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Review article
Epilepsy in children with Down syndrome
Ravindra Arya, Madhulika Kabra, Sheffali Gulati
with a reported prevalence of epilepsy of 1-13%. Infantile spasms (IS) or West syndrome (WS) is the most frequent epilepsy syndrome in children with DS. IS occur in 0.6-13% of children with DS, representing 4.5-47% of seizures in these children. Curiously, these patients have electroencephalographic (EEG) characteristics of idiopathic rather than symptomatic WS. Despite a lack of consensus on therapeutic approach, no significant difference has been reported among the different regimens with regards to achieving clinical remission or EEG normalisation. It appears that DS patients have better seizure control compared to other patients with symptomatic IS, and early initiation of appropriate treatment may contribute to the prevention of late seizure development and better developmental outcome. Lennox-Gastaut syndrome (LGS) also exhibits some distinctive features in children with DS including later onset and high incidence of reflex seizures. Other seizure types including partial and generalised tonic clonic seizures have also been described in children with DS. There is a high rate of EEG abnormalities in children with DS, even among children without epilepsy, however, no patterns specific to DS have been identified and EEG does not correlate with outcome. Various cellular and molecular mechanisms contribute to epileptogenesis in DS and offer an interesting field of study.
Original article
Electroclinical features of benign infantile seizures with mild gastroenteritis
Imad Yassin Saadeldin
entity. From 30 patients who were included in the study, five were excluded (two with developmental delay, one with microcephaly and two lost during follow-up). Twenty-five patients who fulfilled the diagnostic criteria for benign infantile seizures with mild gastroenteritis were assessed and followed at the Pediatric Department, Armed Forces Hospital, Southern Region, Khamis Mushayt, Saudi Arabia, between January 2004 and January 2009.
<i> </i>The median age at presentation was 10.4 months. Of the infants, 14 were females and 11 were males. Seizures were focal tonic or clonic in eight (32%) patients, focal with secondary generalisation in three (12%), generalised tonic-clonic in nine (36%) and consisted of staring only with no motor components in five (20%). Interictal electroencephalograms and brain imaging were normal for all patients. No patient required treatment with antiepileptic drugs. All the patients were found to have normal psychomotor development and neurological examination after a follow-up period of between 15 and 56 months.
<i> </i>The limitations of this study are the relatively small sample size, relatively short study period and the fact that the study was conducted in a tertiary referral hospital. The prevalence of this entity may be more common at the level of primary health centres. Increasing the awareness of clinicians regarding the existence of this syndrome and its benign nature in children will limit unnecessary investigations.
<i>[Published with video sequences]</i>
Treatment of malignant migrating partial epilepsy of infancy with rufinamide: report of five cases
Martina Vendrame, Annapurna Poduri, Tobias Loddenkemper, Gerhard Kluger, Giangennaro Coppola, Sanjeev V. Kothare
prognosis. In this study, we investigated retrospectively the efficacy of rufinamide (RUF) in five infants with MMPEI, by examining seizure type and frequency, EEG features and antiepileptic drug use. The data demonstrate that two of the five patients showed good efficacy and tolerability for RUF with a > 50% reduction in seizure frequency. The use of RUF for the treatment of MMPEI may therefore provide some hope for such infants and should be further validated.
<i>[Published with video sequences]</i>
The efficacy of bromides, stiripentol and levetiracetam in two patients with malignant migrating partial seizures in infancy
Milena Djuric, Ruzica Kravljanac, Gordana Kovacevic, Jelena Martic
therapeutic problem. In this study, we present two patients who were diagnosed with the disease, based on electroclinical symptoms. The patients were treated with a combination of sodium bromide, stiripentol and levetiracetam. The first patient unequivocally responded, following a course of ineffective conventional drugs, and the second, who was diagnosed and treated immediately, showed a more significant therapeutic response. Antiepileptic drugs, previously reported to be beneficial in case reports, when given concomitantly, may substantially reduce the number and severity of seizures, without influence on psychomotor development.
<i>[Published with video sequences]</i>
Impact of side of lesion, seizure outcome and interictal epileptiform discharges on attention and memory after surgery in tempo
Ilona Wisniewski, Anne-Sophie Wendling, Bernhard J. Steinhoff
who had undergone unilateral mesial temporal lobe resection. Ninety-four patients were investigated pre- and postoperatively by means of a standardised neuropsychological battery measuring subcomponents of attention, as well as short-term, working and long-term memory. The side of epilepsy surgery, seizure outcome and the presence of postoperative IEDs, as well as their possible relationship to the neuropsychological changes, were assessed. Statistical data were analysed using a repeated-measures MANOVA. The absence of seizures following surgery had a positive effect on short-term memory and attentional control. The occurrence of IEDs was found in patients with impaired figural learning. In terms of attentional control and working memory, patients who continued to present IEDs had also scored lower in these domains prior to surgery. IEDs had an effect independent of seizure presence, but were found to have a “supplementary negative effect” when the two variables were combined.
Predictive factors for a good prognosis following surgery for temporal lobe epilepsy: a cohort study in Spain
María Isabel Forcadas-Berdusán, José Luis Bustos-Sánchez, Elena Valle-Quevedo, Jon Aurrecoechea Obieta, Beatriz Mateos Goñi, Lorea Martinez-Indart, Ana Molano Salazar, Juan Carlos Gomez-Esteban, Iñigo Garamendi-Ruíz
and four years. This retrospective study included 115 selected patients who underwent surgery for temporal lobe epilepsy between 1996 and 2007. In the second year after surgery 86.1% of patients had a good prognosis for seizure control (73.9% Engel class I and 12.2% Engel class II) and 89.2% (76.3% Engel class I and 12.9% Engel class II) in the fourth year. Sixty-four of 93 (68.8%) patients were free of disabling seizures (Engel class I) during the entire period and 78 (83.8%) had good prognosis (Engel class I and II). For the second year, logistic regression analysis revealed the following variables to be independently predictive of good seizure control: absence of two or more seizure episodes in the first year after surgery, normal postoperative video-EEG, and age at surgery of less than 35 years. In the fourth year, mesial temporal sclerosis, female sex and normal postoperative video-EEG were the predictive factors. For the group with a good prognosis in both the second and the fourth year, the predictive variables were: absence of two or more seizure episodes in the first year after surgery (OR: 13.762, CI 95%: 2.566-73.808, p<0.002) and normal postoperative video-EEG (OR: 16.301, CI 95%: 3.704-71.740, p<0.001). This study illustrates the sustained benefit of temporal lobe epilepsy surgery. The multivariate logistic regression analysis failed to identify a good predictive model composed of preoperative variables alone, although it was possible to build such a model with either pre- and postoperative variables or only postoperative variables.
Hemispherectomy: a basis for mental development in children with epilepsy
Monique M.J. van Schooneveld, Aag Jennekens-Schinkel, Peter C. van Rijen, Kees P.J. Braun, Onno van Nieuwenhuizen
(median age at epilepsy onset of 0.0 years and at hemispherectomy 1.5 years; epilepsy duration of 0.2-2.6 years) and 12 older children (median age at onset of 1.0 year and at hemispherectomy 8.3 years; epilepsy duration of 1.1-11.7 years) with pharmacologically intractable seizures due to developmental, acquired or progressive pathology. Prospective study with consecutive inclusion of children, fixed assessment intervals (shortly before and 6, 12 and 24 months after hemispherectomy) and assessment using developmental scales and intelligence scales. Dependent variables included mental developmental index (MDI), mental age (MA) and mental developmental delay (MDD) in younger children and intelligence quotient (IQ) in older children. Mental development had arrested or deteriorated prior to hemispherectomy in 14 children (82%) assessed with developmental scales. In 14 children, it was not possible to more precisely determine MDI than “below the lowest MDI that the test manual provided” either before or after hemispherectomy. MA, however, increased in 16 children. Overall, IQ changed negligibly over two years after hemispherectomy, although an individual approach revealed variability. Children with Rasmussen encephalitis did not recover from the significant presurgical deterioration of intelligence. Removal of the affected hemisphere enables epileptic children, even those with severe mental delay, to further develop mentally.
Addressing overtreatment in patients with refractory epilepsy at a tertiary referral centre in Brazil
Veriano Alexandre Jr, Edna Almeida Monteiro, Priscila Freitas-Lima, Kylvia Dantas Pinto, Tonicarlo Rodrigues Velasco, Vera Cristina Terra, Ana Paula Pinheiro-Martins, Roberto Molina Souza, Emilio Perucca, Americo Ceiki Sakamoto
the impact of adverse effects on QOL and the utility of a structured instrument to help the physician manage adverse effects in patients with refractory epilepsy.
<i>Methods</i>. Clinical characteristics, drug treatment and adverse effects were evaluated in 102 patients with refractory epilepsy at a single tertiary referral centre. The Adverse Events Profile (AEP) and Quality of Life in Epilepsy-31 (QOLIE-31) questionnaires were completed at baseline and after six months. At baseline, patients with a high burden of adverse effects (AEP scores ≥45) were randomized to an intervention or control group. AEP scores in the intervention group were available to the physician as an instrument to help to reduce adverse effects.
<i>Results</i>. Ninety-five patients (93.1%) were on polytherapy. Sixty-six completed the questionnaires and, of these, 43 (65.1%) had a high AE burden and were randomized to the intervention and control group. QOLIE-31 scores were inversely correlated with AEP scores at both visits. Among randomized patients, AEP scores tended to decrease between the baseline and the final visit without significant differences between groups (intervention group: 54.1 ± 6.1
<i>vs</i> 51.1 ± 9.1; control group: 55.8 ± 5.8
<i>vs</i> 50.5 ± 12.2). QOLIE-31 scores did not change substantially between visits (intervention group: 45.9 ± 17.4
<i>vs</i> 48.4 ± 14; control group: 47.5 ± 15.7
<i>vs</i> 45.2 ± 18.9).
<i>Conclusion</i>. A significant proportion of patients had a high toxicity burden which had an impact on their QOL. Reduction of overtreatment is a difficult challenge which cannot be addressed solely by providing a structured assessment of adverse effects, but requires a more comprehensive approach aimed at optimizing the many components of the management strategy.
Refractory epilepsy is highly associated with severe dentoalveolar and maxillofacial injuries
Andre Luiz F. Costa, Clarissa Lin Yasuda, Marcondes C. França Jr, Marcia Elisabete Morita, Fernando Cendes
study was to describe the occurrence of orofacial injuries in patients with epilepsy. One hundred and nine consecutive patients (60 women; mean age 38.81 ± 14 years), treated for refractory epilepsy (45 with extratemporal epilepsy and 64 with temporal epilepsy) at the outpatient clinic of our University Hospital, were included in the present study. Orofacial injury occurring as a direct result of a seizure was determined by clinical examination and interview. In addition, seizure frequency, use of medication, and the occurrence and type of injury to other parts of the body, were documented. We employed regression analyses to investigate the association between teeth fractures and frequency of seizures. The majority of injuries were crown fractures (42 subjects), followed by mandibular fractures (eight subjects) and tooth avulsion (eight subjects). Sixteen patients had more than two fractured teeth.
<b> </b>Patients with mandibular trauma also suffered concomitant injuries (teeth fracture, avulsion and dislocation). The number of fractured teeth was associated with seizure frequency (r
<sup>2</sup> = 0.59, p < 0.001). The data suggest that there is an increased rate of dentoalveolar and maxillofacial injuries in patients with poorly controlled epileptic seizures.
Association between ABCB1 polymorphism and response to sodium valproate treatment in Malaysian epilepsy patients
Batoul Sadat Haerian, Kheng Seang Lim, Hui Jan Tan, Elsa Hanifa Mejia Mohamed, Chong Tin Tan, Azman Ali Raymond, Chee Piau Wong, Sau Wei Wong, Haslyna Omar, Harun Roslan, Zahurin Mohamed
we investigated the possible association between
<i>ABCB1</i> polymorphisms and sodium valproate (VPA) treatment in Malaysian epilepsy patients. Genotypes were assessed in 249 drug-resistant and 256 drug-responsive Malaysian patients for C1236T, G2677T/A, and C 5T polymorphisms in the
<i>ABCB1</i> gene. No genotypes, alleles, or haplotypes were associated with the response to VPA in either the overall group or Chinese, Indian, and Malay subgroups. Our data suggest that C1236T, G2677T/A, and C3435T polymorphisms in the
<i>ABCB1</i> gene do not contribute to the response to VPA in patients with epilepsy.
Clinical commentary
Sturge-Weber syndrome: a favourable surgical outcome in a case with contralateral seizure onset and myoclonic-astatic seizures
Premysl Jiruska, Petr Marusic, John G.R. Jefferys, Pavel Krsek, Roman Cmejla, Vera Sebronova, Vladimir Komarek
with refractory epilepsy which requires surgical treatment. We present a case of a patient who initially presented with partial seizures of temporo-occipital origin, ipsilateral to the pial angiomatosis. During the course of the disease, the patient developed medically refractory epilepsy with partial seizures originating predominantly from the contralateral temporo-occipital area as well as myoclonic and myoclonic-astatic seizures. Resection of the occipital and temporal lobe affected by the pial angioma resulted in favourable outcome. Bilateral dysfunction observed in Sturge-Weber syndrome may result in an increased capability of focal discharges to generate synchronous epileptiform activity leading to an increased incidence of generalised seizures, most probably via a mechanism of secondary bilateral synchrony.
<i>[Published with video sequences]</i>
Epilepsia partialis continua as the presenting symptom in probable sporadic Creutzfeldt-Jakob disease
Aslihan Taskiran, F. Irsel Tezer, Serap Saygi
continua. Here, we present a review and a case study, with video-EEG sequences and characteristic MRI findings, of a fifth patient who presented with epilepsia partialis continua in the very early stage of the disease and followed a fatal course which was faster than expected.
<i>[Published with video sequences]</i>
Late-onset Rasmussen's encephalitis and long-term remission
Laura Kupila, Leena Jutila, Arto Immonen, Ritva Vanninen, Esa Mervaala, Anders Pateau, Liisa Luostarinen, Reetta Klsquälvilsquäinen
with Rasmussen's encephalitis who has become completely symptomless, at least for three years, on enhanced antiepileptic and immunological medication.
Focal seizure propagation illustrated by fMRI
Hasan H. Sonmezturk, Victoria Morgan, Bassel Abou-Khalil
occipital with later spread to the left hemisphere and, to a lesser extent, the right hemisphere. The fMRI showed initial increase in blood oxygen level-dependent (BOLD) signal in the left occipital pole. The increased signal then propagated to the right occipital-posterotemporal region, and subsequently, the right and left mesial temporal regions. fMRI can be an effective tool to study seizure onset localization and seizure propagation in patients with frequent recurrent seizures.
Facio-mandibular myoclonus: a rare cause of nocturnal tongue biting
Udaya Seneviratne
which can be misdiagnosed for nocturnal seizures. The use of surface electromyographic channels over the facial muscles during video-EEG monitoring is helpful in confirming the diagnosis based on a typical burst pattern. This case report highlights difficulties in the diagnosis of facio-mandibular myoclonus and useful features which differentiate it from similar conditions.
Tonic status and electrodecremental paroxysms in an adult without epilepsy
Lyle W. Ostrow, Peter W. Kaplan
a 24-year-old woman who presented to the emergency department with prolonged tonic status epilepticus which evolved into stimulus-induced diffuse voltage attenuation (SIDVA) pattern in the setting of aseptic meningoencephalitis. There was no history of seizures or further events after recovery. This is the first report of a SIDVA pattern in an adult without a history of epilepsy.
Extraoccipital photoparoxysmal response in a case of focal encephalitis
Anis Jukkarwala, Haseeb Hassan, Muralidharan Nair, Rajesh S. Iyer
to be of occipital origin. Only six temporal lobe epilepsy patients have been reported in the literature with focal PPR at extraoccipital sites. We report a four-year-old girl with possible encephalitis who presented initially with epilepsia partialis continua of limbs on the right side. Interictally, she had left centro-parietal periodic lateralized epileptiform discharges (PLEDs). She responded to medical treatment and was free of seizures and motor and cognitive deficits at six months follow-up. Repeat EEG at follow-up showed left centro-parietal spikes accentuated by IPS. This is the first report of an extraoccipital, extratemporal focus showing PPR. The possible mechanism of PPR from this fronto-parietal epileptogenic focus is discussed.
Propofol withdrawal seizures: non-epileptic nature of seizures in a patient with recently controlled status epilepticus
Salman Zubair, Thomas Patton, Kenneth Smithson, Hasan H. Sonmezturk, Amir Arain, Bassel Abou-Khalil
mechanism of this phenomenon are not well understood and several case reports on these phenomena have presented only indirect evidence. We report on a patient who was administered propofol in order to control status epilepticus with success. However, every attempt at propofol withdrawal was followed by convulsive seizure-like activity. Continuous EEG monitoring showed muscle artefacts without any ictal discharges. Based on this finding, the propofol treatment was withdrawn and the seizure-like activity eventually attenuated and resolved. We propose that seizure-like phenomena associated with propofol withdrawal may not be ictal in nature and should not lead to unnecessary resumption of propofol infusion without documentation of an epileptic origin by EEG.
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