Neuronal Ceroid Lipofuscinoses are different types of Battens Disease.
The first symptoms are motor or/and cognitive decline or epilepsy. Patients with classic LINCL generally present with a standstill of mental development or the onset of severe epilepsy around the third year of life. The disorder progresses to complete loss of almost all motor and mental capacities before school age. As visual loss is not a prominent finding in the early stages of the disease course, it is frequently not recognised.
There is no curative treatment, only palliative treatment including administration of anticonvulsive drugs, treatment of spasticity with muscular relaxants and gastronomy feeding in the later stages of the disease.
The prognosis is poor with most children becoming severely disabled by mid-childhood. Most patients do not survive to reach adulthood.
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