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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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Alper Disease
Alper Syndrome
Alper's Disease
Alper's Syndrome
Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis
Alpers Disease
Alpers Huttenlocher Syndrome
Alpers Progressive Infantile Poliodystrophy
Alpers Syndrome
Alpers' Disease
Alpers' Syndrome
Alpers-Huttenlocher Syndrome
Balo Concentric Sclerosis
Balo's Concentric Sclerosis
Cerebral Sclerosis, Diffuse
Concentric Sclerosis, Balo
Concentric Sclerosis, Balo's
Diffuse Cerebral Scleroses
Diffuse Cerebral Sclerosis
Diffuse Scleroses, Myelinoclastic
Diffuse Sclerosis, Myelinoclastic
Disease, Alpers'
Disease, Schilder
Disease, Schilder's
Encephalitis Periaxialis
Encephalitis Periaxialis Concentrica
Encephalitis Periaxialis Diffusa
Myelinoclastic Diffuse Scleroses
Myelinoclastic Diffuse Sclerosis
Neuronal Degeneration Of Childhood With Liver Disease, Progressive
Poliodystrophia Cerebri
Progressive Neuronal Degeneration of Childhood with Liver Disease
Progressive Sclerosing Poliodystrophies
Progressive Sclerosing Poliodystrophy
Schilder Disease
Schilder's Disease
Schilders Disease
Scleroses, Balo's Concentric
Scleroses, Myelinoclastic Diffuse
Sclerosis, Diffuse Cerebral
Sclerosis, Myelinoclastic Diffuse
Sudanophilic Cerebral Sclerosis
Syndrome, Alpers
Syndrome, Alpers-Huttenlocher
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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
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