Esitystapa käsitteiden/termien suhteille:
Näytä käsitteiden/termien suhteet:
Näytä käsitteiden/termien suhteet:
Pelizaeus-Merzbacherin sairaus
Laajemmat termit: | ∟ ∟ ∟ ∟ Pelizaeus-Merzbacherin sairaus ∟ ∟ ∟ ∟ ∟ Pelizaeus-Merzbacherin sairaus ∟ ∟ ∟ ∟ ∟ Pelizaeus-Merzbacherin sairaus ∟ ∟ ∟ ∟ Pelizaeus-Merzbacherin sairaus |
historyNote*: | 2000; use CEREBRAL SCLEROSIS, DIFFUSE 1979-1999
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Huomautus: | Diffuse Cerebral Sclerosis of Schilder (1966-1999) Multiple Sclerosis (1966-1999) |
Huomautus: | 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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publicMeSHNote*: | 2000; see CEREBRAL SCLEROSIS, DIFFUSE 1979-1999
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activeMeSHYear*: | 2003 2004 2005 2006 2006A 2007 |
dateCreated*: | 1999-11-04 |
dateEstablished*: | 2000-01-01 |
dateRevised*: | 2005-07-21 |
recordAuthorizer*: | ags |
recordMaintainer*: | ags |
recordOriginator*: | KEV |
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Nimikkeet ja vastaavat käsitteet: | Cockayne-Pelizaeus-Merzbacherin tauti (fi, korvattu) Pelizaeus-Merzbacherin tauti (fi, korvattu) Cockayne-Pelizaeus-Merzbacherin sairaus (fi, korvattu) Pelizaeus-Merzbachers sjukdom (sv) Cockayne-Pelizaeus-Merzbachers sjukdom (sv, korvattu) Pelizaeus-Merzbacher Disease (en) Transitional Pelizaeus-Merzbacher Disease (en, korvattu) Pelizaeus-Merzbacher Disease, Adult (en, korvattu) Pelizaeus-Merzbacher Disease, Classic (en, korvattu) Atypical Pelizaeus-Merzbacher Disease (en, korvattu) |
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