loading...| Preferred Name |
Diffuse Cerebral Sclerosis of Schilder |
| Synonyms |
Myelinoclastic Diffuse Sclerosis |
| Definitions |
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) |
| ID |
http://purl.bioontology.org/ontology/MESH/D002549 |
| altLabel |
Myelinoclastic Diffuse Sclerosis Myelinoclastic Diffuse Scleroses Sclerosis, Myelinoclastic Diffuse Alpers Progressive Infantile Poliodystrophy Diffuse Scleroses, Myelinoclastic Disease, Schilder's Alper Disease Alpers Disease Alper's Syndrome Alpers Huttenlocher Syndrome Syndrome, Alpers Scleroses, Balo's Concentric Alpers' Syndrome Cerebral Sclerosis, Diffuse Alpers' Disease Balo's Concentric Sclerosis Disease, Alpers' Poliodystrophia Cerebri Schilder Disease Scleroses, Myelinoclastic Diffuse Diffuse Sclerosis, Myelinoclastic Alpers Syndrome Encephalitis Periaxialis Concentrica Sclerosis, Diffuse Cerebral Syndrome, Alpers-Huttenlocher Alper Syndrome Diffuse Cerebral Scleroses Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis Concentric Sclerosis, Balo Progressive Sclerosing Poliodystrophy Schilders Disease Balo Concentric Sclerosis Progressive Sclerosing Poliodystrophies Sudanophilic Cerebral Sclerosis Neuronal Degeneration Of Childhood With Liver Disease, Progressive Alper's Disease Progressive Neuronal Degeneration of Childhood with Liver Disease Diffuse Cerebral Sclerosis Alpers-Huttenlocher Syndrome Disease, Schilder Encephalitis Periaxialis Diffusa Encephalitis Periaxialis Concentric Sclerosis, Balo's Schilder's Disease |
| 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 |
| cui |
C0205710 C0007795 C0004712 |
| DC |
1 |
| 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) |
| DX |
20000101 |
| HN |
2000(1963) |
| Inverse of AQ |
http://purl.bioontology.org/ontology/MESH/Q000401 http://purl.bioontology.org/ontology/MESH/Q000150 http://purl.bioontology.org/ontology/MESH/Q000188 http://purl.bioontology.org/ontology/MESH/Q000517 http://purl.bioontology.org/ontology/MESH/Q000201 http://purl.bioontology.org/ontology/MESH/Q000145 http://purl.bioontology.org/ontology/MESH/Q000503 http://purl.bioontology.org/ontology/MESH/Q000453 http://purl.bioontology.org/ontology/MESH/Q000208 http://purl.bioontology.org/ontology/MESH/Q000134 http://purl.bioontology.org/ontology/MESH/Q000821 http://purl.bioontology.org/ontology/MESH/Q000382 http://purl.bioontology.org/ontology/MESH/Q000601 http://purl.bioontology.org/ontology/MESH/Q000235 http://purl.bioontology.org/ontology/MESH/Q000523 http://purl.bioontology.org/ontology/MESH/Q000276 http://purl.bioontology.org/ontology/MESH/Q000191 http://purl.bioontology.org/ontology/MESH/Q000652 http://purl.bioontology.org/ontology/MESH/Q000662 http://purl.bioontology.org/ontology/MESH/Q000151 http://purl.bioontology.org/ontology/MESH/Q000628 http://purl.bioontology.org/ontology/MESH/Q000196 http://purl.bioontology.org/ontology/MESH/Q000534 http://purl.bioontology.org/ontology/MESH/Q000266 http://purl.bioontology.org/ontology/MESH/Q000532 http://purl.bioontology.org/ontology/MESH/Q000139 http://purl.bioontology.org/ontology/MESH/Q000378 http://purl.bioontology.org/ontology/MESH/Q000175 http://purl.bioontology.org/ontology/MESH/Q000000981 http://purl.bioontology.org/ontology/MESH/Q000097 http://purl.bioontology.org/ontology/MESH/Q000473 http://purl.bioontology.org/ontology/MESH/Q000451 http://purl.bioontology.org/ontology/MESH/Q000209 |
| Inverse of SIB |
http://purl.bioontology.org/ontology/MESH/D001928 http://purl.bioontology.org/ontology/MESH/D009188 http://purl.bioontology.org/ontology/MESH/D004684 http://purl.bioontology.org/ontology/MESH/D001926 http://purl.bioontology.org/ontology/MESH/D004681 http://purl.bioontology.org/ontology/MESH/D002534 http://purl.bioontology.org/ontology/MESH/D001930 http://purl.bioontology.org/ontology/MESH/D020236 http://purl.bioontology.org/ontology/MESH/D009471 http://purl.bioontology.org/ontology/MESH/D001480 http://purl.bioontology.org/ontology/MESH/D001922 http://purl.bioontology.org/ontology/MESH/D001304 http://purl.bioontology.org/ontology/MESH/D019586 http://purl.bioontology.org/ontology/MESH/D000071072 http://purl.bioontology.org/ontology/MESH/D004673 http://purl.bioontology.org/ontology/MESH/D001932 http://purl.bioontology.org/ontology/MESH/D003704 http://purl.bioontology.org/ontology/MESH/D002526 http://purl.bioontology.org/ontology/MESH/D013353 http://purl.bioontology.org/ontology/MESH/D001925 http://purl.bioontology.org/ontology/MESH/D002561 http://purl.bioontology.org/ontology/MESH/D013786 http://purl.bioontology.org/ontology/MESH/D004827 http://purl.bioontology.org/ontology/MESH/D007027 http://purl.bioontology.org/ontology/MESH/D001929 http://purl.bioontology.org/ontology/MESH/D009103 http://purl.bioontology.org/ontology/MESH/D065166 http://purl.bioontology.org/ontology/MESH/D019150 http://purl.bioontology.org/ontology/MESH/D056784 http://purl.bioontology.org/ontology/MESH/D006849 http://purl.bioontology.org/ontology/MESH/D019585 http://purl.bioontology.org/ontology/MESH/D020773 http://purl.bioontology.org/ontology/MESH/D000405 http://purl.bioontology.org/ontology/MESH/D000550 |
| Machine permutation |
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 |
| Mapped from | |
| MDA |
19990101 |
| MMR |
20130708 |
| MN |
C10.228.140.695.562.112 C10.114.375.112 C10.228.140.400 C20.111.258.250.175 C10.314.350.112 |
| notation |
D002549 |
| prefLabel |
Diffuse Cerebral Sclerosis of Schilder |
| TERMUI |
T014245 T769477 T014248 T812087 T007434 T014251 T007432 T014252 T752574 T014249 T365199 T007433 T007431 T752575 T769476 T014247 T014250 T365192 T007435 T811449 T840857 T769475 |
| TH |
NLM (2011) NLM (1993) UNK (19XX) NLM (1990) NLM (2000) NLM (1966) NLM (2010) NLM (2014) ORD (2010) OMIM (2013) GHR (2014) |
| tui |
T047 |
| subClassOf |