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P3-79 Case series of recurrent painful ophthalmoplegic neuropathy C.M.P.C.D. Peeters-Scholte. Department of Neurology, University Medical Center, Leiden, The Netherlands
Leiden
Objective: To describe 3 cases of children with recurrent painful ophthalmoplegic neuropathy (RPON), formerly called ophthalmoplegic migraine. Case description: Case 1: A 2,5-year-old male patient was admitted with right oculomotor nerve palsy with unilateral headache, mainly situated around the right eye with photophobia and phonofobia. No nausea or vomiting was present. During the attack, enhancement and thickening of the right oculomotor nerve was present on the MRI. No positive antibodies in CSF were found. Family history was positive for migraine. Later on he developed six times per year an RPON attack, often triggered by fever or infection. Migraine prophylaxis had no effect on the frequency of the attacks. Monthly IVIG administrations lowered the frequency and the intensity of the attacks. Case 2: An 11-year-old female patient was admitted because of severe headache and vomiting, followed by rightsided oculomotor paralysis. At the age of 5, 7 and 9 years old she also experienced episodes with ptosis and diplopia. MRI scan showed ictal enhancement and thickening of the cisternal segment of the right oculomotor nerve, interictally no abnormalities were seen. Because of the low frequency of attacks only corticosteroids were administered during attacks. Case 3: A 2year-old male patient was admitted after a period of fever and vomiting with progressive ptosis and mydriasis caused by right oculomotor nerve palsy with photophobia. Family history was positive for migraine. He was successfully treated with corticosteroids after MRI revealed right oculomotor enhancement. RPON attack frequency has to be awaited. Conclusion: RPON often start in early infancy with attacks of ptosis and diplopia, often triggered by infection or vaccination. In later years the oculomotor palsy is often preceded by migrainous headache. Although the exact etiology is still unclear, a demyelinating disorder is postulated, and IVIG therapy might be helpful in controlling attack frequency.
http://dx.doi.org/10.1016/j.ejpn.2017.04.1096 P3-80 Patients with Profound Intellectual and Multiple Disabilities (PMID) and access to the pediatric neurologist: An opportunity for Telemedicine? M. Hully, C. Brisse, M. Bredillot, R. Brault, Y. Lhermitte, C. Coiffier, A. Belorgey-Frain, M. Gaulard, S. Pik, P. Sellier, I. Fontaine, L. Baba Aissa, J. Bonheur, J.M. Pinard, C. Bellesme, I. Desguerre, T. Billette de Villemeur. Department of Pediatric Neurology, Necker Enfants Malades Hospital, Paris, France Objective: The healthcare project of PMID children is most often complex, involving both medico-social institutions (MSI) and pediatric hospitals (PH). The access to specialized secondary or tertiary consultations for example with a neuropediatrician is often limited, and mostly occurs in a context of emergency. In order to improve the healthproject of these children, with potential preventive measures, and to promote the cooperation between the different persons involved in that project, a telehealth program has been implemented (partially founded by the Regional Health Agency, ARS) between 9 MSI and 5 PH with a pediatric neurology department in Ile-de-France. We first identified the data concerning the follow-up in the pediatric
neurology department for the patients in those MSI. Methods: Each MSI transmitted the list of their patients with the identified neuropediatrician for each patient. For the two first pediatric hospitals involved in the project, we then identified how many patients had consulted a neuropediatrician in 2014. Results: In 2015, April the 1st, 422 children were followed in the 9 MSI. 118 of them were not followed in one of the pediatric neurology departments of the Ile-de-France. 192 patients were followed in one of the two first PH involved in the project. In 2014, 115 (60%) had met their neuropediatrician at least once. Conclusion: Our data confirm that specific neuropediatric consultations lack for those PMID patients, even when they have a neuropediatrician referent since 40% did not met the neuropediatrician at least once a year. The aim of this Telemedicine project is thus to improve the neuropediatric follow up of those patients, in order to implement potential preventive measures in those fragile patients, but also to promote interactions between the different actors involved in those complexe healthprojects. The global cost-effectiveness of such program will also be evaluated.
http://dx.doi.org/10.1016/j.ejpn.2017.04.1097 P3-81 Neurological appearance of celiac disease N. Olgac Dundar, D. Cavusoglu, O. Oztekin, P. Gencpinar, P. Arican, M. Baran. Department of Pediatric Neurology, Faculty of Medicine, Izmir Katip Celebi University, Izmir, Turkey Objective: Celiac disease may present as one or more neurological signs and/or symptoms. It may be associated with or complicated by neurological manifestations. We aimed to define the incidence of accompanying neurologic manifestations in children diagnosed celiac disease and interpret our outcomes compare with results of other studies in literature. Methods: The prospective study included 121 children diagnosed with celiac disease. The medical records of all patients were also reviewed. Results: In neurological evaluation, 38 (31.4% ) of the 121 celiac patients had a neurological finding including headache, migraine, vertigo, epilepsy, sensorimotor polyneuropathy, Down syndrome, Turner syndrome and a patient with subcortical white matter changes in MRI. Vitamin deficiency (vitamin B12 and/or vitamin D and/or folic acid) was statistically more common among patients with neurological manifestations. Conclusion: It is important to consider on clinical course of celiac disease different neurological manifestations may be presented.
http://dx.doi.org/10.1016/j.ejpn.2017.04.1098 P3-82 Reliability and validity of the Greek Version of BRIEF and BRIEF-SR S. Stabouli, E. Papadimitriou, D. Gidaris, A. Petralias, N. Printza, J. Dotis, K. Chrysaidou, E. Vargiami, M. Kyriazi, D. Zafeiriou. 1st Department Pediatrics, Aristotle University of Thessaloniki, Greece Objective: The Behavior Rating Inventory of Executive Function (BRIEF) completed by parents, and the Behavior Rating Inventory of Executive Function Self-Report Version (BRIEF-SR) completed by adolescents in complement to parent ratings, are scales that evaluate different aspects of executive function behaviors during child’s everyday life. This study aims to demonstrate reliability and validity of the Greek Version of BRIEF and BRIEF-SR. Methods: Healthy Greek children were administered the aforementioned
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questionnaires. A second identical questionnaire was administered for assessing test- retest reliability. Each subscale was separately evaluated for its psychometric performance according to established criteria. Acceptability, reliability, and internal validity analysis were conducted. Results: Parents of 59 children and adolescents (children’s age range 5-18 years), and 25 adolescents participated in this study. Seventeen % of the parents and 25% of the adolescents returned the second questionnaire. There was no missing data in all subscales. Cronbach’s alpha acceptable levels were demonstrated for BRIEF subscales ranging from 0.77 to 0.90. Similarly, Cronbach’s alpha levels were acceptable for BREF-SR subscales ranging from 0.70 to 0.91. Reliability was also supported by test-retest analysis for most of subscales of BRIEF (Intraclass Correlation Coefficient [ICC] ranging from 0.70 to 0.85, except for Monitor and Initiate subscales), and for all subscales of BRIEF-SR (ICC ranging from 0.70 to 0.85). For both BRIEF and BRIEF-SR individual subscale items’ correlations with the subscale score to which they belonged were greater than their correlation with the summary score of the other subscale. Internal validity by evaluating correlations between the global assessment item of each subscale and both summary subscale scores, showed Spearman correlation coefficients ranging from 0.30 to 0.53 for BRIEF, and 0.29 to 0.54 for BRIEF-SR subscales. Conclusion: The Greek versions of BRIEF and BRIEF-SR questionaires are both reliable and valid for the assessment of executive function in children and adolescents.
http://dx.doi.org/10.1016/j.ejpn.2017.04.1099 P3-83 Neurological involvement in hemolytic e Uremic syndrome: our experience at the children’s hospital regina margherita in turin G. Rosso, C. Canavese, M. Gandione, A. Tocchet, L. Peruzzi, R. Bonaudo, B. Gianoglio. Department of Pediatric Neurology and Psychiatry O.I.R.M., University of Turin, Turin, Italy Objective: The hemolytic-uremic syndrome (HUS) is a clinical triad of microangiopathic hemolytic anemia, impaired renal function and thrombocytopenia, primarily affecting pre- schoolaged children. Central nervous system involvement is a severe and not uncommon complication. Our purpose is to describe our records regarding neurological signs and symptoms, their management and outcome. Methods: we describe a small cohort of 10 patients with a diagnosis of hemolit-uremic syndrome (HUS) and neurological involvement admitted from year 2011 throughout 2016 at the Pediatric Hospital “Regina Margherita” in Turin. We analyze clinical features at the onset of HUS, treatment and outcome at the discharge and at follow up. Results: 34 HUS cases were identified. 10 out of 34 presented neurological involvement (30%), characterized by occurrence of seizures, consciousness alterations, dysarthria, dysphagia, nystagmus, focal deficit. 9 cases out of 10 presented prodromal diarrhea (D+HUS), 1 not (DHUS). Verotoxigenic E.Coli infection was documented in 5 out of 10 cases. EEG anomalies were found in 9 patients: 5 cases needed Antiepileptic drugs. MRI was performed in 8 cases and 4 of them showed abnormal findings. Plasma infusion was utilized in 8 of 10; 6 patients needed dialysis; 2 patients were treated with eculizumab. Conclusion: Neurological involvement in HUS is a severe complication. In our cohort 1 patient out of 10 had a poor outcome while 9 patients had a good outcome at the discharge and at follow up. This underlines the importance of attention on extra-renal manifestations in acute phase and the crucial role of a timely identification and treatment. Our records of neurological
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involvement and his course appear to be in line with reports from the literature.
http://dx.doi.org/10.1016/j.ejpn.2017.04.1100 P3-84 Imaging clues in the congenital cytomegalovirus infection Andrei-Dan Marinescu, Alice Dica, Oana Tarta-Arsene, Carmen Burloiu, Iuliu Bacos, Diana Barca. Alexandru Obregia Hospital, Pediatric Neurology Department, Romania Objective: Congenital cytomegalovirus (CMV) infection is the most frequent congenital infection, a real public health issue because its implications on psychomotor development, with a small percentage of symptomatic patients, the majority being asymptomatic at birth, some presenting later with various symptoms and brain abnormalities. The aim of our study is to emphasize the spectrum of imaging abnormalities through cases with neonatal diagnostic or late diagnostic, established retrospectively, important clues being offered by cerebral MRI - white matter abnormalities suggesting leukoencephalopathy +/- malformations of cortical development (MCD) and confirmation possible through PCR for viral DNA on dried blood spot (DBS) from the neonatal screening. Methods: 6 patients are presented e onset, admission, milestones, the neurological picture, the imaging. 2 children onset in infancy - congenital CMV infection; 4 cases at older age - 2 children at 5, respectively 14 years for progressive hearing loss, onset ~2 years, and 2 with congenital microcephaly, global developmental delay, pyramidal syndrome, pharmacoresistant epilepsy, leukoencephalopathy. Results: All children have abnormal imaging: periventricular calcifications, subcortical frontal/parietal micronodular lesions (CTs), while the MRI's showed static leukoencephalopathy, affecting the parietal and temporal regions, with cystic transformation, widening of inferior horns of the lateral ventricles, important signs of congenital CMV infection. The diagnosis was established prenatally in 1 case (positive mother serology), in the neonatal period in 1 case, retrospectively in 4 cases - on clinical and imaging (leukoencephalopathy+/- MCD), 2 confirmed by PCR on neonatal DBS. Conclusion: The appearance of static leukoencephalopathy +/MCD can guide diagnosis for congenital CMV infection. Thus, the role of MRI acquires a new value alongside classic role of CT in arguing this pathology. Taking into consideration the CMV diagnosis in cases with later presentation can avoid extensive, expensive investigations on metabolic/genetic line, the retrospective diagnostic being confirmed by PCR on neonatal DBS.
http://dx.doi.org/10.1016/j.ejpn.2017.04.1101 P3-85 Electroneurographic evaluation and early autonomic damage in diabetes mellitus type I (DMT1) adolescents C. Canavese, M.C. Bertello, C. Baietto, M. Giacobbi, A. Palmitessa, A. Tocchet, I. Rabbone. Dipartimento di pediatria e speciaita della Salute pediatriche, neuropsichiatria Infantile e Diabetologia, Citta e del ola Scienza di Torino, Italy Objective: Type 1 diabetes mellitus (DM) is one of the most common chronic diseases in children and diabetic neuropathy (DN) is its most frequent chronic complication. Objective of the study was to screen asymptomatic adolescents with DM. Methods: We evaluated patients with a DMT1 diagnosis of at least 5 years. All patients were treated with an intensified insulin regimen.