| Descriptor English: | Diffuse Cerebral Sclerosis of Schilder | ||||||
| Descriptor Spanish: |
Esclerosis Cerebral Difusa de Schilder
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| Descriptor Portuguese: | Esclerose Cerebral Difusa de Schilder | ||||||
| Descriptor French: | Sclérose cérébrale diffuse de Schilder | ||||||
| Entry term(s): |
Alper Disease Alper Syndrome Alper's Disease Alper's Syndrome Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis Alpers Disease Alpers Huttenlocher Syndrome Alpers Progressive Infantile Poliodystrophy Alpers Syndrome Alpers' Disease Alpers' Syndrome Alpers-Huttenlocher Syndrome Balo Concentric Sclerosis Balo's Concentric Sclerosis Cerebral Sclerosis, Diffuse Concentric Sclerosis, Balo Concentric Sclerosis, Balo's Diffuse Cerebral Scleroses Diffuse Cerebral Sclerosis Diffuse Scleroses, Myelinoclastic Diffuse Sclerosis, Myelinoclastic Disease, Alpers' Disease, Schilder Disease, Schilder's Encephalitis Periaxialis Encephalitis Periaxialis Concentrica Encephalitis Periaxialis Diffusa Myelinoclastic Diffuse Scleroses Myelinoclastic Diffuse Sclerosis Neuronal Degeneration Of Childhood With Liver Disease, Progressive Poliodystrophia Cerebri Progressive Neuronal Degeneration of Childhood with Liver Disease Progressive Sclerosing Poliodystrophies Progressive Sclerosing Poliodystrophy Schilder Disease Schilder's Disease Schilders Disease Scleroses, Balo's Concentric Scleroses, Myelinoclastic Diffuse Sclerosis, Diffuse Cerebral Sclerosis, Myelinoclastic Diffuse Sudanophilic Cerebral Sclerosis Syndrome, Alpers Syndrome, Alpers-Huttenlocher |
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| 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 |
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| RDF Unique Identifier: | https://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) |
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| Allowable Qualifiers: |
BL blood CF cerebrospinal fluid CI chemically induced CL classification CN congenital CO complications DG diagnostic imaging DH diet therapy DI diagnosis DT drug therapy EC economics EH ethnology EM embryology EN enzymology EP epidemiology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PA pathology PC prevention & control PP physiopathology PS parasitology PX psychology RH rehabilitation RT radiotherapy SU surgery TH therapy UR urine VE veterinary VI virology |
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| 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 |
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| History Note: | 2000(1963) |
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| DeCS ID: | 2589 | ||||||
| Unique ID: | D002549 | ||||||
| NLM Classification: | WL 348 | ||||||
| Documents indexed in the Virtual Health Library (VHL): | Click here to access the VHL documents | ||||||
| Date Established: | 2000/01/01 | ||||||
| Date of Entry: | 1999/01/01 | ||||||
| Revision Date: | 2013/07/08 |
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Diffuse Cerebral Sclerosis of Schilder
- Preferred
Balo Concentric Sclerosis
- Related but not broader or narrower
Alpers Syndrome
- Related but not broader or narrower
| Concept UI |
M0003893 |
| 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) |
| Preferred term | Diffuse Cerebral Sclerosis of Schilder |
| Entry term(s) |
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 |
| Concept UI |
M0007338 |
| Preferred term | Balo Concentric Sclerosis |
| Entry term(s) |
Balo's Concentric Sclerosis Concentric Sclerosis, Balo Concentric Sclerosis, Balo's Scleroses, Balo's Concentric |
| Concept UI |
M0003894 |
| Preferred term | Alpers Syndrome |
| Entry term(s) |
Alper Disease Alper Syndrome Alper's Disease Alper's Syndrome Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis Alpers Disease Alpers Huttenlocher Syndrome Alpers Progressive Infantile Poliodystrophy Alpers' Disease Alpers' Syndrome Alpers-Huttenlocher Syndrome Disease, Alpers' Neuronal Degeneration Of Childhood With Liver Disease, Progressive Progressive Neuronal Degeneration of Childhood with Liver Disease Progressive Sclerosing Poliodystrophies Progressive Sclerosing Poliodystrophy Syndrome, Alpers Syndrome, Alpers-Huttenlocher |
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