MeSH Browser

MeSH Heading: Diffuse Cerebral Sclerosis of Schilder
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
Unique ID: D002549
RDF Unique Identifier: http://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)
Entry Term(s): 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; Cerebral Sclerosis, Diffuse; Encephalitis Periaxialis; Encephalitis Periaxialis Concentrica; Encephalitis Periaxialis Diffusa; Myelinoclastic Diffuse Sclerosis; Neuronal Degeneration Of Childhood With Liver Disease, Progressive; Poliodystrophia Cerebri; Progressive Neuronal Degeneration of Childhood with Liver Disease; Progressive Sclerosing Poliodystrophy; Schilder Disease; Schilder's Disease; Sudanophilic Cerebral Sclerosis
NLM Classification #: WS 341
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
History Note: 2000(1963)
Date Established: 2000/01/01
Date of Entry: 1999/01/01
Revision Date: 2013/07/08

Allowable Qualifiers: blood (BL)

cerebrospinal fluid (CF)

chemically induced (CI)

classification (CL)

complications (CO)

congenital (CN)

diagnosis (DI)

diagnostic imaging (DG)

diet therapy (DH)

drug therapy (DT)

economics (EC)

embryology (EM)

enzymology (EN)

epidemiology (EP)

ethnology (EH)

etiology (ET)

genetics (GE)

history (HI)

immunology (IM)

metabolism (ME)

microbiology (MI)

mortality (MO)

nursing (NU)

parasitology (PS)

pathology (PA)

physiopathology (PP)

prevention & control (PC)

psychology (PX)

radiotherapy (RT)

rehabilitation (RH)

surgery (SU)

therapy (TH)

urine (UR)

veterinary (VE)

virology (VI)

Nervous System Diseases [C10]
- Autoimmune Diseases of the Nervous System [C10.114]
  - Demyelinating Autoimmune Diseases, CNS [C10.114.375]

Nervous System Diseases [C10]
- Central Nervous System Diseases [C10.228]
  - Brain Diseases [C10.228.140]

Nervous System Diseases [C10]
- Central Nervous System Diseases [C10.228]
  - Brain Diseases [C10.228.140]
    - Leukoencephalopathies [C10.228.140.695]
      - Demyelinating Autoimmune Diseases, CNS [C10.228.140.695.562]
        
        Diffuse Cerebral Sclerosis of Schilder [C10.228.140.695.562.112]
        
        Encephalomyelitis, Acute Disseminated [C10.228.140.695.562.225]
        
        Encephalomyelitis, Autoimmune, Experimental [C10.228.140.695.562.250]

Nervous System Diseases [C10]
- Demyelinating Diseases [C10.314]
  - Demyelinating Autoimmune Diseases, CNS [C10.314.350]

Immune System Diseases [C20]
- Autoimmune Diseases [C20.111]
  - Autoimmune Diseases of the Nervous System [C20.111.258]
    - Demyelinating Autoimmune Diseases, CNS [C20.111.258.250]

Diffuse Cerebral Sclerosis of Schilder Preferred

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)
Terms: Diffuse Cerebral Sclerosis of Schilder Preferred Term
Term UI T365199
Date11/04/1999
LexicalTag EPO
ThesaurusID; Encephalitis Periaxialis Diffusa
Term UI T014249
Date12/29/1988
LexicalTag NON
ThesaurusID NLM (1990); Myelinoclastic Diffuse Sclerosis
Term UI T014252
Date12/29/1988
LexicalTag NON
ThesaurusID NLM (1990); Poliodystrophia Cerebri
Term UI T007432
Date12/16/1991
LexicalTag NON
ThesaurusID NLM (1993); Schilder Disease
Term UI T014250
Date06/15/1984
LexicalTag EPO
ThesaurusID; Schilder's Disease
Term UI T014251
Date03/30/1974
LexicalTag EPO
ThesaurusID UNK (19XX); Sudanophilic Cerebral Sclerosis
Term UI T811449
Date11/15/2011
LexicalTag NON
ThesaurusID OMIM (2013); Cerebral Sclerosis, Diffuse
Term UI T007431
Date01/01/1999
LexicalTag NON
ThesaurusID NLM (1966); Encephalitis Periaxialis Concentrica
Term UI T014247
Date12/29/1988
LexicalTag NON
ThesaurusID NLM (1990); Encephalitis Periaxialis
Term UI T014248
Date01/01/1999
LexicalTag NON
ThesaurusID NLM (1966)

Alpers Syndrome Related

Balo Concentric Sclerosis Related

Diffuse Cerebral Sclerosis of Schilder MeSH Descriptor Data 2025