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IAP023 Juvenile dermatomyositis presenting with anasarca
Abstracts: Poster Presentations, the Seventh European Paediatric Neurology Society (EPNS) Congress presented epileptic seizures had antiphospholipid syndrome and interictal epileptic abnormalities on EEG. Despite the relatively common involvement of CNS in SLE, presentation of this disorder with neurologic symptoms appears to be uncommon. True incidence of seizures in SLE is difficult to determine, owing to multiple potential etiologies. Epileptic seizures were observed in 12.4% (JSLE) patients. The case who had recurrent seizure was associated with antiphospholipid antibody syndrome. Keywords: SLE, Seizure, Antiphospholipid syndrome. IAP023 Juvenile dermatomyositis presenting with anasarca F. Alehan *, S. Saygı, E. Baskin, I. Erol, N. Ozbek. Baskent University, Ankara, Turkey Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by inflammation of the muscles, connective tissue, skin, gastrointestinal tract and small nerves. Although localized edema of muscles and periorbital region might be associated with JDM, anasarca-type edema is very rarely seen in this disease. We here report a 4-year-old boy with juvenile dermatomyositis presenting with a 2-week history of fatigue, myalgia and weakness along with swelling of the extremities, face, chest, abdomen and scrotum. Of the diagnostic criteria of JDM, severe symmetric weakness of the proximal musculature, characteristic cutaneous changes, elevated serum muscle enzymes and myopathic electromyographic abnormalities were present. Magnetic resonance imaging of the upper extremities showed marked diffuse edema in the subcutaneous tissue, muscles and myofascia. Edema and weakness resolved after steroid therapy. In conclusion, JDM should be included in the differential diagnosis in patients presenting with anasarca-type edema and weakness.
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IAP025 Prevalence of antiphospholipid and antinuclear antibodies in children with epilepsy 1 1 *, T. Constantin1 , T. Kalovics , K. Sallai2 , R. Kalm ´ ´ anchey ´ E. Nagy2 , P. Gergely2 , G. Fekete1 , A. Ponyi1 . 1 Department of Pediatrics II., Semmelweis University, Budapest, Hungary, 2 Centrali Laboratory of Immunology, Semmelweis University, Budapest, Hungary
Objective: The antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent venous thrombosis or arterial occlusive events associated with elevated levels of antiphospholipid antibodies (aPL). Many neuropsychiatric manifestations have been described in association with APS. Methods: The presence of antinuclear-, anti-b2-glycoprotein I- and anticardiolipin antibodies were investigated in 60 consecutively selected children with epilepsy. Results: Almost 50% of our patients were ANA positive. 20% patients have moderate titer of ANA. Anti-C1q antibody was positive in 4 cases (6%), all of them are symptome-free considering renal manifestation of lupus. Interestingly only 1 child (1.5%) had aCL antibody, while 14% patients were LAC positive. Anti-b2-glycoprotein I-antibody positivity was not detected in this cohort of patients. Discussion: The clinical relevance of aPL tests in childhood are difficult to explain. In the present study obviously lower total prevalence of aPLs was observed in children with epilepsy than in the previously reported investigations (20−30%). The higher amount of LAC positive patients indicates that coagulation studies (LAC) should be included in the neuroimmunological assessment of suspected APS patients with epileptic disorders. The difference between the results of serological and LAC studies could be explained by the possible positivity of other non-investigated antibodies.
C. Cerminara, F. Refice, R. Bombardieri, M. Pinci, E. D’Agati, P. Curatolo *. Pediatric Neurology Unit, Neuroscience Dept., Tor Vergata University, Rome, Italy
IAP026 Central nervous system involvement in paediatric SLE: Do we need an MRI? E. Demirkaya4 , Y. Bilginer4 , D. Yalnizoglu1 *, K.K. Oguz3 , V. Isikhan2 , N. Besbas4 , A. Bakkaloglu4 , S. Ozen4 . 1 Hacettepe University Dept. of Pediatric Neurology, 2 Hacettepe University Dept. of Social Work, 3 Hacettepe University Dept of Radiology, 4 Hacettepe University Dept. of Pediatric Nephrology and Rheumatology, Ankara, Turkey
Juvenile idiopathic arthritis (JIA) represents the main form of arthritis affecting children. Epileptic seizures are uncommon in patients with JIA and are usually due to meningovasculitis. We describe two patients affected by JIA whit epileptic manifestations. Case 1: a 3-year-old girl affected by JIA and segmental myoclonic jerks, with focal epileptiform abnormalities on EEG. Good seizures control was reached with VPA. After a reactivation of JIA, epileptic manifestations reappeared. VPA was increased and actually, she takes VPA, MTX, Flubioprophen and cycles of corticosteroids and is seizurefree. Case 2: a 14-year-old boy affected by Juvenile Absence (seizure-free with Lamotrigine and VPA) and JIA with partial remission. We describe the first cases of association between JIA and idiopatic epileptic manifestations suggesting the possible role of the immunological system in the pathogenesis of some epileptic seizures. The presence of autoantibodies in epilepsy or seizure-associated disorders and the anticonvulsivant efficacy of immunomodulatory drugs might suggest a role of immunity in the pathogenesis of some epileptic disorders. In case 1 is therefore possible that the remission of epileptic manifestations is both due to an appropriate anti-epileptic drug and a good control of JIA.
Rationale: The diagnosis of neurological involvement in SLE is complex due to the wide spectrum of manifestations. We studied the neurological symptoms and signs in paediatric patients with SLE. Methods: Patients with SLE presenting to the paediatric nephrology and rheumatology clinic in a 3-month period in 2006 were evluated. MRI was obtained in all patients, MRA and EEG were performed in selected cases. The burden of disease was assessed by Symptom Distress Check List (SCL90-R) questionnaire. Results: The mean age at the time of evaluation was 15.8 (range = 6−22). The mean age at the time of diagnosis was 11.4 (range = 6−16). Four patients had obvious neurological signs and/or symptoms at onset or admission. Overall 2/3 of the patients had neurological symptoms and/or signs. MRI was abnormal in four patients who had overt neurological symptoms. According to the SCL-90-R questionnaire 4 patients had significant depression, two had significant anxiety. 37.5% of adolescents expressed a decline in school performance. Discussion and Conclusions: MRI is probably not required in patients who do not have any neurological symptoms or signs. The decline in school performance may be partially attributed to the loss of school days. Further studies may guide the need for more advanced neuroimaging studies.
IAP024 Juvenile idiopathic arthritis and epileptic manifestations: report on two cases
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IAP025 Prevalence of antiphospholipid and antinuclear antibodies in children with epilepsy 1 1 *, T. Constantin1 , T. Kalovics , K. Sallai2 , R. Kalm ´ ´ anchey ´ E. Nagy2 , P. Gergely2 , G. Fekete1 , A. Ponyi1 . 1 Department of Pediatrics II., Semmelweis University, Budapest, Hungary, 2 Centrali Laboratory of Immunology, Semmelweis University, Budapest, Hungary
Objective: The antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent venous thrombosis or arterial occlusive events associated with elevated levels of antiphospholipid antibodies (aPL). Many neuropsychiatric manifestations have been described in association with APS. Methods: The presence of antinuclear-, anti-b2-glycoprotein I- and anticardiolipin antibodies were investigated in 60 consecutively selected children with epilepsy. Results: Almost 50% of our patients were ANA positive. 20% patients have moderate titer of ANA. Anti-C1q antibody was positive in 4 cases (6%), all of them are symptome-free considering renal manifestation of lupus. Interestingly only 1 child (1.5%) had aCL antibody, while 14% patients were LAC positive. Anti-b2-glycoprotein I-antibody positivity was not detected in this cohort of patients. Discussion: The clinical relevance of aPL tests in childhood are difficult to explain. In the present study obviously lower total prevalence of aPLs was observed in children with epilepsy than in the previously reported investigations (20−30%). The higher amount of LAC positive patients indicates that coagulation studies (LAC) should be included in the neuroimmunological assessment of suspected APS patients with epileptic disorders. The difference between the results of serological and LAC studies could be explained by the possible positivity of other non-investigated antibodies.
C. Cerminara, F. Refice, R. Bombardieri, M. Pinci, E. D’Agati, P. Curatolo *. Pediatric Neurology Unit, Neuroscience Dept., Tor Vergata University, Rome, Italy
IAP026 Central nervous system involvement in paediatric SLE: Do we need an MRI? E. Demirkaya4 , Y. Bilginer4 , D. Yalnizoglu1 *, K.K. Oguz3 , V. Isikhan2 , N. Besbas4 , A. Bakkaloglu4 , S. Ozen4 . 1 Hacettepe University Dept. of Pediatric Neurology, 2 Hacettepe University Dept. of Social Work, 3 Hacettepe University Dept of Radiology, 4 Hacettepe University Dept. of Pediatric Nephrology and Rheumatology, Ankara, Turkey
Juvenile idiopathic arthritis (JIA) represents the main form of arthritis affecting children. Epileptic seizures are uncommon in patients with JIA and are usually due to meningovasculitis. We describe two patients affected by JIA whit epileptic manifestations. Case 1: a 3-year-old girl affected by JIA and segmental myoclonic jerks, with focal epileptiform abnormalities on EEG. Good seizures control was reached with VPA. After a reactivation of JIA, epileptic manifestations reappeared. VPA was increased and actually, she takes VPA, MTX, Flubioprophen and cycles of corticosteroids and is seizurefree. Case 2: a 14-year-old boy affected by Juvenile Absence (seizure-free with Lamotrigine and VPA) and JIA with partial remission. We describe the first cases of association between JIA and idiopatic epileptic manifestations suggesting the possible role of the immunological system in the pathogenesis of some epileptic seizures. The presence of autoantibodies in epilepsy or seizure-associated disorders and the anticonvulsivant efficacy of immunomodulatory drugs might suggest a role of immunity in the pathogenesis of some epileptic disorders. In case 1 is therefore possible that the remission of epileptic manifestations is both due to an appropriate anti-epileptic drug and a good control of JIA.
Rationale: The diagnosis of neurological involvement in SLE is complex due to the wide spectrum of manifestations. We studied the neurological symptoms and signs in paediatric patients with SLE. Methods: Patients with SLE presenting to the paediatric nephrology and rheumatology clinic in a 3-month period in 2006 were evluated. MRI was obtained in all patients, MRA and EEG were performed in selected cases. The burden of disease was assessed by Symptom Distress Check List (SCL90-R) questionnaire. Results: The mean age at the time of evaluation was 15.8 (range = 6−22). The mean age at the time of diagnosis was 11.4 (range = 6−16). Four patients had obvious neurological signs and/or symptoms at onset or admission. Overall 2/3 of the patients had neurological symptoms and/or signs. MRI was abnormal in four patients who had overt neurological symptoms. According to the SCL-90-R questionnaire 4 patients had significant depression, two had significant anxiety. 37.5% of adolescents expressed a decline in school performance. Discussion and Conclusions: MRI is probably not required in patients who do not have any neurological symptoms or signs. The decline in school performance may be partially attributed to the loss of school days. Further studies may guide the need for more advanced neuroimaging studies.
IAP024 Juvenile idiopathic arthritis and epileptic manifestations: report on two cases