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Descriptor English: Alexander Disease
Descriptor Spanish: Enfermedad de Alexander
Descriptor enfermedad de Alexander
Scope note: Trastorno hereditario infrecuente de la formación de la mielina. La enfermedad de Alexander es una leucoencefalopatía progresiva cuyo sello distintivo es la acumulación generalizada de inclusiones citoplasmáticas denominadas fibras de Rosenthal. Las fibras contienen PROTEÍNA ÁCIDA FIBRILAR DE LA GLIA asociada con la CADENA B DE LA CRISTALINA ALFA. Las fibras de Rosenthal se encuentran predominantemente en los ASTROCITOS localizados en las áreas subependimarias, subpiales y periventriculares del CEREBRO.
Descriptor Portuguese: Doença de Alexander
Descriptor French: Maladie d'Alexander
Entry term(s): Alexander's Disease
Alexanders Disease
Demyelinogenic Leukodystrophy
Dysmyelinogenic Leukodystrophy
Fibrinoid Degeneration of Astrocytes
Leukodystrophy with Rosenthal Fibers
Tree number(s): C10.228.140.163.100.362.312
C10.228.140.695.625.312
C10.314.400.312
C10.574.500.024
C16.320.400.024
C16.320.565.189.362.312
C18.452.132.100.362.312
C18.452.648.189.362.312
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D038261
Scope note: Rare leukoencephalopathy with infantile-onset accumulation of Rosenthal fibers in the subpial, periventricular, and subependymal zones of the brain. Rosenthal fibers are GLIAL FIBRILLARY ACIDIC PROTEIN aggregates found in ASTROCYTES. Juvenile- and adult-onset types show progressive atrophy of the lower brainstem instead. De novo mutations in the GFAP gene are associated with the disease with propensity for paternal inheritance.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Public MeSH Note: 2003
History Note: 2003
Related: Glial Fibrillary Acidic Protein MeSH
DeCS ID: 37281
Unique ID: D038261
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2003/01/01
Date of Entry: 2002/07/02
Revision Date: 2013/07/08
Alexander Disease - Preferred
Concept UI M0415670
Scope note Rare leukoencephalopathy with infantile-onset accumulation of Rosenthal fibers in the subpial, periventricular, and subependymal zones of the brain. Rosenthal fibers are GLIAL FIBRILLARY ACIDIC PROTEIN aggregates found in ASTROCYTES. Juvenile- and adult-onset types show progressive atrophy of the lower brainstem instead. De novo mutations in the GFAP gene are associated with the disease with propensity for paternal inheritance.
Preferred term Alexander Disease
Entry term(s) Alexander's Disease
Alexanders Disease
Demyelinogenic Leukodystrophy
Dysmyelinogenic Leukodystrophy
Fibrinoid Degeneration of Astrocytes
Leukodystrophy with Rosenthal Fibers



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