Structured Summary
Abstract
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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Synonyms
44 entry terms
- Cerebral Sclerosis, Diffuse
- Encephalitis Periaxialis
- Encephalitis Periaxialis Concentrica
- Encephalitis Periaxialis Diffusa
- Myelinoclastic Diffuse Sclerosis
- Poliodystrophia Cerebri
- Schilder Disease
- Schilder's Disease
- Sudanophilic Cerebral Sclerosis
- Diffuse Cerebral Scleroses
- Diffuse Cerebral Sclerosis
- Diffuse Scleroses, Myelinoclastic
- Diffuse Sclerosis, Myelinoclastic
- Disease, Schilder
- Disease, Schilder's
- Myelinoclastic Diffuse Scleroses
- Schilders Disease
- Scleroses, Myelinoclastic Diffuse
- Sclerosis, Diffuse Cerebral
- Sclerosis, Myelinoclastic Diffuse
- 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
- Neuronal Degeneration Of Childhood With Liver Disease, Progressive
- Progressive Neuronal Degeneration of Childhood with Liver Disease
- Progressive Sclerosing Poliodystrophy
- Alper Disease
- Alper Syndrome
- Alper's Disease
- Alper's Syndrome
- Alpers Huttenlocher Syndrome
- Concentric Sclerosis, Balo
- Concentric Sclerosis, Balo's
- Disease, Alpers'
- Progressive Sclerosing Poliodystrophies
- Scleroses, Balo's Concentric
- Syndrome, Alpers
- Syndrome, Alpers-Huttenlocher
MeSH Record
Aspects Covered
35 allowable subheadings
Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, congenital, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.
MeSH Record
History Note
2000(1963)
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WS 341
AMA Style
References
- National Library of Medicine. Diffuse Cerebral Sclerosis of Schilder. Medical Subject Headings (MeSH). 2026. Unique ID D002549. http://id.nlm.nih.gov/mesh/2026/D002549
- Diffuse Cerebral Sclerosis of Schilder. In: Wikipedia. https://en.wikipedia.org/wiki/Diffuse_myelinoclastic_sclerosis
- Diffuse Cerebral Sclerosis of Schilder. In: Wikidata. https://www.wikidata.org/wiki/Q2909420