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P221 Cerebral venous sinus thrombosis in children and adolescents
Posters P218 Stroke in Swedish children; low incidence but high morbidity ¨ S. Christerson1,2 *. 1 Peadiatric Clinic, University Hospital, Orebro, Sweden; 2 Child and Youth Habilitation, Sweden Objective: To evaluate the incidence and presenting symptoms, time lag to diagnoses, risk factors, investigations and outcome of stroke in a cohort of Swedish children 2000 2006. Methods: A retrospective, population-based incidence and follow-up cohort study of children aged 1 month to 17 years at their first stroke. The geographic area was a Swedish health region representing one fifth of the Swedish population. Children were followed-up 11 /2 −8 years after the stroke episode with an interview of parent and child using a healthrelated quality of life inventory and a participation formula of ICF. A neurological investigation was also done. Children with arterial ischemic stroke (AIS), non traumatic hemorrhagic stroke (HS) and sinus venous thrombosis (CSVT) were included. Results: 51 children fulfilling the criteria were found. The average yearly incidence of stroke was 1.8 per 100,000 children (95% CI 1.3−2.3). Median age was 13 years (7 months to 17 years). 51% had AIS, 41% HS and 8% CSVT. Presentig symptoms were different in the various type of stroke. Time lag to diagnosis was more dependent on doctor’s than patient’s delay. Risk factors and predesposing diseases were found in 92% of the cases. At follow-up 8% of the children had died and 34/42 (81%) had residual impairments. Conclusions: Stroke is still a difficult diagnosis for the paediatrician and has a considable long time morbidity which influences the capability to participate in education and social activities. Reference(s) Pappachan J, Kirkham FJ. Cerebrovascular disease and stroke. Arch Dis Child 2008;93:890−8. McGlennan C, Ganesan V. Delays in investigation and management of acute arterial ischaemic stroke in children. Dev Med Child Neurol 2008;50:537−40. Fullerton HJ, Wu YW, Sidney S, Johnston SC. Recurrent hemorrhagic stroke in children: a population-based cohort study. Stroke 2007;38:2658−62. Friefeld S, Yeboah O, Jones JE, deVeber G. Health-related quality of life and its relationship to neurological outcome in child survivors of stroke. CNS Spectr 2004;9:465−75.
P219 Paediatric arterial ischemic stroke: functional outcome and risk factors C.E. Atsman-Berrevoets1 *, M.H. Cnossen2 , F.K. Aarsen1 , S.L.S. Van den Akker1 , M.A. Danen2 , I.M. Appel1 . 1 Department of Paediatric Neurology, ErasmusMC/Sophia Children Hospital; 2 Department of Paediatric Oncology and Haematology; 3 Centre for Medical Decision Making Erasmus University Rotterdam, The Netherlands Aim: To study functional outcome in children aged 1 month to 18 years after Paediatric Arterial Ischemic Stroke (PAIS) and identify risk factors influencing quality of life. Methods: In a consecutive series of 76 children with PAIS diagnosed in our hospital between 1997 2006 clinical, biochemical, and radiological data were prospectively collected. In 66 children surviving at least one year after PAIS functional outcome could be evaluated following the International Classification of Impairments, Disabilities, and Handicaps of the World Health Organisation. Results: Significant risk factors at presentation for a poor neurological outcome were young age, infarction in the right middle cerebral artery (MCA) territory, and fever at presentation. Fifty-four percent of children had severe neurological impairments at 12 months after PAIS and at last follow-up more than half needed remedial teaching, special education or institutionalisation. Health Related Quality of
S89 Life (HRQOL) questionnaires showed a significant lower HRQOL in all age groups. Children with a longer follow-up had lower HRQOL in the domain cognitive functioning. Interpretation: Our study shows significant morbidity, mortality, and a reduced HRQOL after PAIS depending on age, fever at presentation, and infarction in right MCA territory. Reference(s) DeVeber GA, MacGregor D, Curtis R, Mayank S. Neurologic outcome in survivors of childhood arterial ischemic stroke and sinovenous thrombosis. J. Child Neurol 2000;15:316−4. Hogan AM, Kirkham FJ, Isaacs EB. Intelligence after stroke in childhood: Review of the literature and suggestions for further research. J.Child Neurol. 2000;15:325−32. Simma B, Martin G, Muller T, Huemer M. Risk factors for pediatric stroke: consequences for therapy and quality of life. Ped. Neurol. 2007;37:121−6. Steinlin M, Roellin K, Schroth G. Long-term follow-up after stroke in childhood. Eur. J Paed. 2004;163:245−50. Gordon AL, Ganesan V, Towell A, Kirkham FJ. Functional outcome following stroke in children. J. Child Neurol. 2002;17:429−34.
P220 Cognitive outcome of 16 participants after childhood stroke: neuropsychological investigation of executive functioning S. Gonzalez-Monge1,2 *, N. Khann1 . 1 SMAEC, Lyon, France; 2 Hospices Civils de Lyon, Bron, France Objective: Many clinical studies demonstrated various cognitive deficits after childhood stroke, regardless of the lesion side. The purpose of the current study was to assess if cognitive deficits after cerebrovascular accident (CVA) could be explained by executive function disorders. Methods: There were 16 participants (11 males and 5 females) with CVA occurred between the age of 2 years 11 months and 16 years 11 months. Nine participants had left-sided lesions and 7 had right-sided lesions. Neuropsychological protocol included Wechsler scales according to the participant’s age and several executive functioning tests. For these tests we proceeded to qualitative measures and behavioral analysis. The mean age between CVA and neuropsychological evaluations was 3 y 4 mo (SD 3 y 2 mo; range 2 mo to 12 y 2 mo). Results: Concerning level of intellectual ability, 86% of our population scored 80 or higher. Regarding lesion-side, 92% of participants with left-sided lesion and only 77% with rightsided lesion scored 80 or higher. Performance IQ (PIQ) was reduced compared to Verbal IQ (VIQ), regardless of the lesion side. Concerning executive functioning, all participants presented a disorder. All participants with right-sided lesion presented a severe disorder and only 77% with left-sided lesion. All executive functions were impaired but essentially the inhibition, the speed of treatment and the global apprehension. Conclusions: First, the level of intellectual ability after childhood stroke after 2 years old is normal in a large proportion, but more impaired in right-sided lesions. This result will be discussed in reference with the theory of cerebral plasticity. Secondly, PIQ is inferior to VIQ independently of lesion-side. This result has classically been explained by the well-known “crowding effect”. Nevertheless, given that executive functions are heavily implied in PIQ subtests, PIQ decline could be explained by the severe executive disorders observed. These interpretations have to be debated in order to better target therapeutic strategies. P221 Cerebral venous sinus thrombosis in children and adolescents A. Szakacs ´ 1 *, T. Ek1 . 1 Department of Pediatrics, County Hospital, Halmstad, Sweden Objective: Cerebral venous sinus thrombosis (CVST) in children and adolescents is a relatively rare disease with
S90 an estimated incidence of 0.65/100,000/year but with a significant mortality of 6.3%. CVST requires always multidisciplinary treatment in the emergency management and during the clinical follow-up. Early diagnosis is crucial and requires a good knowledge of the symptoms. An appropriate neuroimaging method must be chosen. The aim of this study was to characterize symptoms, risk factors, clinical management and prognosis to increase awareness of this serious, but treatable, condition. Methods: A presentation of four recent cases at our hospital and a literature review covering a total of 409 patients. Results: The most frequent symptoms at onset are seizures 49%, decreased level of consciousness 46%, generalized weakness 45% and headache 40%. Common findings are fever 39%, hemipares 25%, cranial-nerve palsies 21% and papilledema 15%. The main risk factors are infections, perinatal complications, hypercoagulable/hematological diseases, dehydration and various other conditions. The most common hypercoagulable conditions are Lipoprotein A rise, Protein C deficiency, Factor V Leiden mutation and anticardiolipin antibodies. Venography with CT or MRI is the recommended neuro-radiological method. The therapy is controversial but thrombolysis and anticoagulation are most used in clinical practice. Individualisation is sometimes required and there is a relationship between type of treatment and prognosis. Conclusions: CVST is a multifactorial disease and has to be diagnosed early in the course and treated individually to achieve the best possible prognosis. Venography should be performed generously when suspicion arises. P222 Is headache an important symptom in children with arterial ischemic stroke? V. Brankovic-Sreckovic1 *, V. Milic Rasic1 , S. Todorovic1 . 1 Clinic for Child Neurology and Psychiatry, Belgrade, Serbia Objective: Symptoms and sings of childhood arterial ischemic stroke (AIS) vary regarding age, localization and volume of infarction, but common clinical presentations are hemiparesis, headache, altered consciousness and seizures. The purpose of this study was to evaluate frequency and temporal profile of headaches associated with AIS. Methods: Children with AIS were selected from the Clinic for Child Neurology and Psychiatry, Belgrade, from January 1996-January 2007. Patients including in this study were older than 3- up to 16 yr and were able to verbalize their complaints. Headache associated with AIS was considered as: 1) previous disorder 2) initial symptom of AIS and 3) poststroke headache. Results: There were 49 children, aged from 3−16 years (mean 9.7 yr) with clinically and radiologicaly diagnosed AIS. Recurrent headaches were present in three patients (6.1%) before the first AIS and 2 of them fulfilled the criteria for migraine. 19 (38.8%) of the children with AIS had a headache as an initial simptom, which corresponded with the side of infarction in the majority of them. In the follow up period (1−9 years) 7 (14.3%) children complained of headaches which occured sporadically and were of non-migrainous type. Conclusion: Acute unilateral headache was an important initial symptom in children with AIS. Although migrainerelated stroke was present rarely, it is worthy to consider it as an important risk factor for AIS. Conversely, migrainous headaches were not a prominent feature in the post-stroke period. Headaches after AIS were usually of tension type and in most of the cases were not frequently distressing.
Posters P223 Trombomodulin in ischemic stroke in children E. Pilarska1 *, M. Lemka1 , A. Bakowska2 . 1 Department of Developmental Neurology, Medical University of Gdansk, Poland; 2 Department of Immunopathology, Medical University of Gdansk, Poland Objective: Recently a quantitative systematic review discussed the role of thrombomodulin (Thm) in cerebral strokes in adults. It is still little known about the problem in children. The aim of the study was to analyse: if there is a difference in the values of Thm in children with ischemic stroke and if there is a dependence of values for Thm and on changes in neuroradiological examination. Material and Methods: Seventy patients were included in the study, comprising 40 children hospitalized from January 1995 till December 2005 in the Department of Developmental Neurology, Chair of Neurology Medical University of Gdansk and 30 healthy volunteers. In this group there was 40 patients after an ischemic stroke of an unknown and 30 children (of the same age) − healthy volunteers (no autoimmunologic disease and headaches in interview). The concentration of thrombomodulin (Thm) was determined according to an immunoenzyme method (ELISA). Results: Patients with stroke in comparison with the healthy group characterized significantly higher values of Thm. Patients without changes in cerebral vessels in neuroradiological examinations (MRA and AEG) showed more often the positive values of Thm. P224 Stroke syndromes in children with cardiac diseases V. Murugan1 *, V. Ganesan2 , M. Prengler2 , F. Kirkham1,2 . 1 Southampton General Hospital, Southampton, United Kingdom; 2 UCL, Institute of Child Health, London, United Kingdom Introduction: Congenital heart disease (CHD) is a common cause of paediatric arterial ischemic stroke (AIS); we review our experience. Objectives: 1) to identify clinical characteristics of stroke syndromes in children with CHD and their effect on outcome; 2) to identify the proportion with recent procedures and to define risk factors; 3) to determine whether additional investigations e.g. to exclude primary cerebrovascular disease (CVD), should be undertaken. Methods: Patients presenting to the pediatric neurology service at Great Ormond Street Hospital after a first acute AIS between 1978 and 2000 were reviewed. Results: Of 212 patients with AIS, 33 (16%) aged 0.5 16.3 (median 4.7) years, 23 (70%) male, had CHD (19 acyanotic, 14 cyanotic) with a wide range of diagnoses. Ten had other underlying diagnoses including genetic/chromosomal and neurocutaneous syndromes. Stroke followed cardiac surgery in 17 (52%) patients and catheterization in one, while 15 (49%) were spontaneous. Anterior stroke (n = 30, 90%) was commoner than posterior circulation involvement (n = 3, 10%) which was only diagnosed in acyanotic CHD (2 coarctation, 1 AVSD). Cerebral infarction was purely cortical in 7, purely subcortical in 8, both cortical and subcortical in 18 and was right-sided in 49%, left-sided in 30% and bilateral in 21%. CVD was identified in 16 (80%) of the 20 patients who had vascular imaging (7 occlusion, 1 hypoplasia with primary CVD in 8: 4 stenosis, 2 dissection, 2 moyamoya). Primary CVD was more common in spontaneous than post-procedure stroke (8/17 vs 0/3) but not significantly (p = 0.2). Sixteen (48%) had abnormal hemoglobin, 7 (6 acyanotic) with iron deficiency anaemia, while 9 (7 cyanotic) were polycythemic. Six patients (16%) died, and 10 (16%) had recurrence, more common in those with acyanotic CHD (Log-rank, p = 0.088) as well as CVD (Log-rank, p = 0.001).
P219 Paediatric arterial ischemic stroke: functional outcome and risk factors C.E. Atsman-Berrevoets1 *, M.H. Cnossen2 , F.K. Aarsen1 , S.L.S. Van den Akker1 , M.A. Danen2 , I.M. Appel1 . 1 Department of Paediatric Neurology, ErasmusMC/Sophia Children Hospital; 2 Department of Paediatric Oncology and Haematology; 3 Centre for Medical Decision Making Erasmus University Rotterdam, The Netherlands Aim: To study functional outcome in children aged 1 month to 18 years after Paediatric Arterial Ischemic Stroke (PAIS) and identify risk factors influencing quality of life. Methods: In a consecutive series of 76 children with PAIS diagnosed in our hospital between 1997 2006 clinical, biochemical, and radiological data were prospectively collected. In 66 children surviving at least one year after PAIS functional outcome could be evaluated following the International Classification of Impairments, Disabilities, and Handicaps of the World Health Organisation. Results: Significant risk factors at presentation for a poor neurological outcome were young age, infarction in the right middle cerebral artery (MCA) territory, and fever at presentation. Fifty-four percent of children had severe neurological impairments at 12 months after PAIS and at last follow-up more than half needed remedial teaching, special education or institutionalisation. Health Related Quality of
S89 Life (HRQOL) questionnaires showed a significant lower HRQOL in all age groups. Children with a longer follow-up had lower HRQOL in the domain cognitive functioning. Interpretation: Our study shows significant morbidity, mortality, and a reduced HRQOL after PAIS depending on age, fever at presentation, and infarction in right MCA territory. Reference(s) DeVeber GA, MacGregor D, Curtis R, Mayank S. Neurologic outcome in survivors of childhood arterial ischemic stroke and sinovenous thrombosis. J. Child Neurol 2000;15:316−4. Hogan AM, Kirkham FJ, Isaacs EB. Intelligence after stroke in childhood: Review of the literature and suggestions for further research. J.Child Neurol. 2000;15:325−32. Simma B, Martin G, Muller T, Huemer M. Risk factors for pediatric stroke: consequences for therapy and quality of life. Ped. Neurol. 2007;37:121−6. Steinlin M, Roellin K, Schroth G. Long-term follow-up after stroke in childhood. Eur. J Paed. 2004;163:245−50. Gordon AL, Ganesan V, Towell A, Kirkham FJ. Functional outcome following stroke in children. J. Child Neurol. 2002;17:429−34.
P220 Cognitive outcome of 16 participants after childhood stroke: neuropsychological investigation of executive functioning S. Gonzalez-Monge1,2 *, N. Khann1 . 1 SMAEC, Lyon, France; 2 Hospices Civils de Lyon, Bron, France Objective: Many clinical studies demonstrated various cognitive deficits after childhood stroke, regardless of the lesion side. The purpose of the current study was to assess if cognitive deficits after cerebrovascular accident (CVA) could be explained by executive function disorders. Methods: There were 16 participants (11 males and 5 females) with CVA occurred between the age of 2 years 11 months and 16 years 11 months. Nine participants had left-sided lesions and 7 had right-sided lesions. Neuropsychological protocol included Wechsler scales according to the participant’s age and several executive functioning tests. For these tests we proceeded to qualitative measures and behavioral analysis. The mean age between CVA and neuropsychological evaluations was 3 y 4 mo (SD 3 y 2 mo; range 2 mo to 12 y 2 mo). Results: Concerning level of intellectual ability, 86% of our population scored 80 or higher. Regarding lesion-side, 92% of participants with left-sided lesion and only 77% with rightsided lesion scored 80 or higher. Performance IQ (PIQ) was reduced compared to Verbal IQ (VIQ), regardless of the lesion side. Concerning executive functioning, all participants presented a disorder. All participants with right-sided lesion presented a severe disorder and only 77% with left-sided lesion. All executive functions were impaired but essentially the inhibition, the speed of treatment and the global apprehension. Conclusions: First, the level of intellectual ability after childhood stroke after 2 years old is normal in a large proportion, but more impaired in right-sided lesions. This result will be discussed in reference with the theory of cerebral plasticity. Secondly, PIQ is inferior to VIQ independently of lesion-side. This result has classically been explained by the well-known “crowding effect”. Nevertheless, given that executive functions are heavily implied in PIQ subtests, PIQ decline could be explained by the severe executive disorders observed. These interpretations have to be debated in order to better target therapeutic strategies. P221 Cerebral venous sinus thrombosis in children and adolescents A. Szakacs ´ 1 *, T. Ek1 . 1 Department of Pediatrics, County Hospital, Halmstad, Sweden Objective: Cerebral venous sinus thrombosis (CVST) in children and adolescents is a relatively rare disease with
S90 an estimated incidence of 0.65/100,000/year but with a significant mortality of 6.3%. CVST requires always multidisciplinary treatment in the emergency management and during the clinical follow-up. Early diagnosis is crucial and requires a good knowledge of the symptoms. An appropriate neuroimaging method must be chosen. The aim of this study was to characterize symptoms, risk factors, clinical management and prognosis to increase awareness of this serious, but treatable, condition. Methods: A presentation of four recent cases at our hospital and a literature review covering a total of 409 patients. Results: The most frequent symptoms at onset are seizures 49%, decreased level of consciousness 46%, generalized weakness 45% and headache 40%. Common findings are fever 39%, hemipares 25%, cranial-nerve palsies 21% and papilledema 15%. The main risk factors are infections, perinatal complications, hypercoagulable/hematological diseases, dehydration and various other conditions. The most common hypercoagulable conditions are Lipoprotein A rise, Protein C deficiency, Factor V Leiden mutation and anticardiolipin antibodies. Venography with CT or MRI is the recommended neuro-radiological method. The therapy is controversial but thrombolysis and anticoagulation are most used in clinical practice. Individualisation is sometimes required and there is a relationship between type of treatment and prognosis. Conclusions: CVST is a multifactorial disease and has to be diagnosed early in the course and treated individually to achieve the best possible prognosis. Venography should be performed generously when suspicion arises. P222 Is headache an important symptom in children with arterial ischemic stroke? V. Brankovic-Sreckovic1 *, V. Milic Rasic1 , S. Todorovic1 . 1 Clinic for Child Neurology and Psychiatry, Belgrade, Serbia Objective: Symptoms and sings of childhood arterial ischemic stroke (AIS) vary regarding age, localization and volume of infarction, but common clinical presentations are hemiparesis, headache, altered consciousness and seizures. The purpose of this study was to evaluate frequency and temporal profile of headaches associated with AIS. Methods: Children with AIS were selected from the Clinic for Child Neurology and Psychiatry, Belgrade, from January 1996-January 2007. Patients including in this study were older than 3- up to 16 yr and were able to verbalize their complaints. Headache associated with AIS was considered as: 1) previous disorder 2) initial symptom of AIS and 3) poststroke headache. Results: There were 49 children, aged from 3−16 years (mean 9.7 yr) with clinically and radiologicaly diagnosed AIS. Recurrent headaches were present in three patients (6.1%) before the first AIS and 2 of them fulfilled the criteria for migraine. 19 (38.8%) of the children with AIS had a headache as an initial simptom, which corresponded with the side of infarction in the majority of them. In the follow up period (1−9 years) 7 (14.3%) children complained of headaches which occured sporadically and were of non-migrainous type. Conclusion: Acute unilateral headache was an important initial symptom in children with AIS. Although migrainerelated stroke was present rarely, it is worthy to consider it as an important risk factor for AIS. Conversely, migrainous headaches were not a prominent feature in the post-stroke period. Headaches after AIS were usually of tension type and in most of the cases were not frequently distressing.
Posters P223 Trombomodulin in ischemic stroke in children E. Pilarska1 *, M. Lemka1 , A. Bakowska2 . 1 Department of Developmental Neurology, Medical University of Gdansk, Poland; 2 Department of Immunopathology, Medical University of Gdansk, Poland Objective: Recently a quantitative systematic review discussed the role of thrombomodulin (Thm) in cerebral strokes in adults. It is still little known about the problem in children. The aim of the study was to analyse: if there is a difference in the values of Thm in children with ischemic stroke and if there is a dependence of values for Thm and on changes in neuroradiological examination. Material and Methods: Seventy patients were included in the study, comprising 40 children hospitalized from January 1995 till December 2005 in the Department of Developmental Neurology, Chair of Neurology Medical University of Gdansk and 30 healthy volunteers. In this group there was 40 patients after an ischemic stroke of an unknown and 30 children (of the same age) − healthy volunteers (no autoimmunologic disease and headaches in interview). The concentration of thrombomodulin (Thm) was determined according to an immunoenzyme method (ELISA). Results: Patients with stroke in comparison with the healthy group characterized significantly higher values of Thm. Patients without changes in cerebral vessels in neuroradiological examinations (MRA and AEG) showed more often the positive values of Thm. P224 Stroke syndromes in children with cardiac diseases V. Murugan1 *, V. Ganesan2 , M. Prengler2 , F. Kirkham1,2 . 1 Southampton General Hospital, Southampton, United Kingdom; 2 UCL, Institute of Child Health, London, United Kingdom Introduction: Congenital heart disease (CHD) is a common cause of paediatric arterial ischemic stroke (AIS); we review our experience. Objectives: 1) to identify clinical characteristics of stroke syndromes in children with CHD and their effect on outcome; 2) to identify the proportion with recent procedures and to define risk factors; 3) to determine whether additional investigations e.g. to exclude primary cerebrovascular disease (CVD), should be undertaken. Methods: Patients presenting to the pediatric neurology service at Great Ormond Street Hospital after a first acute AIS between 1978 and 2000 were reviewed. Results: Of 212 patients with AIS, 33 (16%) aged 0.5 16.3 (median 4.7) years, 23 (70%) male, had CHD (19 acyanotic, 14 cyanotic) with a wide range of diagnoses. Ten had other underlying diagnoses including genetic/chromosomal and neurocutaneous syndromes. Stroke followed cardiac surgery in 17 (52%) patients and catheterization in one, while 15 (49%) were spontaneous. Anterior stroke (n = 30, 90%) was commoner than posterior circulation involvement (n = 3, 10%) which was only diagnosed in acyanotic CHD (2 coarctation, 1 AVSD). Cerebral infarction was purely cortical in 7, purely subcortical in 8, both cortical and subcortical in 18 and was right-sided in 49%, left-sided in 30% and bilateral in 21%. CVD was identified in 16 (80%) of the 20 patients who had vascular imaging (7 occlusion, 1 hypoplasia with primary CVD in 8: 4 stenosis, 2 dissection, 2 moyamoya). Primary CVD was more common in spontaneous than post-procedure stroke (8/17 vs 0/3) but not significantly (p = 0.2). Sixteen (48%) had abnormal hemoglobin, 7 (6 acyanotic) with iron deficiency anaemia, while 9 (7 cyanotic) were polycythemic. Six patients (16%) died, and 10 (16%) had recurrence, more common in those with acyanotic CHD (Log-rank, p = 0.088) as well as CVD (Log-rank, p = 0.001).