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SP08 Neurological changes in children with the congenital heart disease
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Abstracts: Poster Presentations, the Seventh European Paediatric Neurology Society (EPNS) Congress
SP07 Neonatal arterial ischaemic stroke and prothrombotic factors: a case report S. Lekka1 , A. Xaidara1 *, H. Platokouki2 , S. Youroukos1 . 1 Dept. of Pediatrics of Athens University, 2 Hemophilia Center, Hemostasis Unit, Aghia Sophia Children’s Hospital, Athens, Greece Stroke in children is a heterogeneous disorder associated with significant morbidity and mortality. Incidence has been estimated at approximately 1 per 4,000 for neonates and has ranged from 1 per 7,000 to 1 per 70,000 for older children (1 month to 18 years). The most frequently reported risk factors for stroke in children include cardiac disorders, prothrombotic disorders, head trauma, inborn errors of metabolism, vascular disorders and infection. In up to two thirds of children no specific cause can be identified. Case report: We describe a three year old boy with developmental delay, admitted in the hospital because of febrile seizures. He was born after an uneventful, full term pregnancy. Since birth, hypertonia and right hemiplegia of moderate severity were noticed. Ultrasonography of the brain performed at that age revealed hyperdense areas in the caudate nucleus and the left cortical area. At the age of 16 months he presented with febrile seizures. An EEG performed was asymmetrical with slow waves and spikes on the left side. He started treatment with Oxcarbazepine. Despite anticonvulsant therapy, he continued to present with seizures. During his last admission the findings of the MRI were indicative of an extensive lesion affecting the anterior and the middle cerebral arteries. Laboratory evaluation for risk factors for stroke revealed that the child was compound heterozygote for the MTHFR C677T and Factor V Leiden G1619A mutations, as was his father. Homocysteine levels were within normal limits for both people. There are some previous reports of stroke associated with factor V Leiden mutations in near or at term neonates. Thus, thrombophilia screening, although expensive and involving drawing of large volume of blood, is justified in children presenting with first arterial ischaemic stroke. Diagnosis of a thrombophilic tendency may be important in relation to the patient’s overall management, in particular, in relation to prevention of recurrence. SP08 Neurological changes in children with the congenital heart disease M. Nurmukhamedova *. Nurmukhamedova, Tahskent Pediatric Medical Institute, Uzbekistan One of the leading causes of children’s mortality is due to congenital anomalies of the cardio-vascular system. The real quantity of such kind pathology among the first year period of time is several times higher than diagnosed patients. Aim: Evaluation of neurological symptoms at patients with the congenital heart disease nevertheless which kind of treatment tactics was chosen. Material and Methods: 30 children with the congenital heart diseases aged 1 to 14 years were included to study. (18 girls and 12 boys). 12 patients had a ventricular septum defect, 9 had a atrial septum defect, 6 had patent ductus arteriosus and 4 children had Fallot tetrade. Results: 5 patients suffered from headache, at 9 patients we observed paroxysm of systemic vertigo and at 22 patients we revealed irritability, tearfulness, troubled slumber, undiligence, distraction, 4 patients disturbance of convergention, weakness of a adductor muscles of eye, 5 central 2 paresis of the facial nerve, 19 evenly enlivening of tendinal and periostal reflexes, 4 anizoreflexia, 8 athological reflex athological reflex of Rassolimo. At half of Babinskiy, 12 of patients we observed a disorders of neurological system, which makes a reasonable of a detailed examination of children with the congenital heart disease.
SP09 Reversible posterior leukoencephalopathy syndrome in childhood: report of nine cases and review of the literature H. Gum ¨ 3 , A. Yikilmaz4 , ¨ u¸ ¨ s1 *, H. Per1 , S. Kumanda¸s1 , M. Kose Z. Gund ¨ uz ¨ 2 . 1 Department of Pediatric Neurology, 2 Department of Pediatric Nephrology, 3 Department of Pediatrics, 4 Department of Pediatric Radiology, Erciyes University, Medical Faculty, Kayzeri, Turkey Reversible posterior leukoencephalopathy syndrome (RPLS) is recently described disorder with typical radiologic findings in the posterior regions of the cerebral hemisphere and cerebellum. Its clinical symptoms include headache, decreased alertness, mental abnormalities, such as confusion, diminished spontaneity of speech, and changed behavior ranging from drowsiness to stupor, seizures, vomiting and abnormalities of visual perception like cortical blindness. RPLS is caused by various heterogeneous factors, the commonest being hypertension, followed by nonhypertensive causes such as eclampsia, renal diseases and immunosuppressive therapy. It is also seen different drugs. We presented 9 patients with RPLS who had primary diagnoses such as acute poststreptococcal glomerulonephritis, idiopathic hypertension, the performing of intravenous immunoglobulin for infection with crescentic glomerulonephritis, erythrocyte transfusion for severe iron deficiency, L-Asparaginase treatment for acute lymphoblastic leukemia and performing of granulocyte-colony stimulating factor for ulcerative colitis. Early recognition of RPLS as complication during different diseases and therapy in childhood may facilitate precise diagnosis and appropriate treatment. In this report, we described the first child patient who developed RPLS during the treatment of granulocyte-colony stimulating factor. SP010 Brain damage in shaken-impact syndrome M. Chuchin1 , T. Shvabrina2 , L. Ushakova2 . 1 Russian Medical Academy of Postgraduate Education; 2 Child’s Clinical Speransky Hospital, Russia Aim: Pathogenesis of brain damage in SIS and its management. Material: 28 infants first year of life. Methods: Fundoscopy, lumbal punction, repeated brain CT without contrast. Diagnosis SIS was established according to criteria of American Academy of Pediatrics Committee of Child Abuse and Neglect. Radiologic characteristics (CT) corresponded to SIS criteria. Radiologic characteristics (first week of admission) corresponded as well to sinovenous thrombosis criteria (all 28 cases), with (25) or without (3) venous cortico-subcortical brain infarctions. Another pathologic events typical to SIS (appearance and progression of subdural haematomas, progression of thrombosis on deep brain venous system, repeated retinal or preretinal haemorragias and even subarachnoid haemorragias could be logically explained as complications of untreated sinothrombosis combined with jatrogenic medical actions. Control group consists of 8 patients, was managed traditionally. Outcome in all cases was pessimistic. Comparison group consists of 12 patients. Management of sinovenous thrombosis was delated or provided only partly. 10 patients became invalided. Experimental group consists of 8 patients. Management was directed at prophylactic of thrombosis and its complications. No one in this group got any complications typical in SIS; outcome in all cases was excellent. Conclusion: SIS require thorough management of sinovenous thrombosis.
Abstracts: Poster Presentations, the Seventh European Paediatric Neurology Society (EPNS) Congress
SP07 Neonatal arterial ischaemic stroke and prothrombotic factors: a case report S. Lekka1 , A. Xaidara1 *, H. Platokouki2 , S. Youroukos1 . 1 Dept. of Pediatrics of Athens University, 2 Hemophilia Center, Hemostasis Unit, Aghia Sophia Children’s Hospital, Athens, Greece Stroke in children is a heterogeneous disorder associated with significant morbidity and mortality. Incidence has been estimated at approximately 1 per 4,000 for neonates and has ranged from 1 per 7,000 to 1 per 70,000 for older children (1 month to 18 years). The most frequently reported risk factors for stroke in children include cardiac disorders, prothrombotic disorders, head trauma, inborn errors of metabolism, vascular disorders and infection. In up to two thirds of children no specific cause can be identified. Case report: We describe a three year old boy with developmental delay, admitted in the hospital because of febrile seizures. He was born after an uneventful, full term pregnancy. Since birth, hypertonia and right hemiplegia of moderate severity were noticed. Ultrasonography of the brain performed at that age revealed hyperdense areas in the caudate nucleus and the left cortical area. At the age of 16 months he presented with febrile seizures. An EEG performed was asymmetrical with slow waves and spikes on the left side. He started treatment with Oxcarbazepine. Despite anticonvulsant therapy, he continued to present with seizures. During his last admission the findings of the MRI were indicative of an extensive lesion affecting the anterior and the middle cerebral arteries. Laboratory evaluation for risk factors for stroke revealed that the child was compound heterozygote for the MTHFR C677T and Factor V Leiden G1619A mutations, as was his father. Homocysteine levels were within normal limits for both people. There are some previous reports of stroke associated with factor V Leiden mutations in near or at term neonates. Thus, thrombophilia screening, although expensive and involving drawing of large volume of blood, is justified in children presenting with first arterial ischaemic stroke. Diagnosis of a thrombophilic tendency may be important in relation to the patient’s overall management, in particular, in relation to prevention of recurrence. SP08 Neurological changes in children with the congenital heart disease M. Nurmukhamedova *. Nurmukhamedova, Tahskent Pediatric Medical Institute, Uzbekistan One of the leading causes of children’s mortality is due to congenital anomalies of the cardio-vascular system. The real quantity of such kind pathology among the first year period of time is several times higher than diagnosed patients. Aim: Evaluation of neurological symptoms at patients with the congenital heart disease nevertheless which kind of treatment tactics was chosen. Material and Methods: 30 children with the congenital heart diseases aged 1 to 14 years were included to study. (18 girls and 12 boys). 12 patients had a ventricular septum defect, 9 had a atrial septum defect, 6 had patent ductus arteriosus and 4 children had Fallot tetrade. Results: 5 patients suffered from headache, at 9 patients we observed paroxysm of systemic vertigo and at 22 patients we revealed irritability, tearfulness, troubled slumber, undiligence, distraction, 4 patients disturbance of convergention, weakness of a adductor muscles of eye, 5 central 2 paresis of the facial nerve, 19 evenly enlivening of tendinal and periostal reflexes, 4 anizoreflexia, 8 athological reflex athological reflex of Rassolimo. At half of Babinskiy, 12 of patients we observed a disorders of neurological system, which makes a reasonable of a detailed examination of children with the congenital heart disease.
SP09 Reversible posterior leukoencephalopathy syndrome in childhood: report of nine cases and review of the literature H. Gum ¨ 3 , A. Yikilmaz4 , ¨ u¸ ¨ s1 *, H. Per1 , S. Kumanda¸s1 , M. Kose Z. Gund ¨ uz ¨ 2 . 1 Department of Pediatric Neurology, 2 Department of Pediatric Nephrology, 3 Department of Pediatrics, 4 Department of Pediatric Radiology, Erciyes University, Medical Faculty, Kayzeri, Turkey Reversible posterior leukoencephalopathy syndrome (RPLS) is recently described disorder with typical radiologic findings in the posterior regions of the cerebral hemisphere and cerebellum. Its clinical symptoms include headache, decreased alertness, mental abnormalities, such as confusion, diminished spontaneity of speech, and changed behavior ranging from drowsiness to stupor, seizures, vomiting and abnormalities of visual perception like cortical blindness. RPLS is caused by various heterogeneous factors, the commonest being hypertension, followed by nonhypertensive causes such as eclampsia, renal diseases and immunosuppressive therapy. It is also seen different drugs. We presented 9 patients with RPLS who had primary diagnoses such as acute poststreptococcal glomerulonephritis, idiopathic hypertension, the performing of intravenous immunoglobulin for infection with crescentic glomerulonephritis, erythrocyte transfusion for severe iron deficiency, L-Asparaginase treatment for acute lymphoblastic leukemia and performing of granulocyte-colony stimulating factor for ulcerative colitis. Early recognition of RPLS as complication during different diseases and therapy in childhood may facilitate precise diagnosis and appropriate treatment. In this report, we described the first child patient who developed RPLS during the treatment of granulocyte-colony stimulating factor. SP010 Brain damage in shaken-impact syndrome M. Chuchin1 , T. Shvabrina2 , L. Ushakova2 . 1 Russian Medical Academy of Postgraduate Education; 2 Child’s Clinical Speransky Hospital, Russia Aim: Pathogenesis of brain damage in SIS and its management. Material: 28 infants first year of life. Methods: Fundoscopy, lumbal punction, repeated brain CT without contrast. Diagnosis SIS was established according to criteria of American Academy of Pediatrics Committee of Child Abuse and Neglect. Radiologic characteristics (CT) corresponded to SIS criteria. Radiologic characteristics (first week of admission) corresponded as well to sinovenous thrombosis criteria (all 28 cases), with (25) or without (3) venous cortico-subcortical brain infarctions. Another pathologic events typical to SIS (appearance and progression of subdural haematomas, progression of thrombosis on deep brain venous system, repeated retinal or preretinal haemorragias and even subarachnoid haemorragias could be logically explained as complications of untreated sinothrombosis combined with jatrogenic medical actions. Control group consists of 8 patients, was managed traditionally. Outcome in all cases was pessimistic. Comparison group consists of 12 patients. Management of sinovenous thrombosis was delated or provided only partly. 10 patients became invalided. Experimental group consists of 8 patients. Management was directed at prophylactic of thrombosis and its complications. No one in this group got any complications typical in SIS; outcome in all cases was excellent. Conclusion: SIS require thorough management of sinovenous thrombosis.