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Diffuse Cerebral Sclerosis of Schilder (CUI C0007795) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0007795

NCI Thesaurus Code: C84670  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

NCIt Definition: A rare congenital demyelinating disorder affecting the central nervous system. It is characterized by a demyelinating destructive lesion affecting an entire brain lobe or hemisphere. Signs and symptoms include dementia, cortical deafness and blindness, pseudobulbar palsy, and hemiplegia.

MSH Definition: 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)

Synonyms & Abbreviations: (see Synonym Details)
Cerebral Sclerosis, Diffuse
Diffuse cerebral degeneration in infancy
Diffuse Cerebral Scleroses
Diffuse Cerebral Sclerosis of Schilder [Disease/Finding]
Diffuse cerebral sclerosis of Schilder
diffuse cerebral sclerosis
Diffuse Scleroses, Myelinoclastic
Diffuse Sclerosis, Myelinoclastic
diffuse sclerosis
Disease, Schilder's
Disease, Schilder
ENCEPH PERIAXIALIS CONCENTRICA
ENCEPH PERIAXIALIS DIFFUSA
ENCEPH PERIAXIALIS
Encephalitis Periaxialis Concentrica
Encephalitis periaxialis diffusa
Encephalitis periaxialis, diffusa
Encephalitis periaxialis, Schilder's
Encephalitis Periaxialis
ENCEPHALITIS, PERIAXIALIS DIFFUSA
Infantile poliodystrophy
LEUKODYSTROPHY, SUDANOPHILIC
LEUKOENCEPHALITIS, SUBACUTE
Myelinoclastic Diffuse Scleroses
Myelinoclastic Diffuse Sclerosis
PNDC
Poliodystrophia cerebri progressiva
Poliodystrophia cerebri
Progressive cerebral poliodystrophy
SCHILDER DISEASE
SCHILDER DIS
Schilder's disease (disorder)
Schilder's disease
Schilders Disease
SCHILDERS DIS
Scleroses, Myelinoclastic Diffuse
Sclerosis, Diffuse Cerebral
SCLEROSIS, DIFFUSE
Sclerosis, Myelinoclastic Diffuse
SCLEROSIS, SUDANOPHILIC CEREBRAL
Sudanophilic Cerebral Sclerosis

External Source Codes: 
NCI Thesaurus Code C84670 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AQL BL CF CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000448009 SNOMEDCT_US
CTV3ID XE15S SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 1849-5479 CSP
DID 2042-2215 CSP
DISEASE_IDENTIFIER_ID 17164 GARD
DISEASE_IDENTIFIER_ID 17165 GARD
DISEASE_IDENTIFIER_ID 17166 GARD
DISEASE_IDENTIFIER_ID 17169 GARD
DISEASE_IDENTIFIER_ID 17170 GARD
DISEASE_IDENTIFIER_ID 17433 GARD
DX 20000101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
HN 2000(1963) MSH
IAN DEFAULT ICD10
ICE Balo's concentric sclerosis; Encephalitis periaxialis: {concentrica [Balo's]; diffusa [Schilder's]} ICD9CM
IDENTIFIER_TYPE_ID 1 GARD
INCLUSION_TERM Periaxial encephalitis ICD10
INCLUSION_TERM Schilder's disease ICD10
IS_ACTIVE true GARD
MDA 19990101 MSH
MESH_DEFINITION 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) NDFRT
MESH_DUI D002549 NDFRT
MESH_NAME Diffuse Cerebral Sclerosis of Schilder NDFRT
MESH_UI M0003893 NDFRT
MIMTYPE 0 OMIM
MIMTYPEMEANING Other, mainly phenotypes with suspected mendelian basis OMIM
MIMTYPEVALUE none OMIM
MMR 20130708 MSH
MN C10.114.375.112 MSH
MN C10.228.140.400 MSH
MN C10.228.140.695.562.112 MSH
MN C10.314.350.112 MSH
MN C20.111.258.250.175 MSH
NDFRT_KIND DISEASE_KIND NDFRT
NUI N0000000726 NDFRT
PM 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 MSH
PRIMARY_PATH 10049020$10012302$10012303$10029205$Encephalitis periaxialis diffusa$Demyelinating disorders NEC$Demyelinating disorders$Nervous system disorders MDR
PRIMARY_SOC 10029205 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 20.0 MDR
PT_IN_VERSION 20.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RXAUI 3131797 RXNORM
RXAUI 3146812 RXNORM
RXAUI 3146813 RXNORM
RXAUI 3153682 RXNORM
RXAUI 3153683 RXNORM
RXAUI 3153684 RXNORM
RXAUI 3200585 RXNORM
RXAUI 3221847 RXNORM
RXAUI 3236720 RXNORM
RXAUI 3236721 RXNORM
RXAUI 5039979 RXNORM
RXCUI 1022252 RXNORM
SMQ_TERM_ADDVERSION 10.0 MDR
SMQ_TERM_ADDVERSION 11.1 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 11.1 MDR
SMQ_TERM_LMVERSION 14.0 MDR
SMQ_TERM_SCOPE 1 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMED_CID 49692006 NDFRT
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS G37.0 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~G37.0 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS G37.0 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~G37.0 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XE15S SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T007431 MSH
TERMUI T007432 MSH
TERMUI T014247 MSH
TERMUI T014248 MSH
TERMUI T014249 MSH
TERMUI T014250 MSH
TERMUI T014251 MSH
TERMUI T014252 MSH
TERMUI T365199 MSH
TERMUI T811449 MSH
TH GHR (2014) MSH
TH NLM (1966) MSH
TH NLM (1990) MSH
TH NLM (1993) MSH
TH NLM (2000) MSH
TH OMIM (2013) MSH
TH UNK (19XX) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0007795

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