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ESES treatment: Topiramate?
A47
Abstracts had positive cognitive effects: memory improvement in 11 children, better concentration and attention in 17, better school achievements in five, easier speech in six, lability of emotions decreased in 11, better self-esteem in six, decreased irritability and hyperactivity in 20, diminished drowsiness in five, and complete disappearance of sedative effect in four. Our results show that treatment of seizures with LTG not only is effective in seizure control, but also improves cognitive development of children and allows them to achieve better quality of life.
The primary group of nurses offers regular consultations based on each child’s special background. Based on age and mental functioning the child will take part in a children’s group. A flip-over named ‘the causes, penguin’ is used to guide them through symptoms, diagnoses and treatment of their epilepsy. This information material is also suitable for teaching the child at home.
327 New hope for CSWS/ESES treatment: 286 Rasmiissen’s encephalitis: thalidomide D SOTTO, P LANDRIEU, CHU
de Bic&re,
Effect of a treatment
with
Pe’diatrique,
Paris, France
389
programme
for children with epilepsy
I L SVENDSEN, B WMIEN CILHUUS National
Universite’ Catholique de Louvain Centre Neurologique William Lennox, Service de Neurope’diatrie et de Neurophysiologie Clinique, Ottignies, Belgium
M TARDIEU
Neurologie
Several treatments have been tested for Rasmussen’s encephalitis including surgery, high-dose immunoglobulin, corticoid, without a clear-cut effect. More recently, in view of the demonstration of anti-glutamate antireceptor antibody, plasmapheresis has been proposed. We report here a case of Rasmussen’s encephalitis in a 6year-old girl treated by plasmapheresis and thalidomide, a known anti-tumour necrosis factor (TNF) agent. The diagnosis of Rasmussen’s encephalitis was established when she was 5 years old in view of a refractory focal epilepsy, focal NMR lesion and continuous degradation of neurological status. Different combinations of antiepileptic drugs were ineffective. A first series of plasmapheresis performed every 3 weeks was initially beneficial but the disease subsequently relapsed without modification of NMR lesions. A treatment with thalidomide (4mg/kg/day) was initiated after 7 months of iterative plasmapheresis. Seizures stopped within 3 weeks. No other plasmapheresis was performed. After 4 months, the patient was able to resume school activities and anti-epileptic drugs were strongly diminished. Thalidomide, through its potential anti TNF effect, might be effective in Rasmussen’s encephalitis.
Information
Topiramate?
S SYMANN, M RUCQUOY, J P MISSON, C GILLAIN, C BONNIER, K van RIJCKEVORSEL
Centre for Epilepsy,
Sandvika,
Norway
This presentation proposes how to meet the need of children with epilepsy and their families for information and support. The National Centre for Epilepsy has for several years developed different information programmes based on enabling families to become more capable of handling their child’s epilepsy. During their stay in the children’s department the parents have the choice of taking part in lessons on various topics. These are among others, social rights, how to classify seizures, types of medical treatment and special educational needs. This programme rolls over every 3 weeks to reach all the parents during the hospital stay which normally lasts for 3-5 weeks.
Two boys, each 9 years old, suffered from an epileptic syndrome characterized by: 1) an early onset (2.5 and 6 years of age); 2) the occurrence of epileptic seizures with generalized spike-and-wave (SW) discharges on EEG; 3) cognitive deterioration with language regression and 4) behavioural problems. Sleep EEG recordings showed almost continuous ( > 85%) diffuse SW activity during slow sleep. The diagnosis of continuous SW during sleep (CSWS or ESES) was made. After several unsuccessful anti-epileptic treatments, alone or combined (valproate, lamotrigine, benzodiazepines, corticoids, etc.), topiramate (TPM) was tried. TPM was initiated at 25mg/day and then progressively increased until 150mg/day within 6 weeks. At the present time, one child is undertaking TPM monotherapy and concomitant anti-epileptic drugs were quite reduced in the other. After 1-2 months of TPM treatment, a dramatic improvement in sleep EEG was observed in both cases with the disappearance of the SW discharges. Cognitive and behavioural problems are clinically improved, but complete neurocognitive testings are ongoing. To our knowledge, this is the first report of the use of TPM in CSWS/ESES with some success. Some additional cases are needed and more prolonged followup is necessary to confirm this positive result.
063 Familial sibling cases of atypical benign partial epilepsy of childhood and benign epilepsy of childhood with centrotemporal spikes KIYOKO SUGITA,’ YOHKO TANAKA,’ KATSUO SUGITA’ ‘Department of Paediatrics, lchikawa General Hospital, Tokyo Dental College; 2Department of Clinical Medicine, Faculty of Education, University of Chiba, Japan
A 6$-year-old girl was referred for evaluation of hypnagogic spasms of the eyelids and perioral area. Interictal EEG revealed centroparietal dominant spikes during sleep. Her seizures at the face and upper extremities were not controlled completely by CBS or PA. At the age of 8 years, the frequency of the seizures increased and another type of seizure appeared: absence seizures, facial spasms secondarily generalized to generalized tonic-clonic seizures and atonic seizures.
Abstracts had positive cognitive effects: memory improvement in 11 children, better concentration and attention in 17, better school achievements in five, easier speech in six, lability of emotions decreased in 11, better self-esteem in six, decreased irritability and hyperactivity in 20, diminished drowsiness in five, and complete disappearance of sedative effect in four. Our results show that treatment of seizures with LTG not only is effective in seizure control, but also improves cognitive development of children and allows them to achieve better quality of life.
The primary group of nurses offers regular consultations based on each child’s special background. Based on age and mental functioning the child will take part in a children’s group. A flip-over named ‘the causes, penguin’ is used to guide them through symptoms, diagnoses and treatment of their epilepsy. This information material is also suitable for teaching the child at home.
327 New hope for CSWS/ESES treatment: 286 Rasmiissen’s encephalitis: thalidomide D SOTTO, P LANDRIEU, CHU
de Bic&re,
Effect of a treatment
with
Pe’diatrique,
Paris, France
389
programme
for children with epilepsy
I L SVENDSEN, B WMIEN CILHUUS National
Universite’ Catholique de Louvain Centre Neurologique William Lennox, Service de Neurope’diatrie et de Neurophysiologie Clinique, Ottignies, Belgium
M TARDIEU
Neurologie
Several treatments have been tested for Rasmussen’s encephalitis including surgery, high-dose immunoglobulin, corticoid, without a clear-cut effect. More recently, in view of the demonstration of anti-glutamate antireceptor antibody, plasmapheresis has been proposed. We report here a case of Rasmussen’s encephalitis in a 6year-old girl treated by plasmapheresis and thalidomide, a known anti-tumour necrosis factor (TNF) agent. The diagnosis of Rasmussen’s encephalitis was established when she was 5 years old in view of a refractory focal epilepsy, focal NMR lesion and continuous degradation of neurological status. Different combinations of antiepileptic drugs were ineffective. A first series of plasmapheresis performed every 3 weeks was initially beneficial but the disease subsequently relapsed without modification of NMR lesions. A treatment with thalidomide (4mg/kg/day) was initiated after 7 months of iterative plasmapheresis. Seizures stopped within 3 weeks. No other plasmapheresis was performed. After 4 months, the patient was able to resume school activities and anti-epileptic drugs were strongly diminished. Thalidomide, through its potential anti TNF effect, might be effective in Rasmussen’s encephalitis.
Information
Topiramate?
S SYMANN, M RUCQUOY, J P MISSON, C GILLAIN, C BONNIER, K van RIJCKEVORSEL
Centre for Epilepsy,
Sandvika,
Norway
This presentation proposes how to meet the need of children with epilepsy and their families for information and support. The National Centre for Epilepsy has for several years developed different information programmes based on enabling families to become more capable of handling their child’s epilepsy. During their stay in the children’s department the parents have the choice of taking part in lessons on various topics. These are among others, social rights, how to classify seizures, types of medical treatment and special educational needs. This programme rolls over every 3 weeks to reach all the parents during the hospital stay which normally lasts for 3-5 weeks.
Two boys, each 9 years old, suffered from an epileptic syndrome characterized by: 1) an early onset (2.5 and 6 years of age); 2) the occurrence of epileptic seizures with generalized spike-and-wave (SW) discharges on EEG; 3) cognitive deterioration with language regression and 4) behavioural problems. Sleep EEG recordings showed almost continuous ( > 85%) diffuse SW activity during slow sleep. The diagnosis of continuous SW during sleep (CSWS or ESES) was made. After several unsuccessful anti-epileptic treatments, alone or combined (valproate, lamotrigine, benzodiazepines, corticoids, etc.), topiramate (TPM) was tried. TPM was initiated at 25mg/day and then progressively increased until 150mg/day within 6 weeks. At the present time, one child is undertaking TPM monotherapy and concomitant anti-epileptic drugs were quite reduced in the other. After 1-2 months of TPM treatment, a dramatic improvement in sleep EEG was observed in both cases with the disappearance of the SW discharges. Cognitive and behavioural problems are clinically improved, but complete neurocognitive testings are ongoing. To our knowledge, this is the first report of the use of TPM in CSWS/ESES with some success. Some additional cases are needed and more prolonged followup is necessary to confirm this positive result.
063 Familial sibling cases of atypical benign partial epilepsy of childhood and benign epilepsy of childhood with centrotemporal spikes KIYOKO SUGITA,’ YOHKO TANAKA,’ KATSUO SUGITA’ ‘Department of Paediatrics, lchikawa General Hospital, Tokyo Dental College; 2Department of Clinical Medicine, Faculty of Education, University of Chiba, Japan
A 6$-year-old girl was referred for evaluation of hypnagogic spasms of the eyelids and perioral area. Interictal EEG revealed centroparietal dominant spikes during sleep. Her seizures at the face and upper extremities were not controlled completely by CBS or PA. At the age of 8 years, the frequency of the seizures increased and another type of seizure appeared: absence seizures, facial spasms secondarily generalized to generalized tonic-clonic seizures and atonic seizures.