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DeCS
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Descriptor English:
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Diffuse Cerebral Sclerosis of Schilder
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Descriptor Spanish:
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Esclerosis Cerebral Difusa de Schilder
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Descriptor Portuguese:
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Esclerose Cerebral Difusa de Schilder
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Synonyms English:
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Alpers Syndrome
Poliodystrophia Cerebri
Cerebral Sclerosis, Diffuse
Myelinoclastic Diffuse Sclerosis
Schilder Disease
Encephalitis Periaxialis
Balo Concentric Sclerosis
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Tree Number:
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C10.114.375.112
C10.228.140.400
C10.228.140.695.562.112
C10.314.350.112
C20.111.258.250.175
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Definition English:
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A rare central nervous system demyelinating condition affecting children and young adults. Pathologic findings include a large, sharply defined, asymmetric focus of myelin destruction that may involve an entire lobe or cerebral hemisphere. The clinical course tends to be progressive and includes dementia, cortical blindness, cortical deafness, spastic hemiplegia, and pseudobulbar palsy. Concentric sclerosis of Balo is differentiated from diffuse cerebral sclerosis of Schilder by the pathologic finding of alternating bands of destruction and preservation of myelin in concentric rings. Alpers' Syndrome refers to a heterogeneous group of diseases that feature progressive cerebral deterioration and liver disease. (From Adams et al., Principles of Neurology, 6th ed, p914; Dev Neurosci 1991;13(4-5):267-73) |
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History Note English:
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2000(1963)
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Allowable Qualifiers English:
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Record Number:
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2589
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Unique Identifier:
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D002549
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Occurrence in VHL:
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Similar:
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DeCS CID-10 SciELO LILACS LIS
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