Concept information
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Central Nervous System Diseases
Brain Diseases
Leukoencephalopathies
Demyelinating Autoimmune Diseases, CNS
Immune System Diseases
Autoimmune Diseases
Autoimmune Diseases of the Nervous System
Demyelinating Autoimmune Diseases, CNS
Preferred term
Diffuse Cerebral Sclerosis of Schilder
Type
-
Topical Descriptor
Broader concept
Entry terms
- Cerebral Sclerosis, Diffuse
- Diffuse Cerebral Scleroses
- Diffuse Cerebral Sclerosis
- Diffuse Scleroses, Myelinoclastic
- Diffuse Sclerosis, Myelinoclastic
- Disease, Schilder
- Disease, Schilder's
- Encephalitis Periaxialis
- Encephalitis Periaxialis Concentrica
- Encephalitis Periaxialis Diffusa
- Myelinoclastic Diffuse Scleroses
- Myelinoclastic Diffuse Sclerosis
- Poliodystrophia Cerebri
- Schilder Disease
- Schilder's Disease
- Schilders Disease
- Scleroses, Myelinoclastic Diffuse
- Sclerosis, Diffuse Cerebral
- Sclerosis, Myelinoclastic Diffuse
- Sudanophilic Cerebral Sclerosis
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)
History note
- 2000(1963)
In other languages
-
Finnish
-
encephalitis periaxialis
-
keskuskermoston diffuusi skleroosi
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morbus Schilder
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periaksiaalinen aivokuume
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periaksiaalinen aivotulehdus
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periaksiaalinen enkefaliitti
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periaxial encefalit
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Schilder
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Schilderin diffuusi aivojen kovettuma
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Schilderin diffuusi aivojen skleroosi
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Schilderin diffuusi aivokovettuma
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Schilderin diffuusi aivoskleroosi
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Schilders sjukdom
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sclerosis diffusa
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Swedish
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Alpers syndrom
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Balos koncentrisk skleros
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Encephalitis periaxialis
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Periaxial encefalit
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Schilders sjukdom
URI
http://www.yso.fi/onto/mesh/D002549
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