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Neuronal ceroid lipofuscinoses (NCLS) - Overview

Alternative Names

Lipofuscinoses; Batten disease; Jansky-Bielschowsky; Kufs' disease; Spielmeyer-Vogt

Definition of Neuronal ceroid lipofuscinoses (NCLS):

Neuronal ceroid lipofuscinoses (NCLS) refers to a group of rare, inherited disorders of the nerve cells.

There are three main types of NCLS:

  • Adult (Kufs' or Parry's disease)
  • Juvenile (Batten disease)
  • Late infantile (Jansky-Bielschowsky)

Causes, incidence, and risk factors:

NCLS is a type of neurodegenerative disorder. It involves the buildup of an abnormal material called lipofuscin in the brain. Evidence suggests that NCLS is caused by problems with the brain's ability to remove and recycle proteins.

Lipofuscinoses are inherited as autosomal recessive traits. That means if both parents carry the trait, each child has:

  • A 1 in 4 chance of having the disease
  • A 2 in 4 chance of not having the disease but carrying the trait
  • A 1 in 4 chance of not having the disease and not being a carrier
  • Reviewed last on: 10/14/2009
  • Luc Jasmin, MD, PhD, Departments of Anatomy and Neurological Surgery, University of California, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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