diffuse cerebral sclerosis of schilder

Preferred Label : diffuse cerebral sclerosis of schilder;

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 synonym : myelinoclastic diffuse sclerosis; schilder disease; diffuse sclerosis, myelinoclastic; myelinoclastic diffuse scleroses; disease, schilder's; schilders disease; sclerosis, diffuse cerebral; sclerosis, myelinoclastic diffuse; diffuse cerebral sclerosis; encephalitis periaxialis; encephalitis periaxialis concentrica; cerebral sclerosis, diffuse; diffuse cerebral scleroses; encephalitis periaxialis diffusa; poliodystrophia cerebri; disease, schilder; schilder's disease; scleroses, myelinoclastic diffuse; diffuse scleroses, myelinoclastic; sudanophilic cerebral sclerosis;

Related MeSH term : balo concentric sclerosis; alpers syndrome; Concentric Sclerosis, Balo; Balo's Concentric Sclerosis; Concentric Sclerosis, Balo's; Scleroses, Balo's Concentric; Syndrome, Alpers; Alpers Disease; Alpers Progressive Infantile Poliodystrophy; Progressive Sclerosing Poliodystrophy; Progressive Sclerosing Poliodystrophies; Alpers' Syndrome; Alper Syndrome; Alper's Syndrome; Alpers-Huttenlocher Syndrome; Alpers Huttenlocher Syndrome; Syndrome, Alpers-Huttenlocher; Neuronal Degeneration Of Childhood With Liver Disease, Progressive; Progressive Neuronal Degeneration of Childhood with Liver Disease; Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis; Alpers' Disease; Alper Disease; Alper's Disease; Disease, Alpers';

Wikipedia link : https://en.wikipedia.org/wiki/Alper's disease;

Details

Origin ID

D002549;

UMLS CUI

C0007795;

Allowable qualifiers
- blood [MeSH Qualifier]
- cerebrospinal fluid [MeSH Qualifier]
- chemically induced [MeSH Qualifier]
- classification [MeSH Qualifier]
- complications [MeSH Qualifier]
- congenital [MeSH Qualifier]
- diagnosis [MeSH Qualifier]
- diagnostic imaging [MeSH Qualifier]
- diet therapy [MeSH Qualifier]
- drug therapy [MeSH Qualifier]
- economics [MeSH Qualifier]
- embryology [MeSH Qualifier]
- enzymology [MeSH Qualifier]
- epidemiology [MeSH Qualifier]
- ethnology [MeSH Qualifier]
- etiology [MeSH Qualifier]
- genetics [MeSH Qualifier]
- history [MeSH Qualifier]
- immunology [MeSH Qualifier]
- metabolism [MeSH Qualifier]
- microbiology [MeSH Qualifier]
- mortality [MeSH Qualifier]
- nursing [MeSH Qualifier]
- parasitology [MeSH Qualifier]
- pathology [MeSH Qualifier]
- physiopathology [MeSH Qualifier]
- prevention and control [MeSH Qualifier]
- psychology [MeSH Qualifier]
- radiotherapy [MeSH Qualifier]
- rehabilitation [MeSH Qualifier]
- surgery [MeSH Qualifier]
- therapy [MeSH Qualifier]
- urine [MeSH Qualifier]
- veterinary [MeSH Qualifier]
- virology [MeSH Qualifier]
Automatic exact mappings (from CISMeF team)
- Adrenoleukodystrophy [NCIt concept]
- Adrenoleukodystrophy (disorder) [SNOMED CT concept]
- Adult neuronal ceroid lipofuscinosis (disorder) [SNOMED CT concept]
- Argentinian hemorrhagic fever (disorder) [SNOMED CT concept]
- Atherosclerosis susceptibility [OMIM Phenotype]
- Autoimmune lymphoproliferative syndrome [ORDO Disease]
- Autoimmune lymphoproliferative syndrome [OMIM Phenotype]
- Basedow's disease [MedDRA Preferred Term]
- Bauxite fibrosis of lung (disorder) [SNOMED CT concept]
- Borries' syndrome (disorder) [SNOMED CT concept]
- Brass-founders' fever (disorder) [SNOMED CT concept]
- Bruns' syndrome (disorder) [SNOMED CT concept]
- Capgras' syndrome [MedDRA LLT]
- Capgras' syndrome (finding) [SNOMED CT concept]
- Carotid artery insufficiency syndrome (disorder) [SNOMED CT concept]
- Chagas' disease [MedDRA LLT]
- Coats' disease [MedDRA LLT]
- Coats' disease (disorder) [SNOMED CT concept]
- Corbus' disease [MedDRA LLT]
- Cotunnius' disease [MedDRA LLT]
- Crush syndrome (disorder) [SNOMED CT concept]
- Diffuse cerebral sclerosis [HPO term]
- Disease [PASCAL descriptor]
- Disease (disorder) [SNOMED CT concept]
- Disease or Disorder [NCIt concept]
- Disease related state (finding) [SNOMED CT concept]
- Disorder of face (disorder) [SNOMED CT concept]
- Eales' disease [MedDRA Preferred Term]
- Eales' disease (disorder) [SNOMED CT concept]
- Edwards' syndrome [MedDRA LLT]
- Eosinophilic cellulitis (disorder) [SNOMED CT concept]
- Erythema infectiosum [MedDRA Preferred Term]
- Exudative retinopathy [MedDRA Preferred Term]
- Exudative retinopathy (disorder) [SNOMED CT concept]
- Flax-dressers' disease (disorder) [SNOMED CT concept]
- Forbes' disease [MedDRA LLT]
- Fuchs' syndrome [MedDRA Preferred Term]
- GRAVES' DISEASE [WHO-ART Included Term]
- General health [LOINC component]
- Glycogen storage disease type III [MedDRA Preferred Term]
- Glycogen storage disease, type III (disorder) [SNOMED CT concept]
- Graves Disease [NCIt concept]
- Graves' disease [MedDRA LLT]
- Graves' disease (disorder) [SNOMED CT concept]
- Gross' disease (disorder) [SNOMED CT concept]
- Infection caused by Trypanosoma cruzi (disorder) [SNOMED CT concept]
- Inhalational anthrax (disorder) [SNOMED CT concept]
- Jervis' syndrome (disorder) [SNOMED CT concept]
- Juvenile osteochondrosis of head of humerus (disorder) [SNOMED CT concept]
- Juvenile osteochondrosis of lower ulna (disorder) [SNOMED CT concept]
- Kuess' disease (disorder) [SNOMED CT concept]
- Legionnaires disease [ORDO Disease]
- Lichen nitidus (disorder) [SNOMED CT concept]
- Malignant atrophic papulosis (disorder) [SNOMED CT concept]
- Marginal periodontitis (disorder) [SNOMED CT concept]
- Meigs Syndrome [NCIt concept]
- Meigs' syndrome [MedDRA Preferred Term]
- Meigs' syndrome (disorder) [SNOMED CT concept]
- Menkes' syndrome [MedDRA Preferred Term]
- Milles' syndrome [MedDRA LLT]
- Moebius II syndrome [MedDRA Preferred Term]
- Moyamoya disease (disorder) [SNOMED CT concept]
- Myelinoclastic diffuse sclerosis [ICD-11 More detail]
- Olivopontocerebellar degeneration (disorder) [SNOMED CT concept]
- Oromandibular-limb hypogenesis spectrum (disorder) [SNOMED CT concept]
- Panhypopituitarism (disorder) [SNOMED CT concept]
- Partial lipodystrophy [MedDRA Preferred Term]
- Peripartum cardiomyopathy [ORDO Disease]
- Perthes' disease [MedDRA LLT]
- Poliodystrophia cerebri [MedDRA LLT]
- Polypoid sinus degeneration (disorder) [SNOMED CT concept]
- Primary progressive cerebellar degeneration (disorder) [SNOMED CT concept]
- Simons' disease [MedDRA LLT]
- Sotos' syndrome [MedDRA Preferred Term]
- Sotos' syndrome (disorder) [SNOMED CT concept]
- Spens' syndrome [MedDRA LLT]
- Stokvis' disease (disorder) [SNOMED CT concept]
- Sturge-Weber syndrome [MedDRA Preferred Term]
- Subcorneal pustular dermatosis (disorder) [SNOMED CT concept]
- Syndrome [NCIt concept]
- Trisomy 18 [MedDRA Preferred Term]
- Tularemia (disorder) [SNOMED CT concept]
- Von Hippel-Lindau syndrome (disorder) [SNOMED CT concept]
- adrenoleukodystrophy [DO Concept]
- borries' syndrome [SNOMED Notion]
- bruns' syndrome [SNOMED Notion]
- capgras' syndrome [SNOMED Notion]
- disease [ORDO Disease]
- disease [OBI concept]
- disease, nos [SNOMED Notion]
- down syndrome [MedlinePlus Topic]
- eales' disease [SNOMED Notion]
- fifth disease [MedlinePlus Topic]
- four x syndrome [SNOMED Notion]
- gross' disease [SNOMED Notion]
- jervis' syndrome [SNOMED Notion]
- kuess' disease [SNOMED Notion]
- legionnaires' disease [MedlinePlus Topic]
- meigs' syndrome [SNOMED Notion]
- no disease [ICPC-2 Rubric]
- sotos' syndrome [SNOMED Notion]
- stokvis' disease [SNOMED Notion]
- thyroid diseases [MedlinePlus Topic]
Currated CISMeF NLP mapping
- Alpers disease [PASCAL descriptor]
- Alpers disease [MedDRA Preferred Term]
- Alpers syndrome [DO Concept]
- Alpers' disease [MedDRA LLT]
- Alpers-Huttenlocher syndrome [ICD-11 More detail]
- Alpers-Huttenlocher syndrome [DO Concept]
- Alpers-Huttenlocher syndrome [ORDO Disease]
- Balo Concentric Sclerosis [NCIt concept]
- Balo concentric sclerosis [DO Concept]
- Balo concentric sclerosis (disorder) [SNOMED CT concept]
- Balo's concentric sclerosis [MedDRA LLT]
- Baló concentric sclerosis [ORDO Disease]
- Concentric sclerosis [ICD-11 More detail]
- Concentric sclerosis [Balo] of central nervous system [ICD-10 Sub-category]
- Concentric sclerosis [Baló] [ICD-10 Sub-category (who)]
- Diffuse Cerebral Sclerosis of Schilder [NCIt concept]
- Diffuse sclerosis [ICD-10 Sub-category (who)]
- Diffuse sclerosis of central nervous system [ICD-10 Sub-category]
- Encephalitis periaxialis diffusa [MedDRA Preferred Term]
- Progressive sclerosing poliodystrophy (disorder) [SNOMED CT concept]
- Schilder disease [PASCAL descriptor]
- Schilder disease [ORDO Disease]
- Schilder's disease [ICD-9 code]
- Schilder's disease [MedDRA LLT]
- Schilder's disease (disorder) [SNOMED CT concept]
- Sudanophilic cerebral sclerosis [OMIM Phenotype]
- alpers' disease [MedDRA Preferred Term]
- balo's concentric sclerosis [SNOMED Notion]
- diffuse cerebral sclerosis of Schilder [Disease (Nov.)]
- progressive sclerosing poliodystrophy [SNOMED Notion]
- schilder's disease [SNOMED Notion]
DO Cross reference
- Alpers-Huttenlocher syndrome [DO Concept]
- Balo concentric sclerosis [DO Concept]
HPO term
- Diffuse cerebral sclerosis [HPO term]
Manual NTBT mappings (CISMeF)
- Brain inflammatory disease [ORDO Disease]
- Genetic neurodegenerative disease [ORDO Disease]
- Neurometabolic disease [ORDO Disease]
- Rare neurodegenerative disease [ORDO Disease]
ORDO relation(s)
- Alpers-Huttenlocher syndrome [ORDO Disease]
Record concept(s)
- alpers syndrome [MeSH concept]
- balo concentric sclerosis [MeSH concept]
- diffuse cerebral sclerosis of schilder [MeSH concept]
Related MeSH Supplementary Concept(s)
- Pelizaeus-Merzbacher like brain sclerosis [MeSH Supplementary Concept]
- Pelizaeus-Merzbacher-like disease, autosomal recessive, 2 [MeSH Supplementary Concept]
See also inter- (CISMeF)
- Mitochondrial dna depletion syndrome 4a (alpers type) [OMIM Phenotype]
- progressive sclerosing poliodystrophy [Disease (Nov.)]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- Balo concentric sclerosis [DO Concept]
- Diffuse Cerebral Sclerosis of Schilder [NCIt concept]
- Diffuse cerebral sclerosis [HPO term]
- Diffuse sclerosis [ICD-10 Sub-category (who)]
- Encephalitis periaxialis diffusa [MedDRA Preferred Term]
- Poliodystrophia cerebri [MedDRA LLT]
- Schilder disease [ORDO Disease]
- Schilder disease [PASCAL descriptor]
- Schilder's disease [MedDRA LLT]
- Schilder's disease [ICD-9 code]
- Schilder's disease (disorder) [SNOMED CT concept]
- Sudanophilic cerebral sclerosis [OMIM Phenotype]
- diffuse cerebral sclerosis of Schilder [Disease (Nov.)]
- schilder's disease [SNOMED Notion]

Keyword's position in hierarchy(ies) :

Main resources

You can consult :

recommendations
documents concerning education
documents for patients