Diffuse Cerebral Sclerosis of Schilder
"Diffuse Cerebral Sclerosis of Schilder" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
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)
Descriptor ID |
D002549
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MeSH 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
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Concept/Terms |
Diffuse Cerebral Sclerosis of Schilder- Diffuse Cerebral Sclerosis of Schilder
- Encephalitis Periaxialis
- Encephalitis Periaxialis Concentrica
- Sudanophilic Cerebral Sclerosis
- Myelinoclastic Diffuse Sclerosis
- Diffuse Scleroses, Myelinoclastic
- Diffuse Sclerosis, Myelinoclastic
- Myelinoclastic Diffuse Scleroses
- Scleroses, Myelinoclastic Diffuse
- Sclerosis, Myelinoclastic Diffuse
- Poliodystrophia Cerebri
- Schilder Disease
- Disease, Schilder
- Schilder's Disease
- Disease, Schilder's
- Schilders Disease
- Cerebral Sclerosis, Diffuse
- Diffuse Cerebral Scleroses
- Diffuse Cerebral Sclerosis
- Sclerosis, Diffuse Cerebral
- Encephalitis Periaxialis Diffusa
Balo Concentric Sclerosis- Balo Concentric Sclerosis
- Concentric Sclerosis, Balo
- Balo's Concentric Sclerosis
- Concentric Sclerosis, Balo's
- Scleroses, Balo's Concentric
Alpers Syndrome- Alpers Syndrome
- Syndrome, Alpers
- Alpers Disease
- Alpers Progressive Infantile Poliodystrophy
- Progressive Sclerosing Poliodystrophy
- Progressive Sclerosing Poliodystrophies
- Alpers' Syndrome
- Alper Syndrome
- Alper's Syndrome
- Alpers-Huttenlocher Syndrome
- Alpers Huttenlocher Syndrome
- Syndrome, Alpers-Huttenlocher
- Neuronal Degeneration Of Childhood With Liver Disease, Progressive
- Progressive Neuronal Degeneration of Childhood with Liver Disease
- Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis
- Alpers' Disease
- Alper Disease
- Alper's Disease
- Disease, Alpers'
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Below are MeSH descriptors whose meaning is more general than "Diffuse Cerebral Sclerosis of Schilder".
Below are MeSH descriptors whose meaning is more specific than "Diffuse Cerebral Sclerosis of Schilder".
This graph shows the total number of publications written about "Diffuse Cerebral Sclerosis of Schilder" by people in this website by year, and whether "Diffuse Cerebral Sclerosis of Schilder" was a major or minor topic of these publications.
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Year | Major Topic | Minor Topic | Total |
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2005 | 1 | 0 | 1 |
2006 | 1 | 0 | 1 |
2008 | 0 | 1 | 1 |
2009 | 1 | 1 | 2 |
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Below are the most recent publications written about "Diffuse Cerebral Sclerosis of Schilder" by people in Profiles.
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De novo mutation in POLG leads to haplotype insufficiency and Alpers syndrome. Mitochondrion. 2009 Sep; 9(5):340-5.
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Disease mutations in the human mitochondrial DNA polymerase thumb subdomain impart severe defects in mitochondrial DNA replication. J Biol Chem. 2009 Jul 17; 284(29):19501-10.
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DNA polymerase gamma and mitochondrial disease: understanding the consequence of POLG mutations. Biochim Biophys Acta. 2009 May; 1787(5):312-9.
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Molecular diagnosis of Alpers syndrome. J Hepatol. 2006 Jul; 45(1):108-16.
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Mono-allelic POLG expression resulting from nonsense-mediated decay and alternative splicing in a patient with Alpers syndrome. DNA Repair (Amst). 2005 Dec 08; 4(12):1381-9.
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Cellular oxidation of lignoceric acid is regulated by the subcellular localization of lignoceroyl-CoA ligases. J Lipid Res. 1990 Apr; 31(4):583-95.
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Adrenoleukodystrophy: impaired oxidation of fatty acids due to peroxisomal lignoceroyl-CoA ligase deficiency. Arch Biochem Biophys. 1989 May 01; 270(2):722-8.
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In situ genetic complementation analysis of cells with generalized peroxisomal dysfunction. Hum Hered. 1989; 39(5):298-301.
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Peroxisomal lignoceroyl-CoA ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy. Proc Natl Acad Sci U S A. 1988 Oct; 85(20):7647-51.
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Fatty acid composition of cerebrosides, sulphatides and ceramides in murine leucodystrophy: the quaking mutant. J Neurochem. 1972 Feb; 19(2):307-12.