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Diffuse Cerebral Sclerosis of Schilder MeSH Descriptor Data 2022


MeSH Heading
Diffuse Cerebral Sclerosis of Schilder
Tree Number(s)
C10.114.375.112
C10.228.140.400
C10.228.140.695.562.112
C10.314.350.112
C20.111.258.250.175
Unique ID
D002549
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D002549
Scope Note
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)
Entry Term(s)
Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis
Alpers Disease
Alpers Progressive Infantile Poliodystrophy
Alpers Syndrome
Alpers' Disease
Alpers' Syndrome
Alpers-Huttenlocher Syndrome
Balo Concentric Sclerosis
Balo's Concentric Sclerosis
Cerebral Sclerosis, Diffuse
Encephalitis Periaxialis
Encephalitis Periaxialis Concentrica
Encephalitis Periaxialis Diffusa
Myelinoclastic Diffuse Sclerosis
Neuronal Degeneration Of Childhood With Liver Disease, Progressive
Poliodystrophia Cerebri
Progressive Neuronal Degeneration of Childhood with Liver Disease
Progressive Sclerosing Poliodystrophy
Schilder Disease
Schilder's Disease
Sudanophilic Cerebral Sclerosis
NLM Classification #
WS 341
Public MeSH Note
2000; see CEREBRAL SCLEROSIS, DIFFUSE 1963-1999; for ENCEPHALITIS PERIAXIALIS see ENCEPHALITIS PERIAXIALIS 1963-1999; for BALO'S DISEASE, ENCEPHALITIS PERIAXIALIS DIFFUSA, MYELINOCLASTIC DIFFUSE SCLEROSIS, & SCHILDER'S DISEASE see ENCEPHALITIS PERIAXIALIS 1990-1999
History Note
2000(1963)
Date Established
2000/01/01
Date of Entry
1999/01/01
Revision Date
2013/07/08
Diffuse Cerebral Sclerosis of Schilder Preferred
Balo Concentric Sclerosis Related
Alpers Syndrome Related
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