anec
Table of Contents
acute necrotising encephalopathy of childhood (ANEC)
see also:
Introduction
- a rare type of auto-immune acute encephalopathy mainly affecting children but can occur in adults
- first case described in 1995
- primarily tends to involve
Epidemiology
Aetiology
- mutations in the RANBP2 gene may be involved in familial or recurrent cases
- viral infections appear to be the trigger in most cases
- enterovirus
Clinical features
- often have a viral prodrome
- usually acute onset of encephalitis-like features with neurologic deficits or seizures
Diagnosis
- CT brain:
- hypodense lesions of infected parts
- may have intracranial haemorrhage or cavitation
- MRI brain:
- most have bilateral symmetrical thalamic involvement
- see https://radiopaedia.org/articles/acute-necrotising-encephalopathy for more features
DDx
Rx
- 70% mortality rate
- supportive care
anec.txt · Last modified: 2026/09/02 05:50 by wh