JSON

Diffuse Cerebral Sclerosis of Schilder

Go to external page http://purl.bioontology.org/ontology/MESH/D002549

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)

Instance info

Synonyms

Alper's Syndrome, Alpers Syndrome, Alpers' Syndrome, Alpers Disease, Scleroses, Myelinoclastic Diffuse, Poliodystrophia Cerebri, Baló-Krankheit (DE), Cerebral Sclerosis, Diffuse, Diffuse zerebrale Sklerose (Schilder) (DE), Syndrome, Alpers, Balo Concentric Sclerosis, Alper Disease, Sclerosis, Diffuse Cerebral, Progressive Neuronal Degeneration of Childhood with Liver Disease, Progressive Sclerosing Poliodystrophies, Disease, Schilder's, Concentric Sclerosis, Balo's, Sclerosis, Myelinoclastic Diffuse, Myelinoclastic Diffuse Scleroses, Myelinoclastic Diffuse Sclerosis, Schilder's Disease, Alper's Disease, Disease, Alpers', Encephalitis Periaxialis Concentrica, Alpers' Disease, Alper Syndrome, Alpers-Huttenlocher Syndrome, Schilders Disease, Diffuse Scleroses, Myelinoclastic, Zerebrale Sklerose, diffuse (DE), Disease, Schilder, Concentric Sclerosis, Balo, Encephalitis periaxialis concentrica (DE), Syndrome, Alpers-Huttenlocher, Alpers-Syndrom (DE), Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis, Encephalitis periaxialis diffusa (DE), Encephalitis periaxialis (DE), Neuronal Degeneration Of Childhood With Liver Disease, Progressive, Encephalitis Periaxialis, Poliodystrophia cerebri (DE), Progressive Sclerosing Poliodystrophy, Baló-konzentrische Sklerose (DE), Encephalitis Periaxialis Diffusa, Scleroses, Balo's Concentric, Balo's Concentric Sclerosis, Alpers Huttenlocher Syndrome, Schilder Disease, Diffuse Sklerose, myelinoklastische (DE), Hirnsklerose, diffuse (DE), Diffuse Sclerosis, Myelinoclastic, Diffuse Cerebral Sclerosis, Schilder-Krankheit (DE), Diffuse Cerebral Scleroses, Alpers Progressive Infantile Poliodystrophy, Sudanophilic Cerebral Sclerosis

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

DC

1

DX

20000101

HN

2000(1963)

MDA

19990101

MMR

20130708

MN

C20.111.258.250.175,C10.228.140.695.562.112,C10.228.140.400,C10.114.375.112,C10.314.350.112

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

QB

http://purl.bioontology.org/ontology/MESH/Q000097,http://purl.bioontology.org/ontology/MESH/Q000175,http://purl.bioontology.org/ontology/MESH/Q000196,http://purl.bioontology.org/ontology/MESH/Q000151,http://purl.bioontology.org/ontology/MESH/Q000150,http://purl.bioontology.org/ontology/MESH/Q000191,http://purl.bioontology.org/ontology/MESH/Q000139,http://purl.bioontology.org/ontology/MESH/Q000534,http://purl.bioontology.org/ontology/MESH/Q000378,http://purl.bioontology.org/ontology/MESH/Q000532,http://purl.bioontology.org/ontology/MESH/Q000235,http://purl.bioontology.org/ontology/MESH/Q000652,http://purl.bioontology.org/ontology/MESH/Q000453,http://purl.bioontology.org/ontology/MESH/Q000178,http://purl.bioontology.org/ontology/MESH/Q000134,http://purl.bioontology.org/ontology/MESH/Q000276,http://purl.bioontology.org/ontology/MESH/Q000000981,http://purl.bioontology.org/ontology/MESH/Q000473,http://purl.bioontology.org/ontology/MESH/Q000451,http://purl.bioontology.org/ontology/MESH/Q000517,http://purl.bioontology.org/ontology/MESH/Q000382,http://purl.bioontology.org/ontology/MESH/Q000601,http://purl.bioontology.org/ontology/MESH/Q000821,http://purl.bioontology.org/ontology/MESH/Q000469,http://purl.bioontology.org/ontology/MESH/Q000523,http://purl.bioontology.org/ontology/MESH/Q000401,http://purl.bioontology.org/ontology/MESH/Q000201,http://purl.bioontology.org/ontology/MESH/Q000662,http://purl.bioontology.org/ontology/MESH/Q000266,http://purl.bioontology.org/ontology/MESH/Q000145,http://purl.bioontology.org/ontology/MESH/Q000188,http://purl.bioontology.org/ontology/MESH/Q000628,http://purl.bioontology.org/ontology/MESH/Q000209,http://purl.bioontology.org/ontology/MESH/Q000208,http://purl.bioontology.org/ontology/MESH/Q000503

SIB

http://purl.bioontology.org/ontology/MESH/D009471,http://purl.bioontology.org/ontology/MESH/D001929,http://purl.bioontology.org/ontology/MESH/D001928,http://purl.bioontology.org/ontology/MESH/D013353,http://purl.bioontology.org/ontology/MESH/D004660,http://purl.bioontology.org/ontology/MESH/D004681,http://purl.bioontology.org/ontology/MESH/D002534,http://purl.bioontology.org/ontology/MESH/D004678,http://purl.bioontology.org/ontology/MESH/D000071072,http://purl.bioontology.org/ontology/MESH/D019585,http://purl.bioontology.org/ontology/MESH/D000550,http://purl.bioontology.org/ontology/MESH/D004673,http://purl.bioontology.org/ontology/MESH/D019586,http://purl.bioontology.org/ontology/MESH/D007027,http://purl.bioontology.org/ontology/MESH/D001925,http://purl.bioontology.org/ontology/MESH/D001926,http://purl.bioontology.org/ontology/MESH/D003704,http://purl.bioontology.org/ontology/MESH/D001922,http://purl.bioontology.org/ontology/MESH/D001304,http://purl.bioontology.org/ontology/MESH/D009188,http://purl.bioontology.org/ontology/MESH/D013786,http://purl.bioontology.org/ontology/MESH/D009103,http://purl.bioontology.org/ontology/MESH/D001480,http://purl.bioontology.org/ontology/MESH/D065166,http://purl.bioontology.org/ontology/MESH/D006849,http://purl.bioontology.org/ontology/MESH/D004684,http://purl.bioontology.org/ontology/MESH/D002561,http://purl.bioontology.org/ontology/MESH/D020236,http://purl.bioontology.org/ontology/MESH/D056784,http://purl.bioontology.org/ontology/MESH/D020773,http://purl.bioontology.org/ontology/MESH/D000405,http://purl.bioontology.org/ontology/MESH/D004827,http://purl.bioontology.org/ontology/MESH/D002526,http://purl.bioontology.org/ontology/MESH/D001932,http://purl.bioontology.org/ontology/MESH/D001930,http://purl.bioontology.org/ontology/MESH/D019150

TERMUI

T007434,T007435,T007432,T007433,T014249,T014247,T365192,T014248,T014245,T014252,T812087,T014250,T811449,T014251,T769476,T840857,T769475,T769477,T365199,T752574,T752575,T007431

TH

GHR (2014),NLM (1990),NLM (1993),ORD (2010),OMIM (2013),NLM (2011),NLM (1966),NLM (2000),NLM (2010),UNK (19XX),NLM (2014)

cui

C0205710,C0007795,C0004712

hasSTY

http://purl.bioontology.org/ontology/STY/T047

mapped from

http://purl.bioontology.org/ontology/MESH/C536319,http://purl.bioontology.org/ontology/MESH/C536318

tui

T047

Instance relations

Types