Clinical

Syndromes: Batten disease

A look at Batten disease, a collection of rare and fatal inherited disorders of the nervous system
A woman embraces her five year old son, who has been diagnosed with Batten disease

A look at Batten disease, a collection of rare and fatal inherited disorders of the nervous system

The term Batten disease includes a collection of rare and fatal inherited disorders of the nervous system (National Institute of Neurological Disorders and Stroke 2019) . These disorders are termed neuronal ceroid lipofuscinoses (NCLs) and include nine forms.

Nine genes and age of onset

The nine forms of neuronal ceroid lipofuscinoses, classified by the gene that causes the disorder, are listed here with the age of onset:

  • CLN1 infantile but also late infantile, juvenile and adult
  • CLN2 late infantile but also juvenile
  • CLN3 juvenile
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