familial acute necrotizing encephalopathy
Findings
No curated finding names familial acute necrotizing encephalopathy yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Familial acute necrotizing encephalopathy or ADANE is a potentially fatal neurological disease characterized by neuropathological lesions principally involving the brainstem, thalamus and putamen.
Definition from the Mondo Disease Ontology (MONDO:0011953), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
HPO, annotations 2026-09-02
Features
25 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Acute encephalopathyHPOHP:0006846
- Very frequent (80% to 99% of cases)
- ComaHPOHP:0001259
- Very frequent (80% to 99% of cases)
- Increased CSF protein concentrationHPOHP:0002922
- Very frequent (80% to 99% of cases)
- Abnormal brainstem morphologyHPOHP:0002363
- Frequent (30% to 79% of cases)
- Abnormal brainstem MRI signal intensityHPOHP:0012747
- Frequent (30% to 79% of cases)
- Abnormal pattern of respirationHPOHP:0002793
- Frequent (30% to 79% of cases)
Show the remaining 13
- DysarthriaHPOHP:0001260
- Frequent (30% to 79% of cases)
- FeverHPOHP:0001945
- Frequent (30% to 79% of cases)
- Gait disturbanceHPOHP:0001288
- Frequent (30% to 79% of cases)
- Generalized muscle weaknessHPOHP:0003324
- Frequent (30% to 79% of cases)
- GliosisHPOHP:0002171
- Frequent (30% to 79% of cases)
- HypertoniaHPOHP:0001276
- Frequent (30% to 79% of cases)
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- RANBP2HGNC:9848
- Definitive · G2P · Autosomal dominant · 2026
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2023
- Strong · PanelApp Australia · Autosomal dominant · 2025
- Moderate · ClinGen · Autosomal dominant · 2024
Where it sits
Other names
4 names
Resolves to: familial acute necrotizing encephalopathy
- Also called
- ADANEencephalopathy, acute, infection-induced, 3, susceptibility toencephalopathy, acute, infection-induced, susceptibility to, type 3recurrent acute necrotizing encephalopathy