Juvenile Neuronal Ceroid Lipofuscinosis

Known as: Juvenile Cerebroretinal Degeneration, Batten-Mayou Disease, Cerebroretinal Degenerations, Juvenile 
A condition associated with mutation(s) in the CLN3 gene, encoding battenin. The condition is one of a group of genetically heterogeneous… (More)
National Institutes of Health

Topic mentions per year

Topic mentions per year

1962-2017
05010015019622017

Papers overview

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2011
2011
OBJECTIVE To explore the onset and progression of cardiac involvement in juvenile neuronal ceroid lipofuscinosis (JNCL… (More)
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2011
2011
OBJECTIVE To use the Unified Batten Disease Rating Scale (UBDRS) to measure the rate of decline in physical and functional… (More)
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2009
2009
Juvenile neuronal ceroid lipofuscinosis (JNCL), or Batten disease, is a neurodegenerative disease resulting from a mutation in… (More)
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2008
2008
Juvenile neuronal ceroid lipofuscinosis (JNCL) is an autosomal recessively inherited neurodegenerative disorder that results from… (More)
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2005
2005
BACKGROUND Juvenile neuronal ceroid lipofuscinosis (JNCL) is one of the most common neurodegenerative disorders in childhood and… (More)
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Highly Cited
2004
Highly Cited
2004
Mouse models of neuronal ceroid lipofuscinosis (NCL) exhibit many features of the human disorder, with widespread regional… (More)
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2000
2000
In juvenile neuronal ceroid-lipofuscinosis (JNCL), sleep disorders are common. The purpose of this study was to investigate the… (More)
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1998
1998
We present a clinicopathological study and the first molecular genetic analysis of a family with 2 siblings affected by a rare… (More)
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1996
1996
We studied 30 patients with juvenile neuronal ceroid lipofuscinosis (JNCL). The patients (aged 6-25 years) and 43 age-matched… (More)
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1995
1995
Batten disease, or the juvenile form of neuronal ceroid lipofuscinosis, is an autosomal recessive neurodegenerative disorder… (More)
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