Brain & development · 2026
Child neurology: Early neuroprotective and immunomodulatory intervention in acute shock with encephalopathy and multiorgan failure: Cytokine-storm encephalopathy-case report.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Tsuiki Nobuaki, Aihara Tsuyoshi, Hayakawa Itaru, Moriwaki Taro, Tomita Keiichi, Takahashi Tatsuya, Irie Saeko, Matsumoto Shotaro, Uematsu Satoko, Abe Yuichi
Аннотация
BACKGROUND: Acute shock with encephalopathy and multiorgan failure (ASEM)-within the infection-triggered encephalopathy syndromes (ITES)-is a fulminant para-infectious encephalopathy that can progress to diffuse cerebral edema within hours. Guidance on time-critical immunomodulation remains limited.
CASE: An 8-year-old girl developed abrupt altered mental status on day 2 of an influenza A respiratory illness. Glasgow Coma Scale score was E4V1M5 with hyperpyrexia, hypoxemia, and circulatory instability, prompting intubation. Head CT at 120 min showed early diffuse cerebral edema. At 180 min (transport-team arrival), systolic blood pressure was 60 mmHg; a neuroprotective bundle with vasoactive agents achieved partial stabilization. Methylprednisolone pulse therapy began at 200 min; therapeutic plasma exchange (TPE) at 430 min. Continuous EEG showed diffuse slowing in the 0.5-2 Hz band without suppression or electrographic seizures. Steroid pulses plus TPE were continued for five days, with adjunct intravenous immunoglobulin on days 5-6. Vasopressors were weaned by day 3; renal replacement therapy ended by day 5. Brain MRI on day 7 showed no new abnormalities. She was extubated on day 7; blood cultures remained negative. Neurologic status steadily improved. At 3.5 months, full-scale IQ was 111; at 6 months, only mild truncal ataxia persisted without functional limitation.
CONCLUSIONS: This ASEM case illustrates parallel diagnosis and treatment within a narrow window. Simple triggers-shock with multiorgan failure/disseminated intravascular coagulation and early imaging red flags-can support rapid transfer and early immunomodulation (e.g., steroid pulses and TPE) alongside neuroprotective care, potentially averting irreversible brain injury.
CASE: An 8-year-old girl developed abrupt altered mental status on day 2 of an influenza A respiratory illness. Glasgow Coma Scale score was E4V1M5 with hyperpyrexia, hypoxemia, and circulatory instability, prompting intubation. Head CT at 120 min showed early diffuse cerebral edema. At 180 min (transport-team arrival), systolic blood pressure was 60 mmHg; a neuroprotective bundle with vasoactive agents achieved partial stabilization. Methylprednisolone pulse therapy began at 200 min; therapeutic plasma exchange (TPE) at 430 min. Continuous EEG showed diffuse slowing in the 0.5-2 Hz band without suppression or electrographic seizures. Steroid pulses plus TPE were continued for five days, with adjunct intravenous immunoglobulin on days 5-6. Vasopressors were weaned by day 3; renal replacement therapy ended by day 5. Brain MRI on day 7 showed no new abnormalities. She was extubated on day 7; blood cultures remained negative. Neurologic status steadily improved. At 3.5 months, full-scale IQ was 111; at 6 months, only mild truncal ataxia persisted without functional limitation.
CONCLUSIONS: This ASEM case illustrates parallel diagnosis and treatment within a narrow window. Simple triggers-shock with multiorgan failure/disseminated intravascular coagulation and early imaging red flags-can support rapid transfer and early immunomodulation (e.g., steroid pulses and TPE) alongside neuroprotective care, potentially averting irreversible brain injury.