FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
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General Information
Term Aicardi-Goutieres syndrome ID (Ontology) DOID:0050629 (Human Disease)
Definition A syndrome that is a genetically heterogeneous encephalopathy characterized in its most severe form by cerebral atrophy, leukodystrophy, intracranial calcifications, chronic cerebrospinal fluid lymphocytosis, increased CSF alpha-interferon, and negative serologic investigations for common prenatal infections.
Also Known As "AGS" ; "Cree encephalitis"
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 Genes
 Aicardi-Goutieres syndrome       7
 for disease ribbon | Aicardi-Goutieres syndrome       7
 model of | Aicardi-Goutieres syndrome       7
Spanning Tree (Parents/Children)
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monogenic disease
 |__autosomal genetic disease__
disease                        |
 |__syndrome___________________|
                               Aicardi-Goutieres syndrome  7 rec.
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Is a autosomal genetic disease
syndrome
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Synonyms
  • "AGS" EXACT OMO:0003012
    "Cree encephalitis" EXACT
Secondary IDs
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GARD:575
ICD10CM:G31.8
MIM:225750
MIM:610181
MIM:610329
MIM:610333
MIM:612952
MIM:615010
MIM:615846
MIM:PS225750
ORDO:51