acute necrotising encephalopathy of childhood (ANEC)

see also:

• encephalopathy
• progressive multifocal leukoencephalopathy (PML)
• encephalitis
• leukoencephalopathy (ADEM)
• autoimmune glial fibrillary acid protein astrocytopathy / GFAP astrocytopathy
• Subacute sclerosing panencephalitis (SSPE)

• 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

• mutations in the RANBP2 gene may be involved in familial or recurrent cases
• viral infections appear to be the trigger in most cases
  • influenza
  • parainfluenza
  • varicella-zoster virus (chickenpox/shingles)
  • enterovirus
  • COVID-19 coronavirus (2019-nCoV / SARS-CoV-2)

• often have a viral prodrome
• usually acute onset of encephalitis-like features with neurologic deficits or seizures

• 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

• encephalopathy
• encephalitis
• leukoencephalopathy (ADEM)
• autoimmune glial fibrillary acid protein astrocytopathy / GFAP astrocytopathy

• 70% mortality rate
• supportive care