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Pelizaeus-Merzbacher Disease
Hierarchy: | ∟ ∟ ∟ ∟ Pelizaeus-Merzbacher Disease 24 coordinate concepts∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ Pelizaeus-Merzbacher Disease 24 coordinate concepts∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ Pelizaeus-Merzbacher Disease 24 coordinate concepts∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ Pelizaeus-Merzbacher Disease 24 coordinate concepts∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ |
Broader terms: | ∟ ∟ ∟ ∟ Pelizaeus-Merzbacher Disease ∟ ∟ ∟ ∟ ∟ Pelizaeus-Merzbacher Disease ∟ ∟ ∟ ∟ Pelizaeus-Merzbacher Disease ∟ ∟ ∟ ∟ ∟ Pelizaeus-Merzbacher Disease |
History note: | |
historyNote*: | 2000; use CEREBRAL SCLEROSIS, DIFFUSE 1979-1999
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publicMeSHNote*: | 2000; see CEREBRAL SCLEROSIS, DIFFUSE 1979-1999
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Scope note: | A rare, slowly progressive disorder of myelin formation. Subtypes are referred to as classic, congenital, transitional, and adult forms of this disease. The classic form is X-chromosome linked, has its onset in infancy and is associated with a mutation of the proteolipid protein gene. Clinical manifestations include TREMOR, spasmus nutans, roving eye movements, ATAXIA, spasticity, and NYSTAGMUS, CONGENITAL. Death occurs by the third decade of life. The congenital form has similar characteristics but presents early in infancy and features rapid disease progression. Transitional and adult subtypes have a later onset and less severe symptomatology. Pathologic features include patchy areas of demyelination with preservation of perivascular islands (trigoid appearance). (From Menkes, Textbook of Child Neurology, 5th ed, p190)
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activeMeSHYear*: | |
dateCreated*: | 1999-11-04X |
dateEstablished*: | 2000-01-01X |
dateRevised*: | 2005-07-21X |
recordAuthorizer*: | agsX |
recordMaintainer*: | agsX |
recordOriginator*: | KEVX |
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Labels and equivalent concepts: | Pelizaeus-Merzbacherin sairaus (fi) XCockayne-Pelizaeus-Merzbacherin tauti (fi, replaced) Pelizaeus-Merzbacherin tauti (fi, replaced) Cockayne-Pelizaeus-Merzbacherin sairaus (fi, replaced) Pelizaeus-Merzbachers sjukdom (sv) Cockayne-Pelizaeus-Merzbachers sjukdom (sv, replaced) Transitional Pelizaeus-Merzbacher Disease (en, replaced) Pelizaeus-Merzbacher Disease, Adult (en, replaced) Pelizaeus-Merzbacher Disease, Classic (en, replaced) Atypical Pelizaeus-Merzbacher Disease (en, replaced) |
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