A 7-year-old boy dying of acute encephalopathy.
A 7 year old Chinese boy died of a rapidly progressive encephalopathy after influenza infection. MRI showed bilateral and symmetrical lesions including the thalamus and brainstem tegmentum. The pathology of necrosis and vasculopathy were in keeping with acute necrotizing encephalopathy (ANE). ANE was first described in Japan and carries a high mortality and morbidity. A vasculopathy with breakdown of the blood-brain-barrier was incriminated but the pathogenesis remained obscure and autopsy studies have been limited. A review of the literature showed only nine postmortem reports in the acute stage. Symmetrical brain necrosis always involved the thalamus followed by the tegmentum of the pons and other regions. Exudative vasculopathy was commonly observed and often accompanied by endothelial cell necrosis. In the present case there was inflammatory fibrinoid vasculitis which has not been previously described.
Ng
Wai-Fu
WF
Chiu
Sin-Chuen
SC
Lam
David S-Y
DS
Wong
Yiu-Chung
YC
Tam
Sidney
S
Kwong
Ngai-Shan
NS
Loo
Ka-Tai
KT
Yuen
Kwok-Yung
KY
D001249:Asthma / Q000150:complications; D001933:Brain Stem / Q000473:pathology; D002531:Cerebellum / Q000473:pathology; D002648:Child; D017809:Fatal Outcome; D015600:Glasgow Coma Scale; D006801:Humans; D053118:Influenza A Virus, H1N1 Subtype; D007251:Influenza, Human / Q000150:complications / Q000473:pathology*; D004684:Leukoencephalitis, Acute Hemorrhagic / Q000209:etiology / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D013788:Thalamus / Q000473:pathology