loading...| Preferred Name |
Diffuse Cerebral Sclerosis of Schilder [Disease/Finding] |
|
| Synonyms |
Poliodystrophia Cerebri |
|
| ID |
http://purl.bioontology.org/ontology/NDFRT/N0000000726 |
|
| altLabel |
Poliodystrophia Cerebri Encephalitis Periaxialis Diffusa Schilder's Disease Sudanophilic Cerebral Sclerosis Cerebral Sclerosis, Diffuse Encephalitis Periaxialis Diffuse Cerebral Sclerosis of Schilder Schilder Disease Encephalitis Periaxialis Concentrica Myelinoclastic Diffuse Sclerosis |
|
| cui |
C0007795 |
|
| 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) |
|
| MESH DUI |
D002549 |
|
| MeSH name |
Diffuse Cerebral Sclerosis of Schilder |
|
| MESH UI |
M0003893 |
|
| NDFRT kind |
DISEASE_KIND |
|
| notation |
N0000000726 |
|
| NUI |
N0000000726 |
|
| prefLabel |
Diffuse Cerebral Sclerosis of Schilder [Disease/Finding] |
|
| SNOMED CID |
49692006 |
|
| tui |
T047 |
|
| subClassOf |