What is juvenile Batten disease?
Juvenile Batten disease is an inherited disorder that primarily affects the nervous system. Beginning in childhood, affected individuals develop progressive vision loss, seizures, and intellectual decline. Vision loss is often the first noticeable sign of juvenile Batten disease, beginning between the ages of 4 and 8 years. Vision loss tends to worsen rapidly, leading to blindness. Recurrent seizures begin between the ages of 5 and 18 years. Children with juvenile Batten disease experience a decline in thinking ability (cognition) that worsens with time. They often have behavioral problems, difficulty sleeping, speech abnormalities, and problems with attention that appear in mid- to late childhood. Affected children also develop movement abnormalities similar to those associated with Parkinson disease. These include rigidity or stiffness, slow or diminished movements (hypokinesia), and stooped posture. Affected children may stumble and shuffle their feet when they walk. As the conditio
