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West syndrome: A case report
Abstracts / Journal of the Neurological Sciences 333 (2013) e1–e64
2009 and November 2011 to the Children Hospital at Westmead, Australia, as well as 65 age-matched controls. Patients were clinically assessed and classified according to the revised ILAE classification 2010. Sera were tested blinded to a range of antigens by radioimmuno- and cell-based assays. Results: Eleven out of 114 (9.7%) patients were positive for one or more autoantibodies compared to 3 of 65 controls (4.6%, p = ns). Patients had antibodies to VGKC-complex (n = 4), CASPR2 (n = 3), NMDAR (n = 2), VGKC-complex and NMDAR (n = 2). The proportion of patients with epilepsy of unknown cause was higher in the antibody positive group (7/11; 63%) compared with the antibody negative subjects (23/103; 22%; p = 0.007, Fisher's exact test). Furthermore, 4 of 11 antibody-positive patients had focal epilepsy of unknown cause (36.4%) compared to only 12/113 of the antibodynegative patients (11.7%). Conclusions: Specific neuronal antibodies are present in children with new-onset epilepsy of “unknown cause”, often with focal epilepsies, this group of children may benefit most from autoantibody screening and consideration of immune therapy. doi:10.1016/j.jns.2013.07.221
Abstract — WCN 2013 No: 3150 Topic: 1 — Epilepsy Serum trace element levels in children receiving antiepileptic drug therapy: A cross-sectional study A.K. Kakkara, G. Kumarb, S. Gulatic, Y.K. Guptab. aPharmacology, All India Institute of Medical Sciences, Bhopal, New Delhi, India; b Pharmacology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India; cChild Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India Purpose: Few studies have evaluated the effects of antiepileptic drug (AED) therapy on trace element status in children and their results have been conflicting. The newer AEDs are considered to have a more acceptable safety profile, but this confidence is somewhat guarded in the absence of long-term data. This cross-sectional study was conducted to analyze the serum trace element levels in epileptic children treated with conventional and newer AEDs and compare them with healthy controls. Methods: The study included 92 epileptic children and 28 healthy controls. The participant distribution was as follows, Group I Phenytoin (PHT) monotherapy (n = 35), Group II Valproate (VPA) monotherapy (n = 30), Group III Valproate plus Levetiracetam (VPA + LEV) (n = 27), Group IV Healthy controls (n = 28). Serum levels of seven trace elements i.e. zinc, copper, magnesium, manganese, iron, selenium and strontium were determined using inductively coupled plasma-atomic emission spectrometry (ICP-AES). Results: Phenytoin monotherapy was associated with increased copper (1568.8 μg/L vs. 1053.6 μg/L, p = 0.009) and strontium (37.0 μg/L vs. 30.7 μg/L, p b 0.001) concentrations & decreased manganese levels (1.5 μg/L vs. 1.9 μg/L, p = 0.04). Valproate monotherapy treated children had decreased serum zinc (1010.5 μg/L vs. 1242.9 μg/L, p = 0.01) and selenium levels (67.0 μg/L vs.84.7 μg/L, p = 0.02) as compared to healthy controls. However, in VPA + LEV group no significant differences were observed in trace element profile as compared to healthy children. Conclusions: A significant difference in trace element levels in VPA and PHT treated epileptic children as compared to controls suggests a possible association between AED therapy and trace element alterations. However, levetiracetam when used in combination with
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valproate was not associated with these alterations. These findings further support its favorable adverse effect profile as compared to conventional AEDs. doi:10.1016/j.jns.2013.07.222
Abstract — WCN 2013 No: 3177 Topic: 1 — Epilepsy West syndrome: A case report A. Jusupova. Kyrgyz State Medical Academy, Bishkek, Kyrgyzstan West Syndrome is age-dependent epileptic encephalopathy of infancy, characterized by: 1. The infantile spasms — massive myoclonic, tonic, propulsive, retropulsive, symmetric, asymmetric, and serial isolated spasms of axial and limb muscles. 2. The EEG pattern hypsarrhythmia. 3. Psychomotor retardation. Case: The child was delivered on 42–43 weeks of gestation by cesarean section due to fetal hypoxia. Apgar score was 4/5. The first attack of tonic spasms with turning the head to the right at 3,5 month. On Brain MRI: multiple cysts and encephalomalacia, mixed hydrocephalus. Was appointed konvulex 25 mg/kg/day. Attacks continued. At the age of 6 months. EEG: saved atypical hypsarrhythmia with a regional accent. Sabril added 50 mg/day, the attacks were reduced to 20–30 per day. At the age of 9.5 months. Received synacthen-depot 0.125 mg/day every day 1 week, then every other day 2 weeks. On the 4th day of treatment, the child began to laugh, pronounce sounds, trying to get up on all fours. The age of 1 year and 2 months. Psychomotor retardation, observed dextral asymmetric spasms with turning the head to the right and making pose “swordsman”, single flexor and extensor spasms Taking Sabril 5 mg/kg/day, topiramate 3 mg/kg/day, konvulex 15 mg/kg/ day. Conclusion: This case is a symptomatic form of WS, which leads to resistance in the appointment of AEDs. Appointment of hormones led to a temporary reduction in seizure frequency. The prognosis of West syndrome depends on the time of diagnosis of the disease and prompts adequate therapy. doi:10.1016/j.jns.2013.07.223
Abstract — WCN 2013 No: 3172 Topic: 1 — Epilepsy Investigating the gap in the pharmacological treatment of epilepsy in low- and middle-income countries H. Shaha, A. Stellab, S.L. Moshéc, K. Weerasuriyad, T. Duaa. a Department of Mental Health and Substance Abuse, World Health Organization, Switzerland; bPreviously Affiliated with World Health Organization, Geneva, Switzerland; cSaul R. Korey Department of Neurology, The Montefiore/Einstein Epilepsy Center, Albert Einstein College of Medicine of Yeshiva University, Bronx, NY, USA; dDepartment of Essential Medicines and Health Products, World Health Organization, Geneva, Switzerland Epilepsy affects more than 50 million people worldwide, with many more affected by its consequences. Nearly 80% of the people with
2009 and November 2011 to the Children Hospital at Westmead, Australia, as well as 65 age-matched controls. Patients were clinically assessed and classified according to the revised ILAE classification 2010. Sera were tested blinded to a range of antigens by radioimmuno- and cell-based assays. Results: Eleven out of 114 (9.7%) patients were positive for one or more autoantibodies compared to 3 of 65 controls (4.6%, p = ns). Patients had antibodies to VGKC-complex (n = 4), CASPR2 (n = 3), NMDAR (n = 2), VGKC-complex and NMDAR (n = 2). The proportion of patients with epilepsy of unknown cause was higher in the antibody positive group (7/11; 63%) compared with the antibody negative subjects (23/103; 22%; p = 0.007, Fisher's exact test). Furthermore, 4 of 11 antibody-positive patients had focal epilepsy of unknown cause (36.4%) compared to only 12/113 of the antibodynegative patients (11.7%). Conclusions: Specific neuronal antibodies are present in children with new-onset epilepsy of “unknown cause”, often with focal epilepsies, this group of children may benefit most from autoantibody screening and consideration of immune therapy. doi:10.1016/j.jns.2013.07.221
Abstract — WCN 2013 No: 3150 Topic: 1 — Epilepsy Serum trace element levels in children receiving antiepileptic drug therapy: A cross-sectional study A.K. Kakkara, G. Kumarb, S. Gulatic, Y.K. Guptab. aPharmacology, All India Institute of Medical Sciences, Bhopal, New Delhi, India; b Pharmacology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India; cChild Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India Purpose: Few studies have evaluated the effects of antiepileptic drug (AED) therapy on trace element status in children and their results have been conflicting. The newer AEDs are considered to have a more acceptable safety profile, but this confidence is somewhat guarded in the absence of long-term data. This cross-sectional study was conducted to analyze the serum trace element levels in epileptic children treated with conventional and newer AEDs and compare them with healthy controls. Methods: The study included 92 epileptic children and 28 healthy controls. The participant distribution was as follows, Group I Phenytoin (PHT) monotherapy (n = 35), Group II Valproate (VPA) monotherapy (n = 30), Group III Valproate plus Levetiracetam (VPA + LEV) (n = 27), Group IV Healthy controls (n = 28). Serum levels of seven trace elements i.e. zinc, copper, magnesium, manganese, iron, selenium and strontium were determined using inductively coupled plasma-atomic emission spectrometry (ICP-AES). Results: Phenytoin monotherapy was associated with increased copper (1568.8 μg/L vs. 1053.6 μg/L, p = 0.009) and strontium (37.0 μg/L vs. 30.7 μg/L, p b 0.001) concentrations & decreased manganese levels (1.5 μg/L vs. 1.9 μg/L, p = 0.04). Valproate monotherapy treated children had decreased serum zinc (1010.5 μg/L vs. 1242.9 μg/L, p = 0.01) and selenium levels (67.0 μg/L vs.84.7 μg/L, p = 0.02) as compared to healthy controls. However, in VPA + LEV group no significant differences were observed in trace element profile as compared to healthy children. Conclusions: A significant difference in trace element levels in VPA and PHT treated epileptic children as compared to controls suggests a possible association between AED therapy and trace element alterations. However, levetiracetam when used in combination with
e63
valproate was not associated with these alterations. These findings further support its favorable adverse effect profile as compared to conventional AEDs. doi:10.1016/j.jns.2013.07.222
Abstract — WCN 2013 No: 3177 Topic: 1 — Epilepsy West syndrome: A case report A. Jusupova. Kyrgyz State Medical Academy, Bishkek, Kyrgyzstan West Syndrome is age-dependent epileptic encephalopathy of infancy, characterized by: 1. The infantile spasms — massive myoclonic, tonic, propulsive, retropulsive, symmetric, asymmetric, and serial isolated spasms of axial and limb muscles. 2. The EEG pattern hypsarrhythmia. 3. Psychomotor retardation. Case: The child was delivered on 42–43 weeks of gestation by cesarean section due to fetal hypoxia. Apgar score was 4/5. The first attack of tonic spasms with turning the head to the right at 3,5 month. On Brain MRI: multiple cysts and encephalomalacia, mixed hydrocephalus. Was appointed konvulex 25 mg/kg/day. Attacks continued. At the age of 6 months. EEG: saved atypical hypsarrhythmia with a regional accent. Sabril added 50 mg/day, the attacks were reduced to 20–30 per day. At the age of 9.5 months. Received synacthen-depot 0.125 mg/day every day 1 week, then every other day 2 weeks. On the 4th day of treatment, the child began to laugh, pronounce sounds, trying to get up on all fours. The age of 1 year and 2 months. Psychomotor retardation, observed dextral asymmetric spasms with turning the head to the right and making pose “swordsman”, single flexor and extensor spasms Taking Sabril 5 mg/kg/day, topiramate 3 mg/kg/day, konvulex 15 mg/kg/ day. Conclusion: This case is a symptomatic form of WS, which leads to resistance in the appointment of AEDs. Appointment of hormones led to a temporary reduction in seizure frequency. The prognosis of West syndrome depends on the time of diagnosis of the disease and prompts adequate therapy. doi:10.1016/j.jns.2013.07.223
Abstract — WCN 2013 No: 3172 Topic: 1 — Epilepsy Investigating the gap in the pharmacological treatment of epilepsy in low- and middle-income countries H. Shaha, A. Stellab, S.L. Moshéc, K. Weerasuriyad, T. Duaa. a Department of Mental Health and Substance Abuse, World Health Organization, Switzerland; bPreviously Affiliated with World Health Organization, Geneva, Switzerland; cSaul R. Korey Department of Neurology, The Montefiore/Einstein Epilepsy Center, Albert Einstein College of Medicine of Yeshiva University, Bronx, NY, USA; dDepartment of Essential Medicines and Health Products, World Health Organization, Geneva, Switzerland Epilepsy affects more than 50 million people worldwide, with many more affected by its consequences. Nearly 80% of the people with