What is Batten Disease?

Batten disease is a rare genetic disorder that affects how the brain and nervous system work. It often begins in childhood, but many of those affected by Batten are now living longer into their teens and adulthood thanks to recent medical advances and emerging treatments.

Over time, Batten disease causes a gradual loss of abilities — things like walking, talking, seeing, and doing everyday tasks. The nervous system is heavily affected, which is why seizures are so common across all forms of Batten.

One of the earliest and most noticeable signs is vision loss. Batten disease affects the inside of the eye, and many families first learn something is wrong during an eye exam. Some children are diagnosed because an eye doctor notices that the issue isn’t with the outer eye, but with the retina and underlying genetics.

Batten disease is inherited in an autosomal recessive pattern. This means a child must receive two non‑working copies of the affected gene — one from each parent — to develop the condition. Parents and siblings who carry one non‑working copy are healthy carriers and typically show no symptoms.

Batten disease is part of a group of conditions called neuronal ceroid lipofuscinoses (NCLs), which all involve a slow decline in abilities as the disease progresses.

Batten disease causes this decline because the affected cells cannot properly clear out waste materials. Over time, these substances build up inside neurons, especially in the brain and retina. As the waste accumulates, it damages and eventually destroys these nerve cells, leading to the progressive loss of abilities that families begin to notice over months and years.

Symptoms of ALL Batten Types

Across all forms of Batten disease, families may see:

• Vision loss or blindness

• Seizures

• Developmental regression

• Communication difficulities

• Loss of speech

• Difficulty walking or coordinating movement

• Movement disorders

• Behavioral changes

• Cognitive decline

• Sleep disturbances

• Feeding challenges

• Mobility loss

• Increased care needs over time

These symptoms appear at different ages and progress at different speeds depending on the specific type of Batten disease. As Batten progresses, children often need increasing support with daily activities such as mobility, communication, feeding, and personal care. Every child’s journey is different, but families commonly see a gradual shift from independence to needing more hands‑on help as the disease advances.

Types of Batten Disease

Batten disease is classified into 14 types, based on the gene involved (CLN1–CLN14) and the age when symptoms first appear. These types fall into several major groups:

Infantile Forms

CLN1 – Congenital Form
Symptoms begin at birth or within the first months of life.
Rapid progression with early seizures, developmental delays, and neurological decline.

CLN1 – Infantile Form (INCL)
Symptoms begin between 6 months and 2 years.
Early vision loss, seizures, and rapid developmental regression.

Late Infantile Forms

CLN2, CLN5, CLN6, CLN7, CLN8, CLN10
Symptoms typically begin between ages 2–4 or later childhood.
Seizures, movement difficulties, developmental regression, vision loss, and progressive neurological decline.
CLN2 is the only type with an FDA‑approved treatment.

Juvenile Forms

CLN3, CLN11, CLN12
Symptoms typically begin between ages 5–10 or later childhood.
Often starts with vision loss, followed by seizures, behavioral changes, cognitive decline, and gradual loss of motor abilities.
CLN3 is the most common juvenile form.

Adult Forms

CLN4, CLN13
Symptoms begin in late teens to adulthood.
Slower progression with movement problems, dementia‑like symptoms, and behavioral changes.
Early blindness is less common.

⭐ Did You Know?

CLN1 counts as two types because Congenital and Infantile CLN1 begin at different ages and progress differently, even though they share the same gene.

There is no CLN9 type. Researchers once proposed a ninth Batten gene, but later studies showed the mutation did not consistently cause NCL. The gene was reclassified, and CLN9 was removed from the Batten disease list — which is why the numbering jumps from CLN8 to CLN10.

The most commonly diagnosed types are CLN1, CLN2, and CLN3.

What is CLN7?

CLN7 is the Batten disease type that affects Jordan and Zachary, the brothers behind Brothers With Batten. It is a rare form of Batten disease caused by changes in the MFSD8 gene and is classified as a late‑infantile type, though symptoms may appear in later childhood depending on the variant. CLN7 affects the brain and nervous system, leading to seizures, developmental regression, vision loss, and increasing care needs over time.

Life Expectancy for CLN7

Most individuals diagnosed with CLN7 Batten disease pass in their early to mid‑teenage years, though progression can vary widely from person to person. CLN7 is known for:

• Rapid neurological decline

• Increasing seizure activity

• Loss of mobility

• Loss of communication

• Complex medical needs

• Intensive long-term care and support

In general medical literature, late‑infantile forms like CLN7 are described as more severe and having a shorter average life expectancy than juvenile or adult‑onset Batten types—but individual outcomes can differ, especially in rare or mutated forms.

Why Jordan & Zachary Are Unique

Jordan and Zachary have a rare, mutated form of CLN7 Batten disease, which caused their symptoms and progression to look very different from what is typically expected. Their symptoms began in 2000, but because genetic testing was not widely available at the time, it took nearly ten years before they were officially diagnosed in October 2010—making them among the earliest confirmed CLN7 cases. Their mutation slowed the disease’s decline, allowing them to live far beyond the usual early‑to‑mid‑teenage expectancy and presenting a clinical course that does not match standard patterns.

Their experience reflects several unique characteristics:

• A mutated CLN7 variant that behaves differently than typical cases

• A slower progression than most individuals with this type

• A lifespan far beyond the usual expectancy

Jordan and Zachary’s path represents an exceptionally rare combination of genetics, timing, and resilience—one that continues to shape the mission and purpose behind Brothers With Batten’s.

While Batten disease is challenging, there is real hope in the progress being made. Research continues to grow, new treatments are emerging for certain Batten types, and supportive therapies are helping children live longer and with better quality of life. Families around the world are connected through a strong and compassionate community that shares knowledge, encouragement, and strength. No one faces Batten disease alone, and every effort toward awareness and research brings us closer to improving the lives of all children affected.

Want to know more about Jordan and Zachary and the heart behind our mission?