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Case No. 754
2026-06-01~2026-06-30
출제자 : 이경미
Hit : 399
| Period | 2026-06-01~2026-06-30 |
|---|---|
| Diagnosis | Acute Necrotizing Encephalopathy |
| Clinical information | Chief Complaint> Prolonged febrile seizure, drowsy mentality Present Illness> exanthema subitum Hx(+) Previous Medical History> Admission Hx: 2025-07 bronchiolitis, 2025-09 pneumonia, 2025-11 adenovirus infection Vaccination: done as scheduled |
| Discussion | Rare encephalopathy predominantly affecting infants and children More commonly reported in Japan and Taiwan in the Far East ++Pathogenesis: unknown Key hypothesis: viral infection-associated cytokine storm Influenza, rotavirus, human herpesvirus–6, COVID-19 Vascular endothelial damage; BBB breakdown without direct viral invasion ++Clinical manifestation Viral prodromal symptoms: may have signs of shock, multiorgan failure, DIC Acute encephalopathy: nonspecific symptoms (Seizure, altered mental statis) Recovery: usually with neurological sequelae Diverse course: from complete recovery to death ++Distribution :symmetrical, multifocal lesions Thalamus (most frequent), upper brain stem tegmentum, cerebral white matter, medulla ++Pathology: edema, petechial hemorrhage, tissue necrosis Hemorrhage occurs predominantly in the central portion of the involved deep gray matter, but not in the cerebral white matter ++ADC map Concentric "tricolor pattern" (inner/outer facilitated diffusion, middle restricted) From inner to outer: perivascular hemorrhage/necrosis --> oligodendrocyte swelling (cytotoxic edema) --> extravasations (vasogenic edema) ++Differential Diagnosis --Leigh syndrome (Subacute necrotizing encephalopathy): mitochondrial disease ,Bilateral, symmetric T2 high SI lesios in BG, Dorsomedial thalami can also be involved, Often involves pons/medulla/cerebellum Lab: lactic acidosis -- ADEM: bilateral but asymmetric, mainly involves white matter, basal ganglia, posterior fossa area --Thrombosis of internal and great cerebral vein |
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