First International Congress of the ELA Foundation – october 2006

The 1st International Congress of the ELA Research Foundation & The Myelin Project entitled “The vital function of myelin development and maintenance” took place in Paris from October 5 to 7, 2006. The Scientific Committee in charge of the meeting organization was composed of Prs. Aubourg, Dubois-Dalcq, Boespflug-Tanguy, Lacaze and Baron-Van Evercooren.


Over 200 participants (M.D., Ph.D, Postdocs and Ph.D. students) from around the world specialized in leukodystrophies and myelin repair attended the meeting and presented their latest results.

The congress was divided in four different scientific sessions:
– Myelin development and repair
– Pathological mechanism of myelin disorders
– White matter disease of the premature
– Genetics & Therapeutics of Leukodystrophies

Among the numerous scientific discoveries presented during the meeting, we can point out that various molecules implicated in the production of myelin have been identified and represent potential candidates for future therapeutic applications. Also, new leukodystrophies have been described:

– Leukodystrophy with progressive ataxia*, deafness and cardiomyopathy**
– Leucoencephalopathy with delayed dentition and hypomyelination
– Leucoencephalopathy HCC characterized by hypomyelination and congenital cataract. Identified gene: hyccin
– Pelizaeus-Merzbacher-like disease with ataxia* and spasticity***. Identified gene: GJA12

New imaging technologies that can be used as diagnostic and to monitor disease outcome have been developed. It is now possible to perform MRI in prematures. Moreover, the measure of water diffusion by MRI allows evaluating the brain microstructure in children. Today, blood and urine sulfatides can be measured by mass spectrometry and long chain fatty acids can be quantified using liquid chromatography combined with mass spectrometry in newborns.

*Ataxia: Unsteady movements that result from the brain’s failure to regulate the body’s posture and the strength and direction of movements.
**Cardiomyopathy: Disease weakening the heart muscle which usually causes inadequate heart pumping.
***Spasticity: Stiff or rigid muscles and exaggerated deep tendon reflexes that interferes with muscular activity, gait, movement, or speech.


Downloads :

1st ELA Research Foundation detailed Report (PDF – 26 Ko)