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P01.18 Malignant epilepsy of infancy with migrating multifocal seizures or Coppola Dulac syndrome
S36 68% cases had generalized EEG changes, 22% focal with secondary generalizations and 5.7% have focal character. Normal imaging was present in 57% of cases and pathological in 43%. Conclusions: Children with mild and moderate retardation have epilepsy in 15% of cases-Goulden and all. The tendency for manifestation of epilepsy is 9 times more frequent in children with mental retardation, and 12 times more in children with cerebral palsy-Angers and all. The results of our work are similar with other authors. P01.15 Timely treatment determines outcomes in Landau Kleffner syndrome M. Endziniene1 *, K. Zaveckiene1 , G. Jurkeviciene1 . 1 Kaunas University of Medicine, Lithuania Background: Landau-Kleffner syndrome (LKS) is a rare epileptic syndrome characterized by acquired aphasia with epilepsy. The usual onset is at 3−7 years of age. The initial symptoms are either epileptic seizures or perceptive and/or expressive language disorder. We present two cases with similar clinical manifestations but different outcomes. The first boy came to medical attention at the age of 5 years because of focal secondary generalized seizure at night. He was prescribed valproate (VPA), later lamotrigine, but several months later parents withdrew the treatment as the seizures persisted. Within one year following the onset his epilepsy catastrophically progressed: generalized tonic clonic (GTC) and daily drop attacks were complicated by gradual and complete loss of his language and behavioral deterioration. EEG showed electrical status epilepticus during sleep. Intensive treatment started 2 years after the epilepsy onset with methylprednisone, sultiam (STM), and ethosuximide (ESM) added to VPA. Drop attacks stopped within a few days, GTC became rare, but any significant improvement on receptive or expressive language was never observed. The second case was a 3.5-year-old boy who experienced gradual and complete loss of language and behavioral changes within two months. EEG showed frequent episodes of continuous spike-wave discharges, bilaterally synchronized. He never had any clinical epileptic seizure. Aggressive treatment with steroids, STM, ESM started immediately was very effective: the boy started to regain his language abilities 2 months later, and started normal school at expected age. His medication has been tapered gradually of within the next three years. Conclusions: Inadequate and delayed treatment of LandauKleffner syndrome results in irreversible speech and language difficulties even though seizure control may be nearly complete. Early adequate and aggressive treatment is justifiable as it may have dramatic positive effects on verbal functioning. P01.16 Comparison between simple and complex febrile convulsions: a clinical study D.W. Kim1 *. 1 Inje University Ilsan Paik Hospital, Korea, Republic Of Background: Febrile convulsions are classified into simple and complex types, the latter being characterized by increased risk of recurrence and progression to epilepsy. This study aimed to compare the clinical characteristics of simple and complex febrile convulsions. Methods: Between January 2007 and December 2009, 495 children were diagnosed with febrile convulsions at the Department of Pediatrics, Ilsan Paik Hospital in Korea. Their medical records were retrospectively reviewed for comparison between simple and complex febrile convulsions,
Poster sessions and clinical findings of complex febrile convulsions were clarified. Results: Our subjects comprised a male-to-female ratio of 1.6:1; the age range was from 8 months to 8 years. Simple febrile convulsions comprised 389 cases, i.e., 4 times as many as complex febrile convulsions (106 cases). The causes of febrile illness included acute pharyngotonsillitis (64.9%), pneumonia (10.0%), acute gastroenteritis (6.7%), and otitis media (3.6%). We did not find any significant difference between simple and complex febrile convulsions in most clinical parameters such as gender, age, family history of febrile convulsions, and cause of febrile illness. Regarding subtypes of complex febrile convulsions, repeated convulsions were the most frequent (72.0%), followed by prolonged convulsions (16.9%) and focal convulsions (5.1%). Conclusion: Although our results did not show any significant difference between simple and complex febrile convulsions in most clinical parameters, further studies should be performed to delineate the prognosis of complex febrile convulsions in comparison with simple febrile convulsions. P01.17 Epilepsy − A Historical Review T. Syriopoulou1 , E. Fourlani2 *, E. Karachanidi3,4 , I. Maggana1 , E. Michael4 , K. Straka1 . 1 Pediatrics Department, General Hospital of Kalamata, Kalamata, Greece, 2 Children’s Hospital ”Mitera”, Athens, Greece, 3 Speech-pathology department, TEI of Patras, Greece, 4 General Hospital of Kalamata, Kalamata, Greece Background: Epilepsy is a common neurological disorder and one of the most common diseases described in medical literature. Aim of the study: Brief historical overview of epilepsy (4.500 years BC until 18th -19th century). Methods: The material is drawn from Greek and foreign literature. Results: The main theories about epilepsy developed within the magical powers of ancient American Indians during Vedic period (4500 1500BC). In Ayurvedic literature of Charaka Samhita (400BC) epilepsy is described as a loss of consciousness containing sources regarding the symptoms, causes, diagnosis and treatment of disease. In Babylon (200BC), 40 tables record different types of seizures whilst emphasizing in the supernatural nature of epilepsy, a precursor approach of the Greek terms for “wicked disease”, as described by Hippocrates (500BC). The term “lunatic” was common in descriptions of epileptic persons as they considered to be influenced by the phases of the moon and it‘s goddess, Luna. However, Hippocrates believed that epilepsy is not a curse, but a brain disorder. In the 18th -20th century the disease was deeply understood: In 1857 the first active anti-epileptic drug was used, in 1873 the neurologists Hughlings Jackson and David Ferrier (London) and later Gustav Theodor Fritsch and Eduard Hitzing (Germany) − founded the pathophysiology of epilepsy and in 1922 Hans Berger, a German psychiatrist, developed the human electroencephalograph, an important instrument in the study of epilepsy. Conclusion: From the religious-spiritual approach to epilepsy and the first scientific approach of Hippocrates, it took several centuries to establish epilepsy as a brain disorder among medical fields. P01.18 Malignant epilepsy of infancy with migrating multifocal seizures or Coppola Dulac syndrome A. Kholin1 *. 1 Department of Neurology, Neurosurgery and Medical Genetics, Pediatric Faculty, Russian State Medical University, Moscow, Russian Federation Introduction: Malignant migrating partial seizures in infancy are rare epilepsy syndrome manifesting in the first 6 months
Poster sessions of life and characterized by multiple continuous electroencephalographic and electroclinical focal ictal patterns involved different independent areas of both hemispheres with severe retardation of psychomotor development. First description was made by G. Coppola et al. (1995) and the largest series of cases (n = 20) was published by O. Dulac (2005). Methods: In the period of 2006 2010 in the Child Neurology Department of the Russian Children’s Clinical Hospital we have investigated 16 infants with newly diagnosed malignant migrating partial seizures of infancy (MMPSI). In all the patients we have performed dynamic video-EEG monitoring, MRI and genetic assessment. Results: In our population of infants with MMPSI (n = 16) we have distinguished four subtypes of the syndrome: (1) “classic” form with pharmacoresistant migrated status epilepticus (SE) of migrating multifocal seizures, characterized by absolutely poor prognosis (n = 5); (2) “mild” variant with the evolution from monofocal epilepsy into multifocal with MISF pattern on the EEG (partial epilepsy − multiple independent spike focuses or PE-MISF syndrome) and then into MMPSI with SE episodes. Due to combined AEDs therapy seizures decreased, and this variant had better prognosis for life and psychomotor development (n = 4); (3) Severe mixed form (MMPSI + EME) with the combination of electroclinical characteristics of MMPSI with migrating multifocal SE and “suppression-burst” pattern with diffuse polyspikewaves and chaotic “erratic” myoclonus − features of early myoclonic encephalopathy (EME) (n = 5); (4) “subtle” form with only poor visually identifiable minimal motor and inhibitory seizures, subclinical migrating multifocal SE pattern on the EEG, multiple partial awakenings during sleep due to ictal patterns, severe delay of psychomotor development (n = 2). Conclusions: MMPSI represents a new form of epileptic encephalopathy and a special form of status epileptic in infancy. We propose the definition of this epileptic syndrome as “malignant epilepsy of infancy with migrating multifocal seizures” or “Coppola Dulac syndrome”.
P02. Epilepsy: Epidemiology and Outcome P02.1 10-year outcome of childhood epilepsy in well-functioning children and adolescents − social and psychological factors P. Jonsson1 *, O. Eeg-Olofsson1 . 1 Uppsala University Hospital, Sweden Background: A population based study of epilepsy in children from a Swedish county was published in 2006 (Larsson K, Eeg-Olofsson O. Eur J Paediatr Neurol 2006; 10:107−13). From this material well-functioning children treated for epilepsy in January 1997 (n = 47) were invited 10 years later to participate in a study to investigate the outcome from medical, social and psychological aspects. Aim: The first part (n = 45) described mainly the medical outcome, while this second part describes social and psychological outcome. Methods: An integral system of multi-informant assessment, ASEBA (Achenbach System of Empirically Based Assessment), was used to assess diverse aspects of adaptive and maladaptive functioning and is a further development of behaviour checklists. Results: The response rate was 69%. Thus, 31 individuals, 19 females, aged 11−22 years were evaluated. Seven were <18 years (group 1) and 24 18 years (group 2). At the end of the 10-year period 21 (68%) were seizure free. Four children were still in nine-year compulsory school, 10 attended senior high school, and 17 had passed this
S37 stage. Seven types of behavioural/emotional problems were found (23%). Internalizing symptoms as anxious/withdrawndepressed and somatic complaints were seen in both groups. An externalizing symptom as aggressive behaviour was found in group 1. Attention and thought problems were found in group 2 and social problems in group 1. Conclusions: In well-functioning individuals with childhood onset epilepsy there are few deviating results in just a few individuals. Maladaptive functioning, especially internalizing symptoms, was found. Such symptoms are often found in longitudinal population based studies of both children and adolescents with epilepsy. P02.3 The Effect of Interleukin-1b Secretion in Generation of Mesial Temporal Lobe Epilepsy with Hippocampal Sclerosis N.O. Dundar1 *, B. Aktekin2 , N.C. Ekinci3 , D. Sahinturk3 , U. Yavuzer3 , O. Yegin4 , S. Haspolat5 . 1 Suleyman Demirel University, Faculty of Medicine, Department of Pediatric Neurology, Isparta, Turkey, 2Yeditepe University Hospital, Department of Neurology, Istanbul, Turkey, 3Akdeniz University, Central Research Laboratory, Antalya, Turkey, 4 Akdeniz University, Faculty of Medicine, Department of Allergy and Immunology, Antalya, Turkey, 5 Akdeniz University, Faculty of Medicine, Department of Pediatric Neurology, Antalya, Turkey Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is a common medically intractable epilepsy syndrome. Although pathogenesis of HS still remains highly controversial, genetics may play a role as a predisposing factor. Previous evidence in a Japanese population shows that the homozygotes for allele T at position −511 of the interleukin (IL)-1b gene promoter region (IL-1b −511 T/T) confers susceptibility to the development of HS. But, IL1b levels of MTLE-HS patients were not studied. Our aim is to explain the role of IL-1b in etiopathogenesis of HS, functionally. A single base pair polymorphism at position −511 in the promoter region of the IL-1b gene was analyzed at 30 patients with HS. Spontaneous and 1 ng/ml lipopolysaccharide stimulated production of IL-1b by peripheral blood mononuclear cells after 4 hours and 24 hours of incubation were measured by ELISA method. The results were compared with 32 healthy subjects. The heterozygous type (−511 C/T) is the most common genotype in both groups. Frequency of allele −511 T is not different in patients and controls. Analysis of IL-1b levels, genotype and allele distributions showed no significant difference among the groups (p > 0.05). But it was seen that increased secretion of IL-1b levels in patients who carry T allele compare with, who not. In the present study, systemic secretion of IL-1b level has been considered. Because of the importance of IL-1b in the etiopathogenesis of disease, it is needed further studies with more cases in which locally produced IL-1b levels evaluated. P02.4 Maternal folic acid supplement use in early pregnancy and the risk of febrile seizures; the Generation R Study A. Visser1 *, V. Jaddoe1 , S. Willemsen1 , B. Hofman1 , H. Moll1 , W.F. Arts1 . 1 Erasmus Medical Center Rotterdam, Netherlands Background: Febrile seizures are the most common type of seizures in childhood. Aim of the study: To examine the association between folic acid supplement use during pregnancy and the risk of febrile seizures. Methods: This study was embedded in the Generation R Study, a population-based prospective cohort study from early fetal life onwards. Information on folic acid use was
Poster sessions and clinical findings of complex febrile convulsions were clarified. Results: Our subjects comprised a male-to-female ratio of 1.6:1; the age range was from 8 months to 8 years. Simple febrile convulsions comprised 389 cases, i.e., 4 times as many as complex febrile convulsions (106 cases). The causes of febrile illness included acute pharyngotonsillitis (64.9%), pneumonia (10.0%), acute gastroenteritis (6.7%), and otitis media (3.6%). We did not find any significant difference between simple and complex febrile convulsions in most clinical parameters such as gender, age, family history of febrile convulsions, and cause of febrile illness. Regarding subtypes of complex febrile convulsions, repeated convulsions were the most frequent (72.0%), followed by prolonged convulsions (16.9%) and focal convulsions (5.1%). Conclusion: Although our results did not show any significant difference between simple and complex febrile convulsions in most clinical parameters, further studies should be performed to delineate the prognosis of complex febrile convulsions in comparison with simple febrile convulsions. P01.17 Epilepsy − A Historical Review T. Syriopoulou1 , E. Fourlani2 *, E. Karachanidi3,4 , I. Maggana1 , E. Michael4 , K. Straka1 . 1 Pediatrics Department, General Hospital of Kalamata, Kalamata, Greece, 2 Children’s Hospital ”Mitera”, Athens, Greece, 3 Speech-pathology department, TEI of Patras, Greece, 4 General Hospital of Kalamata, Kalamata, Greece Background: Epilepsy is a common neurological disorder and one of the most common diseases described in medical literature. Aim of the study: Brief historical overview of epilepsy (4.500 years BC until 18th -19th century). Methods: The material is drawn from Greek and foreign literature. Results: The main theories about epilepsy developed within the magical powers of ancient American Indians during Vedic period (4500 1500BC). In Ayurvedic literature of Charaka Samhita (400BC) epilepsy is described as a loss of consciousness containing sources regarding the symptoms, causes, diagnosis and treatment of disease. In Babylon (200BC), 40 tables record different types of seizures whilst emphasizing in the supernatural nature of epilepsy, a precursor approach of the Greek terms for “wicked disease”, as described by Hippocrates (500BC). The term “lunatic” was common in descriptions of epileptic persons as they considered to be influenced by the phases of the moon and it‘s goddess, Luna. However, Hippocrates believed that epilepsy is not a curse, but a brain disorder. In the 18th -20th century the disease was deeply understood: In 1857 the first active anti-epileptic drug was used, in 1873 the neurologists Hughlings Jackson and David Ferrier (London) and later Gustav Theodor Fritsch and Eduard Hitzing (Germany) − founded the pathophysiology of epilepsy and in 1922 Hans Berger, a German psychiatrist, developed the human electroencephalograph, an important instrument in the study of epilepsy. Conclusion: From the religious-spiritual approach to epilepsy and the first scientific approach of Hippocrates, it took several centuries to establish epilepsy as a brain disorder among medical fields. P01.18 Malignant epilepsy of infancy with migrating multifocal seizures or Coppola Dulac syndrome A. Kholin1 *. 1 Department of Neurology, Neurosurgery and Medical Genetics, Pediatric Faculty, Russian State Medical University, Moscow, Russian Federation Introduction: Malignant migrating partial seizures in infancy are rare epilepsy syndrome manifesting in the first 6 months
Poster sessions of life and characterized by multiple continuous electroencephalographic and electroclinical focal ictal patterns involved different independent areas of both hemispheres with severe retardation of psychomotor development. First description was made by G. Coppola et al. (1995) and the largest series of cases (n = 20) was published by O. Dulac (2005). Methods: In the period of 2006 2010 in the Child Neurology Department of the Russian Children’s Clinical Hospital we have investigated 16 infants with newly diagnosed malignant migrating partial seizures of infancy (MMPSI). In all the patients we have performed dynamic video-EEG monitoring, MRI and genetic assessment. Results: In our population of infants with MMPSI (n = 16) we have distinguished four subtypes of the syndrome: (1) “classic” form with pharmacoresistant migrated status epilepticus (SE) of migrating multifocal seizures, characterized by absolutely poor prognosis (n = 5); (2) “mild” variant with the evolution from monofocal epilepsy into multifocal with MISF pattern on the EEG (partial epilepsy − multiple independent spike focuses or PE-MISF syndrome) and then into MMPSI with SE episodes. Due to combined AEDs therapy seizures decreased, and this variant had better prognosis for life and psychomotor development (n = 4); (3) Severe mixed form (MMPSI + EME) with the combination of electroclinical characteristics of MMPSI with migrating multifocal SE and “suppression-burst” pattern with diffuse polyspikewaves and chaotic “erratic” myoclonus − features of early myoclonic encephalopathy (EME) (n = 5); (4) “subtle” form with only poor visually identifiable minimal motor and inhibitory seizures, subclinical migrating multifocal SE pattern on the EEG, multiple partial awakenings during sleep due to ictal patterns, severe delay of psychomotor development (n = 2). Conclusions: MMPSI represents a new form of epileptic encephalopathy and a special form of status epileptic in infancy. We propose the definition of this epileptic syndrome as “malignant epilepsy of infancy with migrating multifocal seizures” or “Coppola Dulac syndrome”.
P02. Epilepsy: Epidemiology and Outcome P02.1 10-year outcome of childhood epilepsy in well-functioning children and adolescents − social and psychological factors P. Jonsson1 *, O. Eeg-Olofsson1 . 1 Uppsala University Hospital, Sweden Background: A population based study of epilepsy in children from a Swedish county was published in 2006 (Larsson K, Eeg-Olofsson O. Eur J Paediatr Neurol 2006; 10:107−13). From this material well-functioning children treated for epilepsy in January 1997 (n = 47) were invited 10 years later to participate in a study to investigate the outcome from medical, social and psychological aspects. Aim: The first part (n = 45) described mainly the medical outcome, while this second part describes social and psychological outcome. Methods: An integral system of multi-informant assessment, ASEBA (Achenbach System of Empirically Based Assessment), was used to assess diverse aspects of adaptive and maladaptive functioning and is a further development of behaviour checklists. Results: The response rate was 69%. Thus, 31 individuals, 19 females, aged 11−22 years were evaluated. Seven were <18 years (group 1) and 24 18 years (group 2). At the end of the 10-year period 21 (68%) were seizure free. Four children were still in nine-year compulsory school, 10 attended senior high school, and 17 had passed this
S37 stage. Seven types of behavioural/emotional problems were found (23%). Internalizing symptoms as anxious/withdrawndepressed and somatic complaints were seen in both groups. An externalizing symptom as aggressive behaviour was found in group 1. Attention and thought problems were found in group 2 and social problems in group 1. Conclusions: In well-functioning individuals with childhood onset epilepsy there are few deviating results in just a few individuals. Maladaptive functioning, especially internalizing symptoms, was found. Such symptoms are often found in longitudinal population based studies of both children and adolescents with epilepsy. P02.3 The Effect of Interleukin-1b Secretion in Generation of Mesial Temporal Lobe Epilepsy with Hippocampal Sclerosis N.O. Dundar1 *, B. Aktekin2 , N.C. Ekinci3 , D. Sahinturk3 , U. Yavuzer3 , O. Yegin4 , S. Haspolat5 . 1 Suleyman Demirel University, Faculty of Medicine, Department of Pediatric Neurology, Isparta, Turkey, 2Yeditepe University Hospital, Department of Neurology, Istanbul, Turkey, 3Akdeniz University, Central Research Laboratory, Antalya, Turkey, 4 Akdeniz University, Faculty of Medicine, Department of Allergy and Immunology, Antalya, Turkey, 5 Akdeniz University, Faculty of Medicine, Department of Pediatric Neurology, Antalya, Turkey Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is a common medically intractable epilepsy syndrome. Although pathogenesis of HS still remains highly controversial, genetics may play a role as a predisposing factor. Previous evidence in a Japanese population shows that the homozygotes for allele T at position −511 of the interleukin (IL)-1b gene promoter region (IL-1b −511 T/T) confers susceptibility to the development of HS. But, IL1b levels of MTLE-HS patients were not studied. Our aim is to explain the role of IL-1b in etiopathogenesis of HS, functionally. A single base pair polymorphism at position −511 in the promoter region of the IL-1b gene was analyzed at 30 patients with HS. Spontaneous and 1 ng/ml lipopolysaccharide stimulated production of IL-1b by peripheral blood mononuclear cells after 4 hours and 24 hours of incubation were measured by ELISA method. The results were compared with 32 healthy subjects. The heterozygous type (−511 C/T) is the most common genotype in both groups. Frequency of allele −511 T is not different in patients and controls. Analysis of IL-1b levels, genotype and allele distributions showed no significant difference among the groups (p > 0.05). But it was seen that increased secretion of IL-1b levels in patients who carry T allele compare with, who not. In the present study, systemic secretion of IL-1b level has been considered. Because of the importance of IL-1b in the etiopathogenesis of disease, it is needed further studies with more cases in which locally produced IL-1b levels evaluated. P02.4 Maternal folic acid supplement use in early pregnancy and the risk of febrile seizures; the Generation R Study A. Visser1 *, V. Jaddoe1 , S. Willemsen1 , B. Hofman1 , H. Moll1 , W.F. Arts1 . 1 Erasmus Medical Center Rotterdam, Netherlands Background: Febrile seizures are the most common type of seizures in childhood. Aim of the study: To examine the association between folic acid supplement use during pregnancy and the risk of febrile seizures. Methods: This study was embedded in the Generation R Study, a population-based prospective cohort study from early fetal life onwards. Information on folic acid use was