Code : 9345-350049      Created Date : Tuesday, July 14, 2015   Update Date : Tuesday, July 14, 2015    Visit : 2386

The 13th Asian and Oceanian Congress of Child Neurology

The report of The 13th Asian and Oceanian Congress of Child Neurology by Dr. Mahmoudreza Ashrafi
 
Application Code :
306-0115-0074
 
Created Date : Wednesday, April 8, 2015 10:54:32Update Date : Tuesday, June 30, 2015 07:59:13
IP Address : 194.225.212.150Submit Date : Tuesday, June 30, 2015 08:00:12Email : mr_ashrafi@yahoo.com
Personal Information
Name : Mahmoudreza
Surname : ashrafi
School/Research center : School of Medicine
If you choose other, please name your Research center :  
Position : Professor
Tel : +98-21-22173370
Information of Congress
Title of the Congress : The 13th Asian and Oceanian Congress of Child Neurology
Title of your Abstract : Childhood Leukodystrophies
country : Taiwan
From : Thursday, May 14, 2015
To : Sunday, May 17, 2015
Abstract(Please copy/paste the abstract send to the congress) : Childhood leukodystrophies
Mahmoud reza Ashrafi 1 , Alireza Tavasoli 2
1- Professor of pediatric neurology. Children’s Medical Center , Tehran University of Medical Science
2-. Assistant professor of pediatric neurology. Children’s Medical Center , Tehran University of Medical Science

Heritable white matter disorders can be classified in two categories for better assessment: A- classic leukodystrophies, that affect principally the myelin of the brain (sometimes also of the peripheral nerves ) and in many cases some involvement of gray nuclei coexists . B- genetic leukoencephalopathy, in which during clinical course of a systemic neurometabolic disorder, white matter of the nervous system is also affected.
The main inheritance pattern of these disorders is autosomal recessive. The reported incidence of leukodystrophies has a wide range from 1/5000 to 2/100000 among live births. The etiology is not determined in about 50% of patients that are categorized as unclassified leukodystrophies and majority of this last group are belonging to hypomyelinating leukodystrophies.
The main clinical manifestations of leukodystrophies are regression in motor developmental milestones and motor disturbances , especially pyramidal and cerebellar symptoms and signs , with slow mental deterioration .
The leukodystrophies may be classified according to enzymatic defect , pathology , etiology , affected organells and age of onset. Classification of WMDs according to Brain MRI findings seems to be applied and clinically oriented .Major factors in brain MRI that help for better diagnosis are: presence of hypomyelination or dysmyelination in T1 and T2 sequences , main location of involvement ( subcortical or periventricular ) ; confluent and symmetric lesions versus multifocal and asymmetric ones; tigroid pattern; ; cystic changes and calcification and contrast enhancement. Important clues for diagnostic approach are : age of onset of symptoms; some hints in history and physical examination such as head circumference ( macrocephaly or microcephaly) , other organ involvement (skeletal, dental , gastrointestinal and ocular) . The diagnostic strategy rests upon clinical clues and MRI patterns, complemented by appropriately selected electrophysiological and laboratory testing. 
Most leukodystrophies are incurable and have a progressive course, leading to premature death. Diagnosis is important as palliative or experimental therapies may offer benefits, for genetic counseling and family screening of currently unaffected individuals. Some beneficial proceedings for improvement of quality of life of patients are: attention to the patient's swallowing and feeding condition; control of pain and spasm and correction of endocrine abnormalities. More fundamental steps that are considered in recent years to treat these patients are: Enzyme Replacement Therapy (ERT) ,Substrate Reduction Therapy ( SRT) , cell-based therapy such as bone marrow transplantation and gene therapy.
In our Neurometabolic registry website that constructed in 2010 about 185 patients have been registered that are belonging to different groups of Neurometabolic disorders. We have registered 80 patients that are categorized into classic and unclassified leukodystrophies. We found that the most common type of leukodystrophy among our patients is Metachromatic leukodystrophy. Other common types were Canavan disease, unclassified leukodystrophies and X-linked Adrenoleukodystrophy.
 
Keywords of your Abstract : Leukodystrophy , Leukoencephalopathy , Hypomyelination , Dysmyelination
Acceptance Letter : http://gsia.tums.ac.ir/images/UserFiles/24423/Forms/306/AOCCN Invitation-.Dr. Mahmoudreza Ashrafi-64_14.pdf
The presentation : Oral
The Cover of Abstract book : http://gsia.tums.ac.ir/images/UserFiles/24423/Forms/306/Picture 003_2.pdf
Published abstract in the abstract book with the related code : http://gsia.tums.ac.ir/images/UserFiles/24423/Forms/306/AOCCN_Abstract_Mahmoudreza Ashrafi_new (2)_2.pdf
Where has your abstract been indexed? : none
If you choose other, please name :  
The Congress Reporting Form
How many volunteers were present at the Congress? : more than 200
Delegates from which countries presented in the congress? : 32 Asian and Oceanian countries ( japan , India ,Australia , Malaysia, Singapore , Turkey , Taiwan , Hong Kong , New Zealand , Pakistan , Bangeladesh , Jordan , China , Thailand , Philippines , Saudia Arabia ,..) and some speakers from Canada , USA , and UK
Were the delegates of any other organizations present in the congress? : No
If yes, please write the names of the organizations in the box :  
What were the responses to your talking points? Were specific questions or concerns raised? : There was 2 questions from India and Singapore about cranial nerve and basal ganglia involvement In leukodystrophies . A short discussion about Alexander disease and its presentations occurred .
If you met staff members, please list their full names & positions. : Ingrid Tein from Canada Chief of the ICNA . ching-shiang chi head of child neurologist of Taiwan
Please inform us if there are any follow up actions we need to talk with the members of the congress : I think that Iranian Child Neurology association have a good contact with International Child Neurology association (ICNA) and Asian and Oceanian Child Neurology association ( AOCCN) that facilitate our cooperations . The next AOCCN congress will be held in Japan that the TUMS can talk with organizing committee of this congress about presence of more Iranian delegates .
Your experiences about the travel processes(Providing ticket, accommodation,...) : very cooperative secretory for landing visa application of Taipei i
Please give a briefing of your own observations and outcomes of the congress: : presence of 2 invited speakers from Iran in the field of neurometabolic disorders and child neurology was a good opportunity for Iranian delegates of child neurology . We presented our experiences about the registry of neurometabolic disorders in Iran and the treatment of some treatable IEM especially Niemann-Pick disease type c ( NPC ) . I think that without registry of the patients we can not be able to compete with other countries . Some plenary speakers have a very good and informative presentations.

 

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