Key result
Acute viral myocarditis presents as a fatal triad with ADEM and refractory seizures.
Why the study?
The phenotype of acute disseminated encephalomyelitis (ADEM) is generally restricted to the brain, and expansion of its clinical spectrum is needed.
Case Report (n=1)
This case expands the phenotype of ADEM to include life-threatening acute lymphocytic myocarditis and cardiogenic shock as part of a postinfectious systemic autoimmune response.
Alerts clinicians to rare fatal post-viral triad in children; leaves open mechanisms linking ADEM, seizures, and myocarditis.
Autoimmune pathology of acute disseminated encephalomyelitis (ADEM) is generally restricted to the brain. Our objective is to expand the phenotype of ADEM. A four-year-old girl was admitted to the pediatric emergency room of a university medical center five days after a common upper respiratory tract infection. Acute symptoms were fever, leg pain, and headaches. She developed meningeal signs, and her level of consciousness dropped rapidly. Epileptic seizure activity started, and she became comatose, requiring intubation and mechanical ventilation. Serial brain magnetic resonance imaging (MRI) illustrated the fulminant development of ADEM. Treatment escalation with high-dose corticosteroids, immunoglobulins, and plasma exchange did not lead to clinical improvement. On day ten, the patient developed treatment-refractory cardiogenic shock and passed away. The postmortem assessment confirmed ADEM and revealed acute lymphocytic myocarditis, likely explaining the acute cardiac failure. Human metapneumovirus and picornavirus were detected in the tracheal secrete by PCR. Data sources-medical chart of the patient. This case is consistent with evidence from experimental findings of an association of ADEM with myocarditis as a postinfectious systemic autoimmune response, with life-threatening involvement of the brain and heart.
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Lademann et al. (2020) conducted a case report in Acute Disseminated Encephalomyelitis (ADEM) with Seizures and Myocarditis (n=1). High-dose corticosteroids, immunoglobulins, and plasma exchange was evaluated on Clinical outcome. A 4-year-old girl developed a fatal triad of acute disseminated encephalomyelitis, seizures, and myocarditis following an upper respiratory tract infection, with postmortem evidence of viral myocarditis.
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