(GIST OF SCIENCE REPORTER) Batten Disease

(NOVEMBER-2025)

Batten Disease

Researchers recently have found that male and female brains show different responses as the Batten Disease progresses and have found a model of the disease that could transform future treatments

About Batten Disease:

It is a very rare genetic disorder that affects the brain and nervous system.
Batten disease is a congenital, progressive, and terminal neurological disease.
Symptoms sometimes begin in infancy and sometimes later in childhood, after apparently normal early development.
Occasionally it may start in adulthood.
In individuals suffering from Batten disease, nervous system function steadily declines, causing a wide variety of neurologic problems, including loss of vision and seizures.
The life expectancy of a child born with Batten disease can vary, depending on the form of the disease and the age of onset.
Some children die in early childhood, while others may be able to live into their teens or twenties.

What Causes Batten Disease?

Batten disease is actually a family of related disorders also known as neuronal ceroid lipofuscinoses.
It can be caused by genetic mutations in any of more than a dozen known genes, collectively referred to as CLN genes (CLN1, CLN2, etc).
There are 13 known forms of Batten disease (CLN1 through CLN14).
Each form is classified by the specific version of the CLN (ceroid lipofuscinosis, neuronal) gene that causes it, with each numbered subtype referring to a different gene that causes its own respective form of the disease.
The most common type of Batten disease is CLN3.

Symptoms:

Most forms of Batten disease cause vision loss, seizures, delayed developmental milestones, behavioral and learning problems, and loss of language and motor skills.
Some children with infantile Batten disease also develop microcephaly.
Vision loss is often the first symptom and can rapidly progress.
Parents also often notice clumsiness and stumbling in older children due to a loss of motor coordination.
Eventually, children with Batten disease become blind, unable to walk, talk, or swallow, and confined to a wheelchair or bed.

Treatment:

Currently, there is no treatment to reverse the effects of Batten disease.
But some medications can help improve symptoms like seizure
Some people with Batten disease get physical or occupational therapy to help them function.