Symptoms of most kinds of Batten Disease include loss of vision, epilepsy (see entry Epilepsy syndromes in Childhood) and progressive loss of abilities, including walking, speech and eating normally. A number of different genetic types are known. In the UK, CLN2 (late infantile) and CLN3 (juvenile) are the most common:
- CLN1 disease, infantile: onset usually between six months and two years with developmental delay and seizures. There are some cases where the onset is much later in childhood or adulthood.
- CLN2 disease, late infantile: onset between two and four years with seizures and slowing down of developmental progress. Atypical cases are recognised with later onset of learning difficulties and movement problems and slower disease progression (some of these have been diagnosed as spinocerebellar ataxia).
- Variant late infantile forms (CLN5, 6, 7, 8 diseases and others): onset between two and six years, often with challenging behaviour, slowed developmental progress and then seizures
- CLN3 disease, juvenile: onset of deteriorating vision between five and nine years. Children remain healthy for several years but then develop epilepsy and slowly become more dependent on others . Sadly, death can occur at any time from the late teens to the mid-thirties
- Early adult dementia