What class/type of anticonvulsants or anti-seizure medications do you use on Batten disease patients?

To treat Batten disease patients the doctors prefer to use anti-seizure medications with a less toxic effect. First, they will choose a more effective medication to control the seizures; second, they will adjust the dosage of the current medication to reduce the damage to the liver functioning and renal functioning. After being admitted to our hospital, the doctors will assess the patient and choose the proper medications and dosage for the treatment. EVERYTHING IS INDIVIDUALIZED.

For the treatment of Batten disease, our doctors prefer to use neural stem cells, and in the treatment procedure, there are 4 separate stem cell injections (once per week) with 5 million stem cells in each injection. According to our clinical research and laboratory tests an individual can only handle a limited number of stem cells from external sources effectively. For children suffering from Batten disease, 5 million stem cells in each injection is the most effective number. If we implanted more stem cells into the patient's body there would be no improvement but it would add to the cost and more importantly, it would add a risk of developing encephalitis and other side effects.

April 2nd, I took over the case of a little five and a half-year-old girl from Russia, her symptoms started two years ago, and was diagnosed with NCL2 (Batten's disease) in 2008. The diagnosis was confirmed on Feb 15th, 2009 by the Enzyme Array (TTP test) in Seattle Children's Hospital (USA).

NCL (neuronal ceroid lipofuscinosis) is a kind of inherited neural degenerative disease affecting mainly children and only occasionally seen in adults. It is an autosomal recessive inheritance neural system disorder. In 1826, Dr. Stengel first discovered the disease and developed the concept of CL (ceroid lipofuscinosis).

Promising results from a team of NewYork-Presbyterian Hospital/Weill Cornell Medical Center physician-scientists show that gene therapy is both safe and effective at slowing the progression of Batten disease, or Late Infantile Neuronal Ceroid Lipofuscinosis (LINCL), a rare, genetic, degenerative neurological disorder that usually becomes fatal in children by the age of 8 to 12.

Batten's disease is a rare, fatal, autosomal recessive inherited disease affecting the nervous system (neurodegenerative disorder) that begins in childhood. It is named after the British pediatrician Frederick Batten who first described the juvenile form of neuronal ceroid lipofuscinosis (NCL) in 1903.