Search on: BATTEN DISEASE, JUVENILE 
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Descriptor English:   Neuronal Ceroid-Lipofuscinoses 
Descriptor Spanish:   Lipofuscinosis Ceroideas Neuronales 
Descriptor Portuguese:   Lipofuscinoses Ceroides Neuronais 
Synonyms English:   Adult Neuronal Ceroid Lipofuscinosis
Amaurotic Idiocy, Adult Type
Batten-Mayou Disease
Batten-Spielmeyer-Vogt Disease
CLN3-Related Neuronal Ceroid-Lipofuscinosis
CLN4A
CLN4B
Ceroid Lipofuscinosis, Neuronal 3, Juvenile
Ceroid Lipofuscinosis, Neuronal 4
Ceroid Lipofuscinosis, Neuronal, 3
Ceroid Lipofuscinosis, Neuronal, 4A, Autosomal Recessive
Ceroid Lipofuscinosis, Neuronal, 4B, Autosomal Dominant
Ceroid Lipofuscinosis, Neuronal, Parry Type
Ceroid Storage Disease
Infantile Neuronal Ceroid Lipofuscinosis
Juvenile Batten Disease
Juvenile Cerebroretinal Degeneration
Juvenile Neuronal Ceroid Lipofuscinosis
Kuf's Disease
Kufs Disease Autosomal Recessive
Kufs Disease, Autosomal Dominant
Kufs Disease, Autosomal Recessive
Kufs Type Neuronal Ceroid Lipofuscinosis
Late-Infantile Neuronal Ceroid Lipofuscinosis
Lipofuscin Storage Disease
Lipofuscinosis, Neuronal Ceroid
Neuronal Ceroid Lipofuscinosis
Neuronal Ceroid Lipofuscinosis Juvenile Type
Neuronal Ceroid Lipofuscinosis, Adult
Neuronal Ceroid Lipofuscinosis, Adult Type
Neuronal Ceroid Lipofuscinosis, Infantile
Neuronal Ceroid Lipofuscinosis, Juvenile
Neuronal Ceroid Lipofuscinosis, Late Infantile
Neuronal Ceroid Lipofuscinosis, Late-Infantile
Neuronal Ceroid-Lipofuscinosis
Spielmeyer-Sjogren Disease
Vogt Spielmeyer Disease
Vogt-Spielmeyer Disease
Batten Disease, Juvenile
Batten Diseases, Juvenile
Batten Mayou Disease
Batten Spielmeyer Vogt Disease
CLN3 Related Neuronal Ceroid Lipofuscinosis
CLN3-Related Neuronal Ceroid-Lipofuscinoses
CLN4As
Cerebroretinal Degeneration, Juvenile
Cerebroretinal Degenerations, Juvenile
Ceroid Lipofuscinosis, Neuronal
Ceroid Storage Diseases
Ceroid-Lipofuscinoses, CLN3-Related Neuronal
Ceroid-Lipofuscinosis, CLN3-Related Neuronal
Disease, Ceroid Storage
Disease, Juvenile Batten
Disease, Kuf's
Disease, Lipofuscin Storage
Disease, Spielmeyer-Sjogren
Disease, Vogt Spielmeyer
Disease, Vogt-Spielmeyer
Diseases, Ceroid Storage
Diseases, Juvenile Batten
Diseases, Lipofuscin Storage
Jansky Bielschowsky Disease
Juvenile Batten Diseases
Juvenile Cerebroretinal Degenerations
Kuf Disease
Lipofuscin Storage Diseases
Neuronal Ceroid Lipofuscinoses
Neuronal Ceroid-Lipofuscinoses, CLN3-Related
Neuronal Ceroid-Lipofuscinosis, CLN3-Related
Santavuori Haltia Disease
Spielmeyer Disease, Vogt
Spielmeyer Sjogren Disease
Spielmeyer Vogt Disease
Storage Disease, Ceroid
Storage Disease, Lipofuscin
Storage Diseases, Ceroid
Storage Diseases, Lipofuscin
Batten Disease
Ceroid-Lipofuscinosis, Neuronal
Jansky-Bielschowsky Disease
Kufs Disease
Santavuori-Haltia Disease
Spielmeyer-Vogt Disease  
Tree Number:   C10.574.500.550
C16.320.400.600
C16.320.565.398.641.509
C18.452.584.687.509
C18.452.648.398.641.509
Definition English:   A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure. 
History Note English:   2007 (1983) 
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications DI diagnosis
DG diagnostic imaging DH diet therapy
DT drug therapy EC economics
EM embryology EN enzymology
EP epidemiology EH ethnology
ET etiology GE genetics
HI history IM immunology
ME metabolism MI microbiology
MO mortality NU nursing
PS parasitology PA pathology
PP physiopathology PC prevention & control
PX psychology RT radiotherapy
RH rehabilitation SU surgery
TH therapy UR urine
VE veterinary VI virology
Record Number:   9664 
Unique Identifier:   D009472 

Occurrence in VHL:
 

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