Symptoms vary greatly from child to child even within families. Every child is born with their own combination of genes which may modify the severity and even specific symptoms. Thus, not being able to predict the course of disease progression.

The earliest symptom of JNCL that gains attention is a deterioration or loss in vision often misdiagnosed as retinitis pigmentosa, leber's congenital amaurosis or some other rare hereditary eye disorder. Many of our children experience this phenomenon between 4 and 7 years of age.

The list of symptoms may include; slurred speech, seizures, dementia, difficulty sleeping, hyperactivity, loss of motor and mental capacity. These are all known as hard symptoms and are more obvious. 

Subtle symptoms prior to vision loss, may include reactions to acute smell, loud sounds and other irritation of the senses such as demanding their socks irritate them unless they are inside out.