Acute disseminated encephalomyelitis (ADEM) is an autoimmune demyelinating syndrome of the central nervous system (CNS) usually following a febrile illness. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is predominantly a respiratory infection with rare direct neurologic involvement. Although neurological symptoms are increasingly reported, it is unclear if these represent a para-infectious or post-infectious manifestation of SARS-CoV-2. We report the first case to our knowledge of pediatric ADEM associated with SARS-CoV-2.
We present a case of a 5 year-old boy with ADEM in the setting of SARS-CoV with histopathology showing meningoencephalitis.
Case report featuring clinical presentation, laboratory, neuroimaging, and histopathology results, and medical decision-making, treatment, and clinical course.
A 5-year-old previously healthy Hispanic boy presented with 3 weeks of headache, right eye blurry vision, and vomiting. Infectious work-up was negative except for SARS-CoV-2 RNA PCR. MRI brain demonstrated multifocal lesions with intense enhancement and diffusion restriction, most prominent in the right frontal lobe, basal ganglia, and cerebellum with vasogenic edema and punctate foci of hemorrhage as well as bilateral swelling of optic nerves and optic chiasm. MRI spine revealed longitudinally extensive myelitis with an intensely enhancing, expansile intramedullary lesion from T1 to T3.
Differential diagnosis included infectious and inflammatory etiologies leading to a brain biopsy which showed foci of lymphohistiocytic perivascular inflammation (predominantly CD3+ T-cells and CD68+ histiocytes, with rare CD20+ B-cells) consistent with meningoencephalitis. There were features suggestive of, but not definitive for demyelination, including occasional macrophages with staining indicating ingestion of myelin breakdown products. No definite viral inclusions were identified by light or electron microscopy.
Neurology was consulted, noting that he was very irritable with decreased upper extremity movements. Based on his clinical syndrome and imaging, ADEM was suspected and started on high dose IV methylprednisolone. Cerebrospinal fluid showed 9 nucleated cells/mm3 (97% lymphocytes), 44 mg/dL protein, 77 mg/dL glucose, enterovirus and herpes simplex virus PCR negative. Due to brain, optic nerve, and spinal cord involvement, MOG related ADEM is suspected (test pending) and PLEX was also initiated.
We report the first case to our knowledge of ADEM in a child in the setting of SARS-CoV2 with histopathology showing meningoencephalitis. Based on his brain, optic nerve, and spinal cord involvement, MOG antibody disease is suspected, presumably triggered by SARS-CoV2 infection rather than resulting from SARS-CoV2 infection itself. Additional studies are needed to further characterize the direct and indirect neurological sequelae of SARS-CoV2.