The DZNE and the NCL Foundation cooperate
Bonn/ Hamburg. 22nd, September 2016. Learning from each other, joint research and as such make new therapies against childhood and senile dementia more tangible – this is what the German Center for Neurodegenerative Diseases (DZNE) and the NCL Foundation, now want to push ahead. Joint scientific workshops with researchers from the different areas, are initially intended to serve this purpose.
Both diseases are accompanied with the loss of cognitive and motor skills, the cause of which is the death of nerve cells. This is why they are designated as neurodegenerative diseases. However, forms of dementia such as Alzheimer's and Parkinson's are better known among the general public, because with approximately 1.5 million people affected in Germany, they constitute the most frequent occurring diseases.
However, rare inherited neurodegenerative diseases, which are often accompanied by memory phenomena in nerve cells, also occur among children and adolescents. Experts predict that more than 1,000 children and adolescents become newly diseased in Germany, every year.
Meanwhile more than 50 different childhood dementias are known. The most common form of the childhood dementia is the so-called Batten disease (neuronal ceroid lipofuscinosis - NCL), whereby the first symptoms can already occur, between the late infant and school age. The progression of this genetically triggered metabolic disorder is dramatic. Due to a disorder of the cell metabolism, the affected nerve cells die. The health consequences manifest themselves in the occurrence of seizures, progressive movement disorder, increasing vision loss, up to blindness. The patients eventually lose all their cognitive and motor skills and sustain premature death.
„The diagnosis NCL is devastating for the children and parents and there are currently no therapies. We do not only just want to push ahead with the cooperation of research, but also together want to draw the attention of the general public, to childhood and senile dementia“, quote from Dr. Frank Stehr, Executive of the Board of the NCL Foundation. „The DZNE has evolved in recent years into one of the most important research centres in this field in Germany. “
Currently about 14 different genetic defects are known, which can lead to NCL diseases. These genetic defects trigger a build up of ceroid lipofuscin in the nerve cells, because the degradation is disturbed.
„We look forward to the scientific exchange with colleagues from the NCL Foundation. NCL is one of many childhood dementia diseases and it is important to systematically analyse common underlying mechanisms. In this, the DZNE plays a central role”, quote from Prof. Pierluigi Nicotera, DZNE’s Scientific Director and Chairman of the Executive Board. “In analysing this mechanism, the DZNE collaborates with Prof. Gärtner from Göttingen. These scientific work is very important for affected children, their families and society”.
About NCL Foundation
The NCL Foundation was founded 2002 by Dr. Frank Husemann, after his then 6-year-old son Tim was diagnosed with JNCL. Neuronal Ceroid Lipofuscinosis is a rare metabolic disorder which leads to a progressive loss of nerve cells. It is the most common form of childhood dementia. Affected children suffer from neurodegeneration affecting different types of neurons, also in the retina, and this results in early blindness, mental deterioration, loss of motor function and the development of epileptic seizures. Many NCL patients do not survive past their third decade.
More information: www.ncl-foundation.com