familial acute necrotizing encephalopathy

ORPHA: 88619

Contents

1. Overview
2. Available Treatments
3. Clinical Presentation
4. Classification & Codes

Overview

Familial acute necrotizing encephalopathy or ADANE is a potentially fatal neurological disease characterised by neuropathological lesions principally involving the brainstem, thalamus and putamen

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with familial acute necrotizing encephalopathy, sourced from HPO and Orphanet clinical annotations.

ComaIncreased CSF protein concentrationAcute encephalopathyIntellectual disabilitySeizureSpasticityDysarthriaHypertoniaGait disturbanceFeverVomitingRigidityGliosisCerebral edemaAbnormal brainstem morphologyDevelopmental regressionSpastic tetraplegiaAbnormal pattern of respirationGeneralized muscle weaknessAbnormal thalamus morphologyChoroid hemorrhageAbnormal brainstem MRI signal intensityAbnormal visual fixationAbnormal putamen morphology

Classification & Codes

Orphanet Code

ORPHA:88619

familial acute necrotizing encephalopathy

Orphanet	`ORPHA:88619`
Treatments	0 drug(s)
Symptoms on record	24 signs
Status	published

Factual Authority

Last Updated3/20/2026

Clinical DataHPO · Orphanet

Drug DataFDA · EMA · CDSCO