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Alexander Disease

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Hierarchy:	Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Genetic Diseases, Inborn ∟ Heredodegenerative Disorders, Nervous System ∟ Alexander Disease 30 coordinate concepts ∟ Adrenoleukodystrophy \| ∟ Canavan Disease \| ∟ Leukodystrophy, Globoid Cell \| ∟ Leukodystrophy, Metachromatic \| ∟ Pelizaeus-Merzbacher Disease \| ∟ Amyloid Neuropathies, Familial \| ∟ Cockayne Syndrome \| ∟ Dystonia Musculorum Deformans \| ∟ Gerstmann-Straussler-Scheinker Disease \| ∟ Hallervorden-Spatz Syndrome \| ∟ Hepatolenticular Degeneration \| ∟ Hereditary Central Nervous System Demyelinating Diseases \| ∟ Hereditary Motor and Sensory Neuropathies \| ∟ Hereditary Sensory and Autonomic Neuropathies \| ∟ Huntington Disease \| ∟ Lafora Disease \| ∟ Lesch-Nyhan Syndrome \| ∟ Menkes Kinky Hair Syndrome \| ∟ Mental Retardation, X-Linked \| ∟ Myotonia Congenita \| ∟ Myotonic Dystrophy \| ∟ Neurofibromatoses \| ∟ Neuronal Ceroid-Lipofuscinoses \| ∟ Optic Atrophies, Hereditary \| ∟ Rett Syndrome \| ∟ Spinal Muscular Atrophies of Childhood \| ∟ Spinocerebellar Degenerations \| ∟ Tourette Syndrome \| ∟ Tuberous Sclerosis \| ∟ Unverricht-Lundborg Syndrome Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Genetic Diseases, Inborn ∟ Heredodegenerative Disorders, Nervous System ∟ Hereditary Central Nervous System Demyelinating Diseases ∟ Alexander Disease 30 coordinate concepts ∟ Adrenoleukodystrophy \| ∟ Canavan Disease \| ∟ Leukodystrophy, Globoid Cell \| ∟ Leukodystrophy, Metachromatic \| ∟ Pelizaeus-Merzbacher Disease \| ∟ Amyloid Neuropathies, Familial \| ∟ Cockayne Syndrome \| ∟ Dystonia Musculorum Deformans \| ∟ Gerstmann-Straussler-Scheinker Disease \| ∟ Hallervorden-Spatz Syndrome \| ∟ Hepatolenticular Degeneration \| ∟ Hereditary Central Nervous System Demyelinating Diseases \| ∟ Hereditary Motor and Sensory Neuropathies \| ∟ Hereditary Sensory and Autonomic Neuropathies \| ∟ Huntington Disease \| ∟ Lafora Disease \| ∟ Lesch-Nyhan Syndrome \| ∟ Menkes Kinky Hair Syndrome \| ∟ Mental Retardation, X-Linked \| ∟ Myotonia Congenita \| ∟ Myotonic Dystrophy \| ∟ Neurofibromatoses \| ∟ Neuronal Ceroid-Lipofuscinoses \| ∟ Optic Atrophies, Hereditary \| ∟ Rett Syndrome \| ∟ Spinal Muscular Atrophies of Childhood \| ∟ Spinocerebellar Degenerations \| ∟ Tourette Syndrome \| ∟ Tuberous Sclerosis \| ∟ Unverricht-Lundborg Syndrome Medical Subject Headings (MeSH) ∟ Nervous System Diseases ∟ Demyelinating Diseases ∟ Hereditary Central Nervous System Demyelinating Diseases ∟ Alexander Disease 30 coordinate concepts ∟ Adrenoleukodystrophy \| ∟ Canavan Disease \| ∟ Leukodystrophy, Globoid Cell \| ∟ Leukodystrophy, Metachromatic \| ∟ Pelizaeus-Merzbacher Disease \| ∟ Amyloid Neuropathies, Familial \| ∟ Cockayne Syndrome \| ∟ Dystonia Musculorum Deformans \| ∟ Gerstmann-Straussler-Scheinker Disease \| ∟ Hallervorden-Spatz Syndrome \| ∟ Hepatolenticular Degeneration \| ∟ Hereditary Central Nervous System Demyelinating Diseases \| ∟ Hereditary Motor and Sensory Neuropathies \| ∟ Hereditary Sensory and Autonomic Neuropathies \| ∟ Huntington Disease \| ∟ Lafora Disease \| ∟ Lesch-Nyhan Syndrome \| ∟ Menkes Kinky Hair Syndrome \| ∟ Mental Retardation, X-Linked \| ∟ Myotonia Congenita \| ∟ Myotonic Dystrophy \| ∟ Neurofibromatoses \| ∟ Neuronal Ceroid-Lipofuscinoses \| ∟ Optic Atrophies, Hereditary \| ∟ Rett Syndrome \| ∟ Spinal Muscular Atrophies of Childhood \| ∟ Spinocerebellar Degenerations \| ∟ Tourette Syndrome \| ∟ Tuberous Sclerosis \| ∟ Unverricht-Lundborg Syndrome Medical Subject Headings (MeSH) ∟ Nervous System Diseases ∟ Neurodegenerative Diseases ∟ Heredodegenerative Disorders, Nervous System ∟ Alexander Disease 30 coordinate concepts ∟ Adrenoleukodystrophy \| ∟ Canavan Disease \| ∟ Leukodystrophy, Globoid Cell \| ∟ Leukodystrophy, Metachromatic \| ∟ Pelizaeus-Merzbacher Disease \| ∟ Amyloid Neuropathies, Familial \| ∟ Cockayne Syndrome \| ∟ Dystonia Musculorum Deformans \| ∟ Gerstmann-Straussler-Scheinker Disease \| ∟ Hallervorden-Spatz Syndrome \| ∟ Hepatolenticular Degeneration \| ∟ Hereditary Central Nervous System Demyelinating Diseases \| ∟ Hereditary Motor and Sensory Neuropathies \| ∟ Hereditary Sensory and Autonomic Neuropathies \| ∟ Huntington Disease \| ∟ Lafora Disease \| ∟ Lesch-Nyhan Syndrome \| ∟ Menkes Kinky Hair Syndrome \| ∟ Mental Retardation, X-Linked \| ∟ Myotonia Congenita \| ∟ Myotonic Dystrophy \| ∟ Neurofibromatoses \| ∟ Neuronal Ceroid-Lipofuscinoses \| ∟ Optic Atrophies, Hereditary \| ∟ Rett Syndrome \| ∟ Spinal Muscular Atrophies of Childhood \| ∟ Spinocerebellar Degenerations \| ∟ Tourette Syndrome \| ∟ Tuberous Sclerosis \| ∟ Unverricht-Lundborg Syndrome Medical Subject Headings (MeSH) ∟ Nervous System Diseases ∟ Neurodegenerative Diseases ∟ Heredodegenerative Disorders, Nervous System ∟ Hereditary Central Nervous System Demyelinating Diseases ∟ Alexander Disease 30 coordinate concepts ∟ Adrenoleukodystrophy \| ∟ Canavan Disease \| ∟ Leukodystrophy, Globoid Cell \| ∟ Leukodystrophy, Metachromatic \| ∟ Pelizaeus-Merzbacher Disease \| ∟ Amyloid Neuropathies, Familial \| ∟ Cockayne Syndrome \| ∟ Dystonia Musculorum Deformans \| ∟ Gerstmann-Straussler-Scheinker Disease \| ∟ Hallervorden-Spatz Syndrome \| ∟ Hepatolenticular Degeneration \| ∟ Hereditary Central Nervous System Demyelinating Diseases \| ∟ Hereditary Motor and Sensory Neuropathies \| ∟ Hereditary Sensory and Autonomic Neuropathies \| ∟ Huntington Disease \| ∟ Lafora Disease \| ∟ Lesch-Nyhan Syndrome \| ∟ Menkes Kinky Hair Syndrome \| ∟ Mental Retardation, X-Linked \| ∟ Myotonia Congenita \| ∟ Myotonic Dystrophy \| ∟ Neurofibromatoses \| ∟ Neuronal Ceroid-Lipofuscinoses \| ∟ Optic Atrophies, Hereditary \| ∟ Rett Syndrome \| ∟ Spinal Muscular Atrophies of Childhood \| ∟ Spinocerebellar Degenerations \| ∟ Tourette Syndrome \| ∟ Tuberous Sclerosis \| ∟ Unverricht-Lundborg Syndrome X
Broader terms:	Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Genetic Diseases, Inborn ∟ Heredodegenerative Disorders, Nervous System ∟ Alexander Disease Medical Subject Headings (MeSH) ∟ Congenital, Hereditary, and Neonatal Diseases and Abnormalities ∟ Genetic Diseases, Inborn ∟ Heredodegenerative Disorders, Nervous System ∟ Hereditary Central Nervous System Demyelinating Diseases ∟ Alexander Disease Medical Subject Headings (MeSH) ∟ Nervous System Diseases ∟ Demyelinating Diseases ∟ Hereditary Central Nervous System Demyelinating Diseases ∟ Alexander Disease Medical Subject Headings (MeSH) ∟ Nervous System Diseases ∟ Neurodegenerative Diseases ∟ Heredodegenerative Disorders, Nervous System ∟ Alexander Disease Medical Subject Headings (MeSH) ∟ Nervous System Diseases ∟ Neurodegenerative Diseases ∟ Heredodegenerative Disorders, Nervous System ∟ Hereditary Central Nervous System Demyelinating Diseases ∟ Alexander Disease Heredodegenerative Disorders, Nervous System > Genetic Diseases, Inborn > Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Medical Subject Headings (MeSH) Hereditary Central Nervous System Demyelinating Diseases > Heredodegenerative Disorders, Nervous System > Genetic Diseases, Inborn > Congenital, Hereditary, and Neonatal Diseases and Abnormalities > Medical Subject Headings (MeSH) Hereditary Central Nervous System Demyelinating Diseases > Demyelinating Diseases > Nervous System Diseases > Medical Subject Headings (MeSH) Heredodegenerative Disorders, Nervous System > Neurodegenerative Diseases > Nervous System Diseases > Medical Subject Headings (MeSH) Hereditary Central Nervous System Demyelinating Diseases > Heredodegenerative Disorders, Nervous System > Neurodegenerative Diseases > Nervous System Diseases > Medical Subject Headings (MeSH) X
historyNote*:	2003 X
publicMeSHNote*:	2003 X
Scope note:	A rare inherited disorder of myelin formation. Alexander disease is a progressive leukoencephalopathy whose hallmark is the widespread accumulation of cytoplasmic inclusions called Rosenthal fibers. The fibers contain GLIAL FIBRILLARY ACIDIC PROTEIN in association with ALPHA-CRYSTALLIN B CHAIN. Rosenthal fibers are found predominantly in ASTROCYTES located in the subependymal, subpial, and periventricular areas of the BRAIN. X
activeMeSHYear*:	2005 \| 2006 \| 2007X
dateCreated*:	2002-07-02X
dateEstablished*:	2003-01-01X
dateRevised*:	2004-07-19X
recordAuthorizer*:	sjnX
recordMaintainer*:	jlsX
recordOriginator*:	jmpX
Type:	Concept X
Coordinate terms:	Adrenoleukodystrophy \| Canavan Disease \| Leukodystrophy, Globoid Cell \| Leukodystrophy, Metachromatic \| Pelizaeus-Merzbacher Disease \| Amyloid Neuropathies, Familial \| Cockayne Syndrome \| Dystonia Musculorum Deformans \| Gerstmann-Straussler-Scheinker Disease \| Hallervorden-Spatz Syndrome \| Hepatolenticular Degeneration \| Hereditary Central Nervous System Demyelinating Diseases \| Hereditary Motor and Sensory Neuropathies \| Hereditary Sensory and Autonomic Neuropathies \| Huntington Disease \| Lafora Disease \| Lesch-Nyhan Syndrome \| Menkes Kinky Hair Syndrome \| Mental Retardation, X-Linked \| Myotonia Congenita \| Myotonic Dystrophy \| Neurofibromatoses \| Neuronal Ceroid-Lipofuscinoses \| Optic Atrophies, Hereditary \| Rett Syndrome \| Spinal Muscular Atrophies of Childhood \| Spinocerebellar Degenerations \| Tourette Syndrome \| Tuberous Sclerosis \| Unverricht-Lundborg Syndrome X
URI:	X
Labels and equivalent concepts:	Alexanderin tauti (fi) Alexander's Disease (en, replaced) Enfermedad de Alexander (es) Alexander-Krankheit (de) Alexanderova nemoc (cs) Leukodystrophie, fibrinoide (de, replaced) Panneuropathie, hyaline (de, replaced) Leukodystrophie, dysmyelinogene (de, replaced) Hyaline Panneuropathie (de, replaced) Dysmyelinogene Leukodystrophie (de, replaced) Fibrinoide Leukodystrophie (de, replaced) Alexander-Syndrom (de, replaced) X
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