You may have heard these names for the disease your child has; JNCL, Batten disease, juvenile neuronal ceroid lipofuscinoses, even juvenile Battens. This can be very frustrating to understand especially if your child has just been diagnosed. A bit of history may help to clear away the confusion.

An Englishman whose name was Batten first described this disease in 1914. In the US, Batten became the name used for a large group of lysosomal diseases. Loosely using the term Batten for all these diseases has made it confusing in diagnoses and in finding specific disease information.

In 1969 Drs. Zeman and Dyken used the term neuronal ceroid lipofuscinoses (NCL) for all the diseases called Batten to separate them from the gangliosides. The following were the NCL diseases, as they were known in 1969;

Today the NCL’s are a group of eight genetically different diseases. No similarities have been found between these diseases.