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What is Krabbe disease?

Krabbe disease (also called globoid cell leukodystrophy) is a rare, inherited neurological condition. It’s pronounced “KRAH-buh.”

Krabbe disease is part of a group of disorders known as leukodystrophies, which result from the loss of myelin (demyelination) in your nervous system. A myelin sheath is a protective sleeve that’s wrapped around each nerve cell that ensures the rapid transmission of nerve signals.

The disease is also characterized by abnormal cells in your brain called globoid cells, which are large cells that usually have more than one nucleus.

The demyelination results in the destruction of brain cells, which causes progressive neurological issues, such as:

Krabbe disease is a degenerative condition, meaning it progressively gets worse. It usually results in death.

The condition gets its name from the Danish neurologist Knud Krabbe.