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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1999 | 1 | 0 | 1 |
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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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Kinetic and structural changes in HsmtPheRS, induced by pathogenic mutations in human FARS2. Protein Sci. 2017 Aug; 26(8):1505-1516.
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Alpers syndrome: an unusual etiology of failure to thrive. Ultrastruct Pathol. 2012 Aug; 36(4):219-21.
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Application of oligonucleotide array CGH in the detection of a large intragenic deletion in POLG associated with Alpers Syndrome. Mitochondrion. 2011 Jan; 11(1):104-7.
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POLG DNA testing as an emerging standard of care before instituting valproic acid therapy for pediatric seizure disorders. Seizure. 2010 Apr; 19(3):140-6.
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Molecular and clinical genetics of mitochondrial diseases due to POLG mutations. Hum Mutat. 2008 Sep; 29(9):E150-72.
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Alpers syndrome with prominent white matter changes. Brain Dev. 2008 Apr; 30(4):295-300.
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Proteolipid protein gene duplications causing Pelizaeus-Merzbacher disease: molecular mechanism and phenotypic manifestations. Ann Neurol. 1999 May; 45(5):624-32.
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Dietary erucic acid therapy for X-linked adrenoleukodystrophy. Neurology. 1989 Nov; 39(11):1415-22.
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Adrenoleukodystrophy: correlating MR imaging with CT. Radiology. 1987 Nov; 165(2):497-504.
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The adrenoleukodystrophies. Crit Rev Neurobiol. 1987; 3(1):29-88.