool, UK

There are many significant questions in epilepsy therapeutics that remain to be answered, not least of which is what to do after the first drug fails. Much has been written on this issue but relatively few investigators have been brave enough to tackle it in a prospective manner. In this issue, Semah and colleagues report the outcome of a 10-year odyssey to shed light on whether it is better to switch or to add after the first medication has proved ineffective. In doing so, they also highlight the many challenges associated with these types of investigations.

In some ways, the results are the least interesting aspect of this article. In patients with recently diagnosed focal epilepsy who have failed their first choice antiepileptic drug due to incomplete seizure control at an adequate dose, there is no apparent difference between switching to another appropriate monotherapy and adding-in a second drug, in terms of either efficacy or tolerability. This is perhaps unsurprising given the size of the study population, their expected pharmacological responsiveness, and what we know from previous work.

Instead, it is the extreme heterogeneity, in both patient characteristics and drug choice, that sparks the greatest interest in this report and which serves as a reminder of the difficulty in delivering a study that is reflective of real-world clinical practice but also scientifically and statistically robust. This article is far from definitive in terms of treatment policy after initial drug failure in epilepsy but the authors should be applauded for their initial ambition, their continuing perseverance and their eventual honesty. There is a salutary lesson here for anyone who dreams of tackling the big issues in the pharmacological management of epilepsy.

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Paediatric epilepsy surgery in the posterior cortex: a study of 62 cases

Epileptic Disorders March 2014

Alexandra Liava, Roberto Mai, Laura Tassi, Massimo Cossu, Ivana Sartori, Lino Nobili, Giorgio Lo Russo, Stefano Francione
Epileptic Disorders 2014; 16(2): 141-64.

Commentary by Pr. Michael Duchowny, Associate Editor
Director, Neurology Training and the Comprehensive Epilepsy Center
Miami Children's Hospital
Professor of Neurology and Pediatrics, University of Miami Miller School of Medicine
Clinical Professor, Department of Neurology Florida International University College of Medicine
Miami, Florida, USA

Intractable focal epilepsy that begins in early postnatal life is associated with long-term neuro-cognitive dysfunction. Recognition of this poor prognosis is a decisive factor in the early surgical referral with a goal of definitive cure. However, candidates for surgical therapy in the first decade of life have many different etiologies and multiple regions of involvement that are approached utilizing different anatomic procedures. Despite these challenges, temporal and extra-temporal pediatric epilepsy surgery data in very early life is now widely available and serves as a helpful guide; the experience with MRI-negative patients has also been described (Duchowny et al, 1998; Jayakar et al, 2009). Missing from this picture, however, are large cohort studies focused more specifically on childhood surgery for focal seizures originating in the posterior hemispheric regions.

The recently published cohort study of 62 children with focal epilepsy arising in the posterior cerebral hemispheres (Liava et al, 2014) is therefore a welcome step towards filling this gap.  Although the outcome of posterior hemisphere pediatric epilepsy surgery has been reported previously (Fogarisi et al, 2003), additional carefully collected data is extremely valuable. Liava and colleagues (2014) describe their experience with 62 cases undergoing excisional procedures and functional disconnections who had thorough pre-operative investigations. The cohort was primarily MRI-positive- only 3 cases had unremarkable MRI studies, and 24 patients (39%) underwent stereo-EEG recordings. Not surprisingly, the cohort was heterogeneous with regard to etiology, surgical procedure and region(s) of involvement. The occurrence of varying degrees of pre-existing cognitive disturbance in a high proportion of cases is fully consistent with both pediatric surgical series and childhood epilepsy in general. The 29% incidence of hemianopic visual field defect is also not surprising in this selected cohort.

The investigators achieved a very high rate of seizure freedom with 86% achieving Engel Class 1 outcomes. This result is even more remarkable given the authors acknowledgement that complete resection “did not necessarily imply the complete resection of the MRI-detectable lesion”. That this cohort could attain such a high rate of seizure-freedom is welcome news for pediatric epilepsy surgery teams.

Although posterior hemispheric cases constitute a minority pediatric epilepsy surgery caseloads, they are by no means rare. Cortical malformations can occur anywhere within the cerebral cortex and osterior cortical involvement is common. The Milano cohort included a high proportion of multi-lobar cases, many of whom had ill-defined resection margins and involvement of eloquent (visual, somatosensory) cortex. Addressing both issues is a primary pediatric epilepsy surgery challenge and often contributes to compromised surgical success. As there is greater experience in adult epilepsy surgical populations with posterior resections and their challenges, the results of the Milano group offer important reassurance that similar approaches can also be used successfully in very young patients.

An important distinguishing feature of the Liava et al (2014) study is their attention to their patients’ seizure semiology. As the authors correctly note in their introduction, seizures that arise in the posterior cortex may propagate widely via multiple fascicular pathways resulting in clinical features more typical of secondary sites. This issue is especially problematic for seizures that arise in the parietal lobe as electrographic seizure onset is often clinically silent and thus associated with a falsely localizing “pseudo-focus” (Jayakar et al, 1991). With this understanding, the authors were able to identify a specific complex of symptoms and signs that were strongly suggestive of primary occipital lobe seizure origin. These included the presence of a pre-existing visual field defect and lateralized complex visual hallucinations in conjunction with oculogyric or oculo-clonic movement at ictal onset. Equally important, the investigators noted that ictal blindness and eye deviation were much less specific. Fear was a frequent subjective ictal complaint that disappeared in six of 7 patients undergoing parietal resection. However, although the perception of fear is described in patients with parietal lobe seizures, caution should be exercised as ictal fear can occur in patients with seizures arising in the frontal and temporal lobes (Toth et al, 2010; Akiyama et al, 2014).

All three MRI-negative patients had poor outcomes. While the presence of a discrete lesion on imaging is a useful marker of the epileptogenic zone, favorable postoperative outcomes are still possible without it (Jayakar et al, 2009). Functional imaging was not employed in the pre-operative evaluations of the Liava et al (2014) cohort and one is left wondering whether including this information might have made a difference. As electrophysiological information was insufficient for surgical planning in approximately one-third of cases and the scalp interictal EEG was judged to be misleading in approximately one-fifth of cases, it is understandable that accurate localization would be extremely difficult. However, for MRI-positive patients, complete removal of the anatomic lesion was not required for seizure-freedom, and the MRI-negative group, though small, likely failed for different reasons. It is known that resection of either the anatomic lesion or the epileptogenic zone can result in good outcome even if the other is not fully excised (Perry et al, 2010). The epilepsy in the MRI-negative patients was probably secondary to Type 1 focal cortical dysplasia and surgical failure was likely due to incomplete resection of a dysfunctionally activated cortical network. Whether the ability to define dysfunctional epileptic networks in the posterior cerebral hemisphere is comparable to anterior networks remains unknown. It is even less certain whether anatomically diverse networks are functionally similar.

The Liava et al (2014) study findings should be viewed as an important step in our progress towards understanding the surgical implications of lesion-driven posterior hemispheric seizure surgery. Lesionectomy, lobectomy, multi-lobar resection and disconnection, the surgical procedures employed in this report, are all preferable to more radical procedures such as hemispherectomy- the ability to achieve function-sparing seizure-freedom is obvious. The rate of seizure-freedom in this series is certainly comparable to the outcomes reported for frontal lobe cases. The justification for this “less is more” approach will become more secure if longitudinal studies can assess the long-term cognitive, behavioral and social outcomes of early posterior hemisphere surgery, particularly after tailored resection and lesionectomy. Pre-operative and post-operative neuropsychological data was collected by the investigators in this study and will hopefully be reported in a future publication.

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  • Akiyama M, Kobayashi K, Tukushi I et al. Five pediatric cases of ictal fear with variable outcomes. Brain Dev (2014),
  • Duchowny M, Jayakar P, Resnick T et al. Epilepsy surgery in the first three years of life. Epilepsia 1998; 39:737-743.
    Fogarasi A, Boesebeck F, Tuxhorn I. A detailed analysis of symptomatic posterior cortex seizure semeiology in children younger than seven years. Epilepsia 2003; 44:89-96.
  • Jayakar P, Duchowny M, Alvarez L et al. Pitfalls and caveats in localizing seizure foci.  J Clin Neurophysiol 1991; 8(4): 414-431. 
  • Jayakar P, Dunoyer C, Dean P et al. Epilepsy surgery in children with normal or non-focal MRI scans: integrative strategies offer longterm seizure relief. Epilepsia 2008; 49:758–764
  • Liava A, Mai R, Tassi L et al. Paediatric epilepsy surgery in the posterior cortex: a study of 62 cases. Epileptic Disord 2014; 16(2): 141-64.
  • Perry S, Dunoyer C, Dean P et al. Predictors of seizure freedom after incomplete resection in children.  Neurology 2010;75:1448–1453
  • Toth V, Fogarasi A, Karadi K et al. Ictal affective symptoms in temporal lobe epilepsy are related to gender and age. Epilepsia. 2010 ;51(7):1126-32.

Cortical network dysfunction in musicogenic epilepsy reflecting the role of snowballing emotional processes in seizure generation: an fMRI-EEG study.

Volker Diekmann & Anselm C. Hoppner
Epileptic Disorders. Volume 16, Number 1, 31-44, March 2014

Comments by Associate Editor Sara Wilson

Within the field of epilepsy research, musicogenic epilepsy has received scant attention, with little known about its underlying mechanisms despite being described over 75 years ago. By contrast, the field of music neuroscience is burgeoning with studies investigating the music networks of the brain, including the potent ability of music to activate reward and emotion processing systems. Musicogenic seizures provide an obvious opportunity to investigate these networks and their dysfunction in people with epilepsy. In the current study this was done in a patient using the gold-standard technique of EEG-fMRI.

An intriguing aspect of musicogenic epilepsy is triggering by highly selective music stimuli, which in this case was a specific type of Russian music as opposed to similar music of different origin. In addition to pink noise, the latter provided an ideal control stimulus, allowing the specificity of network dysfunction to be explored. This revealed activation of the affective network, particularly regions involved in positive emotional processing, at the onset of the Russian music. A clear strength of the study was subsequent exploration of the way this activation changed over time, prior to the emergence of a seizure. This analysis identified maladaptive interactions between components of the cognitive control, autobiographic memory and affective networks, with reduced cognitive down regulation thought to allow ‘snowballing’ activity in emotion-related areas, ultimately triggering the seizure.

By drawing on the broader cognitive neuroscience literature investigating network function in healthy individuals combined with careful examination of this instructive case, this study presents a compelling argument that musicogenic epilepsy may not just arise from musicological features of the stimulus per se. Moreover, the researchers propose that similar mechanisms of cognitive dysregulation of emotion processing networks may apply in other cases of reflexive epilepsy, such as reading epilepsy. This interesting hypothesis provides an exciting direction for future research into this poorly understood form of epilepsy.

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Generalised electrographic seizures presenting as perioral myoclonia

Jennifer Deraborn and Peter Kaplan
Epileptic Disorders. Volume 16, Number 1, 13-18, March 2014

Comments by Associate Editor Yushi Inoue MD PhD

A 41-year-old man had several episodes of rhythmic and intermittent, sometimes lateralized chin twitching lasting over a week for a seven years period. He reported understanding what was said but had difficulty replying due to the chin movement. The EEG during the episode showed paroxysms of polyspike and slow wave activity, maximal over the fronto-central regions, correlating with the chin movements. Brain imaging was normal. On one occasion, the episode was followed by a generalized tonic-clonic seizure. Levetiracetam added to valproate resolved the episode.

The authors along with video presentation guide the reader to the interesting differential diagnosis of this peculiar case with late onset long-lasting repetitive myoclonia localized to the perioral area with preserved consciousness. Published with videosequences

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Extreme startle and photomyoclonic response in severe hypocalcaemia  

Marcello Moccia, Roberto Erro, Elvira Nicolella, Pasquale Striano, Salvatore Striano
Epileptic Disorders. Volume 16, Number 1, 13-18, March 2014

Moccia and colleagues report a case with episodes of sudden generalised muscle contractions resulting in violent falls with injuries and urinary incontinence. The fits appeared to relate to sudden auditory stimuli. During the examination, a sudden and unexpected noise triggered a violent startle with a forceful closure of eyes, cranial muscle contraction, and raising of arms over the head, immediately followed by generalised stiffness for several seconds. Flashing light triggered an excessive startle followed by myogenic potentials. There was no epileptic activity on the EEG. Blood tests revealed severe hypocalcaemia and the symptoms disappeared after calcium supplementation.

This report teaches us that electrolyte disturbances can be associated with abnormal startle responses. Published with videosequences

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Confirming an expanded spectrum of SCN2A mutations: a case series

Dena Matalon, Ethan Goldberg, Livija Medne, Eric D. Marsh
Epileptic Disorders. Volume 16, Number 1, 13-18, March 2014

Comments by Associate Editor Aristea S. Galanopoulou MD PhD

Mutations in voltage-sensitive sodium channels (SCN) have been increasingly identified in human epilepsy syndromes. These syndromes include both “benign” forms (e.g., benign familial neonatal-infantile epilepsy) and more severe types (e.g., Dravet syndrome, infantile epileptic encephalopathies). Matalon and colleagues here describe the clinical features of 3 girls with 3 different de novo SCN2A mutations that presented with early onset epilepsies, poor developmental outcomes and axial hypotonia.  Thinning of the corpus callosum was eventually identified in two of the patients. Two of the girls manifested infantile spasms whereas the third had neonatal tonic seizures. In one of the patients with infantile spasms choreiform movements were also observed.

Of interest, a subsequent independent study by Hackenberg et al (Neuropediatrics, 2014) describes an identical mutation (c.4025T>C) of the SCN2A gene in a different girl who also presented with infantile spasms, choreiform movements, hypersomnia and progressive brain atrophy. These observations lend support to the pathogenicity of this specific mutation but also to its functional impact on both cortical and subcortical structures. Matalon et al nicely review the literature on genotype-phenotype correlations of the known human SCN2A mutations, discussing the inherent heterogeneity but also increasing evidence for a possible involvement of these mutations as modifiers of human epilepsies. Further studies are certainly needed to confirm the functional consequences of these mutations in the developing brain. The need for creation of comprehensive public access genotype-phenotype databases and animal models of the known and emerging epilepsy-related mutations is however becoming increasingly evident, so as to best study their clinical and pathogenic relevance.

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