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For Epileptic - Egyptian Society of Child Neuropsychiatryescnp.net/PACNA 2016.pdfScientific Program 5 Wednesday 06/04/2016 Time Topic 10.30 – 13.30 Workshop 1 ( Learning Disabilities

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For Epileptic ChildrenFor Epileptic Children

For Epileptic ChildrenFor Epileptic Children

Wednesday06/04/2016

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Wednesday06/04/2016

Time Topic10.30 – 13.30 Workshop 1 ( Learning Disabilities & School

Under achievement )Prof Dr tarek omar, mD PediatricsProfessor of Pediatrics and Pediatric Neurology,Department of Pediatrics,Faculty of Medicine,Alexandria UniversityProf Dr rania abdou, mD PhoniatricsAss Professor of Phoniatrics,Phoniatrics Unit, ENT Department,Faculty of Medicine,Alexandria University

13.30 – 15.00 registration15.00 – 15.30 opening ceremony15.30 – 17.10 session 1: child Psychiatry Session

chair : anwar etribi, Hanan azoz, soha Ghobashi

15.30 – 15.50 : Childhood Concussion (Plenary Lecture):anwar etribi, Ain Shams University. Egypt15.50 – 16.10: Disruptive Mood Dysregulation Disorder ( DMDD) : A New Disorder to be Considered:Hanan azoz, Alexandria University, Egypt16.10 – 16.30: Borderline Personality Disorder ( BLPD ) Features Among Children and Adolescents: Soha ghobashi, Prof. of Psychiatry, Alexandria University. Egypt 16.30 – 16.50: Childhood Neurotic Traits: Heba essam abou el-Wafa, Alexandria University. Egypt16.50 – 17.10: Dieting as a Risk Factor in Eating Disorders:Wafaa mehelba, HIPH, Alexandria University ( President of the Medical Association for the Study & Management of Obesity “ MASMO “

17.10 – 17.30 Break

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Time Topic17.30 – 19.10 session2: Static encephalopathies: cerebral

Palsy - Stroke and movement Disorderschair: Bayoumi Gharib, Khalil el diwany, Waleed altwaijri

17.30 – 17.50: Treatable Neonatal Metabolic Epilepsies (Plenary Lecture): Waleed altwaijri, riyadh. President of Saudi Child Neurology Society. KSA17.50 -18.10: Outreach is Our Unique Way to Reach; A Regional Spasticity Model. nermin g el Khouly. King Fahad Specialist Hospital, Dammam, KSA18.10 – 18.30: Urological Problems in Cerebral Palsy and Hyper Kinetic Disorders: Haytham Badawe, Ass Prof of Pediatric Urosurgery, Alexandria University. Egypt18.30 – 18.50: Abnormal Head and Eye Posture & Movements: gamal asker, Assiut University. Egypt18.50 – 19.10: Neonatal and Childhood Stroke : ahmed raouf, ARRC, Egypt

19.10 – 19.30 Break19.30 – 21.00 session 3: infectious and immune Disorders

chair : emad Hammad, nuri M.shembesh, nagwa abdel Meguid

19.30 – 20.00: Autism Spectrum Disorders: Towards a Global: Consensus ?: (Plenary Lecture)Philippe evrard, Paris. France20.00 – 20.20: Role of Toxic Metals in Autistic Spectrum Disorders ehab ragaa, NRC. Egypt20.20 – 20.40: The Efficacy of “Son-Rise” Program for Development of Social and Communication Skills of a Sample of Children with Autism : tarek omar, Alexandria University. Egypt20.40 – 21.00 : Update on the Recent Phamacokinetics Used in the Management of Autism, azhar Daoud, Jordan University of Science and Technology, Amman. Jordan

THURSDAy07/04/2016

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THursday07/04/2016

Time Topic9.00 – 12.00 Workshop 2:

neuro-radiology Workshopneuroimaging for Pediatric neurologistssponsored by: Darweesh Diagnostic Imaging Centre (Darweesh Scan)• Prof. Dr. reda Darweesh, Professor of Diagnostic Radiology, Alexandria University.• Prof. Dr. abdel aziz elnekeidy, Assistant Professor of Diagnostic Radiology, Alexandria University.• Dr. ahmed el Beheiry, Lecturer in Diagnostic Radiology, Alexandria University.• Dr. mohamed akmal, Research Fellow of Diagnostic Radiology, Alexandria University.1- Basics of pediatric neuroimaging: -Imaging modalities and indications -Anatomical landmarks2- Brain development and congenital anomalies: A sad end to a happy dream(Including 3-5 clinical cases)3- Imaging of neuroplasticity in children4- MRS in inherited neurodegenerative diseases: Can we save these children? (Including 3-5 clinical cases)

12.00 – 13.20 session 4: epilepsy and epilepsy equivalentsPhilippe evrard, raidah alBaradie, Ibrahim shokry 12.00 – 12.20: Melatonin, from Neuroprotection to Sleep : (Plenary Lecture)Philippe evrard, Paris. France 12.20 – 12.40: Epileptic Encephalopathies : (Plenary Lecture)raidah S. alBaradie, King Fahd Specialist Hospital-Dammam, President Saudi Epilepsy Society. KSA

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Time Topic12.40 – 13.00: Focal Convulsions; What is Behind ?. omnia el rashidy, Ain Shams University. Egypt13.00 – 13.20: Sleep Study in Patients with Juvenile Myoclonic Epilepsy: Newly Diagnosed and Those on Valproate Drug:Sonia ibrahim elbhrawy, Lecturer of Neurology, Alexandria University, Egypt

13.20 – 13.30 Discussion13.30 – 14.30 session 5: Sanofi Satellite Symposium

chair: saher Hashem, Tarek Tawfik

Childhood Absence Epilepsy: tarek tawfik, Cairo University. EgyptEpilepsy and IQ: Saher Hashem, Cairo University. Egypt

14.30 – 15.00 Break15.00 – 17.20 session 6 : epilepsy and epilepsy equivalents

chair : Farouk Talaat, Mona raafat, nabil al agouz

15.00 – 15.20: Childhood Migraine: (Plenary Lecture)farouk talaat, Alexandria University. Egypt15.20 – 15.40: Growing up with Epilepsy: Seizure intractability & Cognitive outcome”: ismail S. mohamed, Pediatric Neurologist, IWK Health Center. Canada 15.40 – 16.00: Imitative of Epilepsy in Infants and Children: mona raafat, Ain Shams University. Egypt16.00 – 16.20: EEG Changes In Asthmatic Children:azza Kamal al-Shahawy, Tanta University. Egypt16.20 – 16.40: Bone mineral Status in Epileptic Children Receiving Different Regimens of Antiepileptic Drugs: DXA and Biochemical markers: Bothina Hasaneen, Mansoura University. Egypt16.40 – 17.00: Antiepileptic Medication in the Community: What are the Hazards?. noha tharwat. Mansoura University. Egypt 17.00 – 17.15: Looking beyond seizure freedom, mohamed nowair, Clinical pharmacist, Medical Affairs manager. Clavitapharma Sponsered Symposium

17.15 – 17.30 Discussion

FRIDAy08/04/2016

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FrIday06/04/2016

Time Topic9.00 – 12.00 Workshop 3: Botulinum toxin a in the

treatment of children with cerebral Palsy Workshop instructors:1. Prof Dr tarek omar, Professor of Pediatrics and Pediatric Neurology – Faculty of Medicine – Alexandria University - Egypt2. Dr Hayma moustafa – Fellow in Physical Medicine, Rheumatology and Rehabilitation - Faculty of Medicine – Alexandria University - EgyptWorkshop coordinators:1. Dr islam Yousry – Pediatric Specialist – Alexandria University Hospitals – Faculty of Medicine – Alexandria University - Egypt2. Dr islam el Sayed – Pediatric Physical Therapist and – Ras El Teen General Hospital – Alexandria – Egypt

12.00 – 13.30 Prayer time13.30 – 14.10 task force for national guidelines of the Use of

Botulinum toxin a in the treatment of cerebral palsy in children in egypt

ahmed r. a., gouda i. Y. a., fathy K., mahran m. a., el-Sobky m. H. a., el-Sherbini m., gadallah n. a., mostafa S. Y., omar t. e. i., abdel ghany W. a.

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Time Topic14.10 – 16.30 session 7: neuro-genetic and neurometabolic

Disorderschair : azza shahawy, Tarek Omer, Ismail sadek, Mahmoud abdel sayed

14.10 – 14.30 : Impact of Consanguinity on Dysmorphic Syndromes: (Plenary Lecture) nagwa abdel meguid, NRC. Egypt14.30 – 14.50: A Clinical Profile of Mucopolysaccharidosis Type One ( MPS I) in Libyan Children: nuri m.Shembesh, Prof. of Pediatrics and Pediatric Neurology, Head of Pediatric Department Benghazi University Benghazi. Libya14.50 – 15.10: Acute Ataxic Syndromes in Children: nabil al agouz, Azhar University. Egypt 15.10 – 15.30: Profile of Tuberous Sclerosis Complex in Egyptian Children in Alexandria: tarek e.i.omar, Alexandria University. Egypt5. 15.30 – 15.50: Pediatric Neurology Service and Profile of Neurological Disorders: Outpatient Department – Sudan: inaam n mohamed, University of Khartoum, Head Neurology Unit – Gafer Ibn Auf Specialized Hospital. Sudan15.50 – 16.10: Patterns of Pediatric Neurology Disorders in al Azhar University Hospitals: Shora Youssef, Al Azhar University, Egypt16.10 – 16.30: Molybdenum Cofactor and Isolated Sulphite Oxidase Deficiencies: Clinical and Molecular Spectrum Among Egyptian Patients: maha S. Zaki, NRC. Egypt

16.30 – 17.00 Break

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Time Topic17.00 - 18.20 session 8: Hot topics in Pediatric neurology

chair : shoura youssef, Gamal asker, ehab ragaa

17.00 – 17.20: Clinical Presentations of Pediatric Multiple Sclerosis: (Plenary Lecture) nahed Salah el Din, Ain Shams University, Egypt17.20 – 17.40: Pattern and Outcome of Acute Disseminated Encephalomyelitis (ADEM) in Sohag University Hospital : abdelrahim a Sadek, Sohag University. Egypt17.40 – 18.00: Griscelli Syndrome (GS): Clinical and MRI findings, maher Khalifa, Pediatric Neurology unit; Dr Erfan & Bagedo general Hospital. KSA18.00 – 18.20: Autoimmune Encephalitis: Separate Entity in a New Era, noha tharwat, Mansoura University. Egypt

18.30 - 19.30 session 9 : attention Deficit Hyperactivity Disorder (aDHD)chair : Fathy afify, Hussein abdel dayem, Magdy Khalaf

18.30 – 18.50: ADHD: fathy afify, azhar University, Egypt 18.50 – 19.10: ADHD: Update Protocol for Treatment: Hussein abdel Dayem, Alexandria University, Egypt19.10 – 19.30: Quality of life in ADHD Children: reham okasha, Police Hospital, Alexandria, Egypt

Pacna 2016 ABSTRACTS

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pAcnA 2016 AbsTrAcTsWorkshop 1Introductory Course on School Underachievement and Specific Learning DifficultiesThemeThis course is intended to give junior pediatricians an introduction on understanding why do children underachieve at school with special reference to specific learning difficulties and their management.specific objectivesBy the end of the course, participants will be able to:1. Recognize causes behind scholastic under-achievement among children2. Understand difference between developmental and academic learning disabilities3. Identify terms, causes and classifications of learning disabilities4. Discuss diagnostic dilemma regarding learning disabilities5. Appreciate the role of multi-disciplinary team approach in assessment and management of learning disabilitiescourse Topics:1. Introduction2. Definitions and Terminology3. Classification and Types: Reading – Language – Written expression – Mathematics4. Characteristics of children with learning disabilities5. Etiologic Considerations6. Epidemiology7. Diagnosis and Differential Diagnosis8. Co-morbidity9. Multi-disciplinary Assessment Team10. Managementcourse instructors:1. Prof Dr tarek omar, mD PediatricsProfessor of Pediatrics and Pediatric Neurology, Department of Pediatrics, Faculty of Medicine, Alexandria University2. Prof Dr rania abdou, mD PhoniatricsAss Professor of Phoniatrics, Phoniatrics Unit, ENT Deparment, Faculty of Medicine, Alexandria University

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Topic insTrucTor Time1. Introduction2. Definitions and Terminology

Prof dr Tarek Omar 30 minUteS

3. Classification and Types: Reading – Language – Written expression – Mathematics

Prof dr rania abdou 30 minUteS

4. Characteristics of children with learning disabilities5. Etiologic Considerations6. Epidemiology

Prof dr Tarek Omar 30 minUteS

7. Diagnosis and Differential Diagnosis8. Co-morbidity9. Multi-disciplinary Assessment Team

Prof dr Tarek OmarProf dr rania abdou

45 minUteS

1. Management Prof dr Tarek OmarProf dr rania abdou

45 minUteS

course AgendA

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clinicAl presenTATions of pediATric mulTiple sclerosisnahed Salah-eldin, mD; Hala al-Khawas, mD; Heba Hamed, mD; and mohamed el-ShafeiBackground and purpose: pediatric onset multiple sclerosis (POMS) is increasingly recognized. The aim of this study is to identify the typical presentation of MS in a sample of Egyptian children.methods: A total of 20 subjects with onset of MS before age 18 years of age were retrospectively included in the study. Case records were reviewed for symptoms at disease onset, cerebrospinal fluid (CSF) findings, magnetic resonance imaging (MRI) and Evoked potentials (EPs) at the first demyelinating event.results: 20 % of patients were males and 80% were females. One patient (5%) was under the age of six, 3 patients (15%) were aged from 7-12years and 16 patients (80%) in the age from 13-18 years. As regard the first presentation; 6 (30%) of patients had sensory symptoms, 5 (25%) had motor symptoms, 1 (5%) had visual symptoms (diplopia and blurred vision), 4 (20%) had optic symptoms, 3 (15%) had sphincteric symptoms, 3 (15%) had ADEM and 2 (10%) had cranial nerve affection (mainly facial nerve and oculomotor palsy). With 7 (35%) of patients had monofocal presentation and 13 (65%) had polyfocal presentation at disease onset.conclusion: Awareness of pediatric MS symptoms and age dependent risk for MS might improve early diagnosis of MS in children and adolescents.nahed salah eldinProf. of neurology Ain Shams Univeristy

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pATTern And ouTcome of AcuTe disseminATed encephAlomyeliTis (Adem) in sohAg universiTy hospiTAlabdelrahim a Sadek*, mostafa ashry mohamed, marrwa ibrahim; *assistant Professor and Head of Pediatric neurology Unit, Sohag University Background:

Acute disseminated encephalomyelitis (ADEM) is an autoimmune disease marked by widespread attack of inflammation in the brain and spinal cord. It typically damage myelin causing destruction of white matter. It is often triggered following viral infection or vaccination. aim of the studyOur study aimed to delineate ADEM cases , their clinical profile and outcome.Patients and methods:

All cases had ADEM clinically and radiologically were included in our study resultsDuring the period of the study 18 Child were diagnosed as acute disseminated encepalomyelitis syndrome by clinical examination andneuroradiological studies (MRI Brain). The mean age of the studied group was 6.7 ±5.56 years; there was no significant difference regarding sex as 10 children (55.56%) were males and 8 (44.44%) were females Delayed motor milestones were noted in 2 children (11.11 %), as well as delayed speech development and sphincteric development was also noted in the same number. Regarding the complaint we found that disturbed conscious level was the main presenting complaint detected in 15 children (83.33%) whereas convulsions were the main complaint in 2 cases (11.11 %). Paresis and limb weakness was the complaint of one case (5.56%). There was history of symptoms of viral or bacterial infection in 13 cases (72.22%). Only two cases had GCS less than 6 (11.11%), nine cases (50.00%) had GCS from 6 to 10, while 7 cases (38.89%) had GCS ranging from 11to 14. No case of the studied populations was presented fully conscious. 6 cases had Quadriparesis (33.33%), 6 cases had Hemiparesis (33.33%), 5 cases were free (27.78%) and monoparesis was found in one case (5.56%).It was found that 5 cases had normal muscle tone (27.78%) while 8 cases were flaccid (44.44%) and 5 cases were spastic (27.78%). Reflexes were normal in 4cases (22.22%), while hyporeflexia was found only in one case (5.56%). Hyperreflexia was the predominant sign that was found in13 cases (72.22%).There were 7 cases didn’t have sphincteric affection (38.89%), while it was affected in 11 cases (61.11%).15 cases suffered from convulsions (83.33%), only 3 cases didn’t develop convulsions (16.67%) 11 cases received corticosteroids at the first week of symptoms(61.11%), four cases only received corticosteroids at the first day of symptoms (22.22%) and 3 cases

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didn’t receive corticosteroids until the first week had passed (16.67%). As regard Immunoglobulin; only 5 cases received immunoglobulin at the first week of symptoms (27.78%),4 cases received it after the first week (22.22%) and 9 cases didn’t receive immunoglobulin at all (50.00%). MRI for all patients showed demyelination patches that were widespread in 9 cases (50.00%), mainly subcortical in 5cases (27.78%), at the central white matter in 3 cases (16.67%) and in the cortical gray-white junction in one case (5.56%).The size of those demyelination patches was from 5 mm to 5 cm in 15 cases (83.33%), more than 5 cm in 2 cases (1l.l1 %) and less than 5 mm in one case (5.56%).The distribution of demyelination, patches was typically bilateral, (100%) in all the 18 cases. After the course of treatment had finished, 8 cases completely cured (44.44%), the other 8 cases cured with remaining neurological deficit (Motor deficit or convulsions) (44.44%). Death was the fate of two cases, one of them died due to respiratory failure following aspiration pneumonia and the other had central respiratory suppression. After 3 months 13 cases were fully conscious (81.25), while 3 cases (18.79%) had conscious level ranging from (11-14). After 6 months another case gained its conscious level to make the total number of improved patients 14 case (87.50%) while 2 cases still remained disturbed conscious level ranging from (11-14) (12.50%)According to Stanford -Binet Intelligence Scale IQ was done to the studied cases and was noticed that: After 3 months of illness: 8 cases (50.00%) were below average, 3cases (18.75%) had moderate mental retardation (MR), 2 cases were mild MR (12.50%), one case had sever MR, one case was profound MR and one case was super average (6.25%)After 6 months, 8 cases (50.00%) were below average, 4 cases were mildMR (25.00%), 3 cases were super averge (18.75%), and one case had profound MR (6.25%).

The efficAcy of “son-rise” progrAm for developmenT of sociAl And communicATion skills of A sAmple of children WiTh AuTismomar tei*, abou Zeid maH**, ibrahim mHm****tarek el-Sayed ismail omar Professor of Pediatrics and Pediatric Neurology, Faculty of Medicine, Alexandria University, Egypt.**medhat abdel Hamid abou Zeid Professor and Head of Department of Psychology, Faculty of Arts, Alexandria University, Egypt.***mohamed Hassan morsi ibrahimClinical Psychologist, Department of Psychology, Faculty of Arts, Alexandria University, Egypt.For Correspondence: The first Author’s Email: [email protected]

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abstract:The Son-Rise Program (SRP) is an intensive, child-centered parent-mediated approach for autism intervention. It incorporates strategies to promote child-initiated social interactions. The program involves parent training as a core element in its implementation. Furthermore, the program is intended to be implemented as a home-based program. This study examined The Efficacy of “Son-Rise” program for development of social and communication skills of a sample of children with autism. In the present study, parents of 10 children with autism were enrolled in an intensive training on Son-Rise program techniques for 24 days that included 7 consecutive stages of the program: program overview (2 days), start up program (5 days), maximum impact (5 days), new frontiers (5 days), preparation of the individual program (2 days) and intensive program (5 days). Children were aged between 3 and 10 years and they were 9 boys and one girl. Children were evaluated before and after the training program using Childhood Autism Rating Scale (CARS). In addition, specific evaluation form was created using the four components of the Developmental Social Model of Son-Rise Program: Eye Contact, Communication, Interactive Attention Span and Flexibility and was used to evaluate the children before and after implementation of the intensive program (last stage). A three-month’ follow up evaluation was done using the same tools to assess sustainability and short-term outcome of the program. An age and sex – matched control group was included in the initial evaluation and follow up evaluation using Childhood Autism Rating Scale (CARS). The study showed significant changes between the pre-and post-treatment CARS scores among the study group as well as between the study group and the control group. Also, significant improvement was observed in the study group with regard to the four components of the social developmental model of the program. In addition, follow up CARS scores were significantly different during the follow up evaluation from the initial evaluation which confirms sustainability of the effectiveness of Son-Rise Program on the short term. Parents who implemented the skills of SRP reported significant improvements in communication, sociability, and sensory and cognitive awareness in their children. These findings suggest that SRP is an important and effective training program for children with autism with its unique principles that stress on the role of parents in facilitating social communication with their children

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profile of Tuberous sclerosis compleX in egypTiAn children in AleXAndriAtarek e.i.omar* elBeheiry a. ahmed **, Hussein m. Haytham****tarek el-Sayed ismail omarProfessor of Pediatrics and Pediatric Neurology, Faculty of Medicine, Alexandria University, Egypt.**ahmed adel elBeheiryLecturer of Radio-diagnosis, Faculty of Medicine, Alexandria University, Egypt.***Haytham mohamed HusseinResident Pediatrician, Abou Kir General Hospital, Ministry of Health, Egypt.For Correspondence: The first Author’s Email: [email protected].

Tuberous sclerosis complex (TSC) is a genetic multisystem disease which its clinical expression is highly variable so that the true prevalence of the disease remains unknown though it is estimated to affect one in 10,000 births. It affects heart, kidneys, eyes, lungs and skin, also it affect the nervous system in the form of seizures, developmental delay and changes in the behavior. It may present at first year of life or may present later in life, it could misdiagnosed for years. Thirty Four Egyptian children with TSC were evaluated using their prospective medical records. Initial presentation and subsequent follow up was described. Description of diagnostic methods was described. Comparison of clinical findings, course and outcomes was presented. The study revealed that the male cases were 23 cases (68%) and the female cases were 11 cases (32%). The cases having positive consanguine parents were 10 cases (29%). The most common type of seizure was epileptic spasms which was detected in 15 cases at early childhood (44%). The most common extra nervous system affection was the heart with 10 cases (29%). This study confirms that the TSC is a disease with multisystem presentations. Successful management necessitates strict follow up and management plan with muli-disciplinary team approach. The long term follow up will depend on the complexity of system affection.

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groWing up WiTh epilepsy: seizure inTrAcTAbiliTy & cogniTive ouTcomeismail S. mohamed,Prof. Pediatric Neurologist, IWK Health Center. Canada

Epilepsy affects approximately 1-2% of children with a favourable outcome in terms of seizure control in most children. In the mature brain, epileptic activity can disrupt the neural networks involved in various cognitive processes. In children, epileptogenesis occurs in a period when the cortex is still maturing and consequently may interfere with normal cerebral development. Academic difficulties and cognitive impairments are well known in children with epilepsy refractory to medical treatment. More recently, neuropsychological deficits were identified in new-onset epilepsy while academic achievement was still unaffected suggesting that there is a window for intervention to ameliorate the impact of epilepsy on school performance. Moreover, a group of children experience progressive cognitive decline secondary to the epileptic process and are referred to as the epileptic encephalopathies. When and how the processes of epileptogenesis disrupt language and cognitive network development is not well understood. This talk will review the evidence of cognitive impairment in epilepsy and the potential impact of early treatment.

disrupTive mood dysregulATion disorder ( dmdd) : A neW disorder To be consideredHanan azoz, Alexandria University, Egypt

Disruptive mood dysregulation disorder ( DMDD) is a new diagnosis in field of mental health. It is added to DSM V mental illness. Children with DMDD have severe and frequent temper tantrums that interfere with their ability to function at home, in school or with their friends. Onset must be before age 10 years, and not below age of 6 years and approximately half of children with severe, chronic irritability will have a presentation that continues to meet criteria for the condition 1 year later. Rates of conversion from severe, non episodic irritability to bipolar disorder are very low but those children are at risk to develop depressive and/or anxiety disorders in adulthood. Differentiation of DMDD from bipolar disorder, oppositional defiant disorder and attention deficit hyperactivity disorder requires particularly careful assessment

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molybdenum cofAcTor And isolATed sulphiTe oXidAse deficiencies: clinicAl And moleculAr specTrum Among egypTiAn pATienTsmaha S. Zaki (1)*, Laila Selim (2), mohamed abdel Hamid (3), Hala t. eL-Bassyouni (1), mahmoud Y. isaa(1), iman mahmoud (2), Samira ismail (1), mariane girgis (2), abdelrahim a Sadek(4), catherine rehder (5), Joseph g gleeson (6).(1) Clinical Genetics department, Human Genetics and Genome research division, national research Centre, Cairo, egypt, 12311.(2) Pediatric department, neurometabolic Clinic, Cairo university, Cairo, egypt.(3) Medical Molecular Genetics department, Human Genetics and Genome research division, national research Centre, Cairo, egypt.(4) Pediatric neurology department, Faculty of Medicine, sohag university, sohag, egypt. (5) department of pathology, duke university, durham, nC27708, usa.(6) department of neurosciences and Pediatrics, Howard Hughes Medical Institute, university of California, san diego, Ca 92093, usa

*Corresponding Authormaha S Zaki (mD, PhD)Clinical Genetics Department, Human Genetics and Genome Research Division, National Research Centre, Cairo, Egypt, 12311.email [email protected]

abstractBackground: Molybdenum cofactor deficiency (MoCD) and isolated sulfite oxidase deficiency (SOD) are rare autosomal recessive devastating inborn errors of sulfur-containing amino acid metabolism with closely similar phenotype. the aim of the work is to elucidate the clinical, radiological, biochemical and molecular findings among Egyptian patients. methods: This study included 9 patients derived from 9 unrelated consanguineous families (6 had MoCD and 3 with SOD). The diagnosis in 8 cases was in the first few months of life based on clinical and radiological findings and confirmed by biochemical tests and targeting gene sequencing of MCOS1, MCOS2, MCOS3, GPRN genes and SUOX gene, in MoCD and SOD, respectively. A single case with atypical presentation was identified by whole exome sequencing.

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results: They were 6 females and 3 males and positive family history of similar deceased sibs in 6 of them. The clinical presentations in 8 cases were neonatal intractable seizures, profound neurodevelopmental delay, microcephaly, axial hypotonia, peripheral spasticity, severe hyperkeplexia and swallowing dysfunction. The late manifested case was started at 6 months after high fever with sudden deterioration and development of epilepsy, axial hypotonia and extrapyramidal manifestations. None of our patients had lens sublaxation and a single patient had hypertrophic cardiomyopathy not reported in the literature. Brain MRI showed the characteristic multiple severe volume loss and subcortical cysts similar to hypoxic ischemic cystic encephalomalacia in all cases except the late presented case, where altered signal of basal ganglia, dentate nucleus and cerebellum white mater were prominent. Novel mutations were in 3 cases with SOD and the single case with MOCS2 gene mutation. Conclusion: Herein we described 9 families with these very rare metabolic errors; one of the largest series reported from same ethnic group. Ascertained 3 families with SOD (37.5%) might point to reconsider the frequency of this disorder especially among our population. To the best of our knowledge, this is the first report from Egypt and Africa.

Keywords: Molybdenum cofactor deficiency, sulfite oxidase deficiency, microcephaly, brain MRI, MOCS1 gene, SUOX genes.

eeg chAnges in AsThmATic childrenazza Kamal al-ShahawyProfessor of Pediatric Neurology, Faculty of Medicine, Tanta University.

aBStractintroduction: Bronchial asthma is a common chronic inflammatory disease of the airways characterized by variable, recurring symptoms and variable degree of airflow obstruction. Symptoms include wheezing, coughing, chest tightness, and shortness of breath. Asthma is clinically classified into mild intermittent, mild moderate and severe persistent asthma. Asthma may also be classified as atopic or non-atopic. aim of work: is to study the possible changes in electroencephalogram in asthmatic children. Subjects and methods: This study was conducted on 50 children suffring from bronchial asthma aged from 6-12 years who were attendants of the Pediatric Chest Unit of Tanta University Hospital. EEG was done for all children. results: In the studied asthmatic children, 8 children (16%) had abnormal EEG. conclusion: Bronchial asthma and epilepsy may be two related paroxysmal disorders.

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borderline feATures in children And AdolescenTsDr Soha abdel Latif ghobashyProf of Psychiatry-Neuropsychiatry DepartmentFaculty of Medicine-Alexandria University

There is a debate in Literature covering Personality Disorders which involve age criteria for diagnosis. Studies have consistently found traits for Borderline personality among children and adolescents, however, couldn’t mark the diagnosis as the subjects didn’t match the age which was designated in many classifications as 18years. This talk will be demonstrating this debate with emphasis on the different traits and features considering the developmental stage and nosology

bone minerAl sTATus in epilepTic children receiving differenT regimens of AnTiepilepTic drugs: dXA And biochemicAl mArkersBothina Hasaneen, Mansoura University. Egypt

Background: Long-term antiepileptic therapy is known to alter bone metabolism in children but still few comparable data are available. Aim of this study is to asses bone mineral status in children with epilepsy, by using dual-energy x-ray absorptiometry (DXA) and biochemical markers. Patients and methods: Study conducted at Mansoura University Children Hospital (MUCH), from January 2014 to June 2015. In this study, we had 152 participants with ages 3-13 years, 70 of these children were diagnosed with epilepsy and others were controls. Children were classified into different groups according to regimen and duration of AEDs. In our study, the biochemical and DEXA markers were studied for total cases versus control group. results: we found that the serum level of calcium, phosphate and ALP were significantly low (p>0.05) in total cases versus control. We found that the serum level of parathrmone (PTH) is significantly high (p>0.05) in total cases versus control. Regarding the DEXA markers there were significant decrease of BMD and Z-score for the total body and lumber area in the total cases versus control (p>0.05). conclusion: Our study showed that, all AEDs (new and old) affect bone mineral status in children receiving therapy more than 6months. Children receiving multiple AEDS are more prone to altered bone mineral status especially with long duration of therapy.Key words: bone mineral status, epilepsy, children, DXA, anti-epileptic drugs

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pediATric neurology service And profile of neurologicAl disorders: ouTpATienT depArTmenT - sudAn*inaam n mohamed , maha elseed, ahlam HamedAssociate professor University of Khartoum, Head Neurology UnitGafer Ibn Auf Specialized Hospital

aBStract:Background: Neurological disorders account for more than 20% of the world’s disease burden with a greater majority of people affected living in Africa. There is no available data from Sudan reflecting the magnitude of the neurological disorders and disabilities in the pediatric age group. This study aims to evaluate the pattern of neurological disorders among Sudanese children.

Patients and methods: This is a retrospective survey of children with epilepsy and other neurodisability disorders who were assessed and followed up in the pediatric neurology outpatient clinic, during the period from January 2007-August 2013. The data of 9600 patients was revised and classified into 24 categories according to the distribution of their neurological presentation.

results: A total of 6019 patients were included in the study. Their age was between 3months and 18 years and male to female ratio was 2:1. The majority of the patients had epilepsy that amounted to 52.8%. That was followed by Cerebral Palsy (19.1%), Congenital Anomalies of the Central Nervous System (6.2%), Neuromuscular Disorders (3.2%), Stroke (2.4%), Ataxia and Movement disorders (1.9%) and assumed genetic Syndromes (1.2%). Demyelinating disorders, Headache, Neurodegenerative, Mitochondrial, Metabolic, Charcot Marie Tooth, Behavioral disorders, in addition to other accounted for the other 14.4%.

conclusion and recommendations: Neurological disorders constitute a major cause of chronic morbidity in pediatric age group. Appropriate allocation and distribution of relevant resources, and other recommendations will be addressed in this presentation.

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Workshop 3 boTulinum ToXin A in The TreATmenT of children WiTh cerebrAl pAlsyobjective:To introduce pediatricians, physiatrists and physical therapists to the concept of diagnostic assessment of children with cerebral palsy and the use of (Botulinum Toxin A) BTX-A in its treatment focusing on selecting eligible patients, recognizing the injection technique, identifying post-injection rehabilitation, identifying the role of adjuvant therapies and evaluating outcome of BTX-A therapyparticipants:Pediatricians, physiatrists and physical therapists working in the field of physical disability or taking care for children with cerebral palsy and their families.Workshop Instructors:1. Prof Dr tarek omar, Professor of Pediatrics and Pediatric Neurology – Faculty of Medicine – Alexandria University - Egypt2. Dr Hayma moustafa, Fellow in Physical Medicine, Rheumatology and Rehabilitation - Faculty of Medicine – Alexandria University - EgyptWorkshop coordinators:1. Dr islam Yousry, Pediatric Specialist – Alexandria University Hospitals – Faculty of Medicine – Alexandria University - Egypt2. Dr islam el Sayed, Pediatric Physical Therapist and – Ras El Teen General Hospital – Alexandria - Egypt

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TAsk force for nATionAl guidelines of The use of boTulinum ToXin A in The TreATmenT of cerebrAl pAlsy in children in egypTahmed r. A.1, gouda i. y. A.2, fathy K.3, mahran m. A.4, el-Sobky m. H. A.5, el-Sherbini m.6, gadallah n. A.4,mostafa S. y.7, omar t. E. I. 2, abdel ghany W. A.4

1 Armed Rheumatology & Rehabilitation Center of Police General Hospital2 Alexandria University3 Mansoura University4 Ein Shams University5 Cairo University6 National Institute of Neuromotor System7 Al Azhar UniversityCerebral palsy is one of the leading causes of childhood disability worldwide, with the greatest burden found in the developing countries. It is now the most common physical disability in children following eradication of poliomyelitis. Prevalence of cerebral palsy (CP) has been found to rise in the last 40 years, with its management becoming an issue of considerable Socio-economic concern worldwide. In an Egyptian study, the prevalence rate of cP is 2.04/1000 (El-Tallawy et al., 2010) of which 65% are of the spastic type. Based on these numbers, it is estimated that there are around 23731 children with cerebral palsy are currently present to which another 6575 new cases will be added annually. There are many challenges facing those children and their families during the journey of treatment and rehabilitation. Botulinum toxin A has recently been used as a treatment for cerebral palsy and has been well established as a standard treatment modality for spasticity in those children (Awaad et al., 2004). However, use of BTX-A in treating spasticity in children with cerebral palsy in Egypt has been limited primarily due to its cost; being out of coverage by the health insurance for children and out of coverage by the Ministry of Health Treatment Decrees and secondarily due to lack of training and experience in its use for this group of patients. An initiative was raised by Tarek Omar; Professor of Pediatrics and Pediatric Neurology; Faculty of Medicine, Alexandria University to gather all experts dealing with cerebral palsy and using Botulinum Toxin A injection in treating spasticity of those children to meet for discussion and consensus on its use. The overall objective for this gathering is to Improve quality of care of children with cerebral palsy in Egypt through establishment of a task force evidence based national guidelines on the use of Botulinum Toxin A in the Treatment of those children.

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An invitation was sent to all experts in Egyptian Universities who are known to use BTX-A in children. Invitation was extended to those who treat children with cerebral palsy but not necessarily using BTX-A. By the start of November 2014, out of a total number of 19 invitees, 9 respondents were recruited from 5 Egyptian Universities as well as one institute belonging to Ministry of Health and another one belonging to Ministry of Interiors. A net search was done to gather all recent references of international guidelines and publications on using BTX-A in treatment of spasticity in children with cerebral palsy during the last 10 years (2004 – 2014). All collected references have circulated by E mail to participants for revision. A scheduled two days’ meeting for the group was agreed upon by the end of November 2014. During the first day, groups reviewed all references according to their disciplines in the assigned groups. Summarization of the evidenced recommendations was done. On the second day, the two groups presented their summaries for the assigned tasks. Then, finally the first draft of the guideline document was designed and written together with the task force charter for the mission. At the end of the meeting, a timetable was established to execute the final draft of the guideline document by January 2015.

ouTreAch is our unique WAy To reAch; A regionAl spAsTiciTy modelAuthors – nermin g el Khouly.MD1, raidah albaradie.MD2, Khaled Darawil.MD11 Department of physical medicine and rehabilitation,King Fahad Specialist Hospital2 Department of Pediatric Neurology, King Fahad Specialist Hospitalcase Diagnosis : Cerebral Palsy (CP) with Spasticity and Gait disturbance

case description:Cerebral Palsy is considered a neurological disorder caused by a non-progressive brain injury or malformation that occurs while the child’s brain is under development, manifested mainly by variable degree of movement and coordination difficulties. The prevalence of CP is high in developing countries. CP is a major cause for gait disturbance and immobility in children. Rehabilitation of children with CP is under developed due to the limited resources and the lack of availability of skilled specialistsin pediatric rehabilitation and pediatric neurology. In this communication the authors presents a case of CP with gait abnormalities and poor functional abilities, and how the prognosis can be improved by accessing a colborativeoutreach regional program.

methodThe clinical scenario, supported by the diagnostic work up, will be discussed. A full review of the rehabilitation course at a tertiary neuroscience center will be depicted.

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discussion:In this case discussion the authors will describe the various physical and cognitive impairments associated with CP, and will review the assessment of these impairments and illustrate how the outcome can beimproved when rehabilitation of certain selected complex CP cases is coordinated by the right multi-professional teamapproach.conclusion:CP can cause various patterns of spasticity and gait abnormalities, and this can lead to contractures, immobility and various other forms of physical and mental disabilities especially if it is not addressed at an early stage. There is currently a huge gap in this service, and the need for anearly multi-professional intervention is never been as greater as nowadays. We intend from this paper to show how by developing a regional model it became possible for these patients to access the expertise of the multidisciplinary team, access the right treatment and the impact this will have on their recovery and their families at an early stage of their life.

griscelli syndrome (gs): clinicAl And mri findingsKhalifa m*, arab n**, Zeinhom f***Pediatric Neurology unit; Dr Erfan & Bagedo general Hospital

aBStract:Griscelli syndrome is a rare autosomal recessive disorder due to mutations in MyO5A, RAB27A, or MLPH. It was initially described by Griscelli and Prunieras, France in 1978. This defect results in pigmentary dilution of the skin and the hair (silver hair) as an isolated feature in GS type3, or with combined T-cell and B-cell immunodeficiency (GS type2) or neurologic signs (GS type1). Hemophagocytic lymphohistiocytosis may occur as a complication in GS type2 and is considered the major cause of mortality. Here, we describe a 2.5 years old child presented with repeated lower respiratory tract infections, convulsions and deteriorating neurologic manifestations. He had silver gray hair and eye brows, neurologic manifestations. Differentiation from Chediak-Higashi syndrome and Elejalde syndrome was warranted due to overlapping features. Laboratory studies showed immune deficiency disorder and in one stage peripheral blood pancytopenia, with absence of characteristic granules characteristic of Chediak-Higashi which was excluded. Elejalde syndrome patients do not have immunologic abnormalities. Both CT and MRI are used to assess GS. Usually, findings are normal at birth. When the disease manifests, imaging findings are abnormal. Skin biopsy showed characteristic hyperpigmented basal melanocytes and sparse pigmentation of adjacent keratinocytes. Different disorders and even subtypes of GS influence prognosis. Mortality is often related to uncontrolled hemophagocytic syndrome rather than infections due to immune deficiency. Bone Marrow Transplantation is the only curative option though it does not improve neurological damage.

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eeg chAnges in Adhd children And AdolescenTs: A sAmple of egypTiAn populATionashour Hala1, Haikal amal1, Bothina Hasaneen2, tamer m. Belal3, almogy ahmed1, ibrahim ibtihal1, riad m. elsayed21Department of Psychiatry, 2Department of Pediatrics/ Neurology Unit, 3Department of Neurology, Faculty of Medicine, Mansoura University, Mansoura, EgyptaBStract:Background: Attention-Deficit/Hyperactivity Disorder (ADHD) has become one of the most common neurodevelopmental and psychiatric disorders of childhood. Electroencephalographic (EEG) changes in ADHD children still an area of debates.

aim of study: To assess the pattern of EEG abnormality in ADHD children and adolescent.

Patients and methods: we studied 109 children, with the age from 6-16 years (69 with ADHD versus 40 normal controls). ADHD diagnosis was done using DSM-IV-TR criteria. EEG, Conner’s rating scale, and IQ test were done for all children. We included only drug naive subjects with IQ > 80 assessed by Stanford Binet-IV. Current cases with convulsive disorders & other neuropsychiatric disorders were excluded.

results: EEG abnormalities were detected in (35%) of ADHD children, in comparison with (12%) of control group (p<0.005). The patterns of epileptiform discharge were mainly focal occipital (39.1%), frontal (34.8%), temporal (18.8), central (5.8%), and parietal (1.5%). Left sided cortical discharges were predominant in 65.2% of ADHD studied group.

conclusion: Subclinical EEG changes were significantly higher in children and adolescence with ADHD. Left sided cortical discharges were predominant in our study group. Key words: ADHD, Children, EEG

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