Author Of 2 Presentations
LB1225 - Acute disseminated encephalomyelitis in a pediatric patient in the setting of SARS-CoV2 with histopathology (ID 2104)
Abstract
Background
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.
Objectives
We present a case of a 5 year-old boy with ADEM in the setting of SARS-CoV with histopathology showing meningoencephalitis.
Methods
Case report featuring clinical presentation, laboratory, neuroimaging, and histopathology results, and medical decision-making, treatment, and clinical course.
Results
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.
Conclusions
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.
LB1238 - Myelin Oligodendrocyte Glycoprotein-antibody positive transverse myelitis presenting as acute flaccid paralysis in a child with enterovirus infection (ID 2123)
Abstract
Background
Acute transverse myelitis is an immune-mediated disorder targeting the spinal cord and manifests with weakness, sensory deficits, and loss of bowel and bladder control. It can occur as a part of a serologically-defined neuroimmunological syndrome or as monophasic post-infectious illness. While these conditions generally respond well to immunotherapies, direct infections of the spinal cord (e.g. enterovirus related acute flaccid myelitis [AFM]) fare poorly and theoretically could be negatively impacted by immunosuppressive treatments. The clinical and radiological overlap in these conditions poses challenges to acute management of a child presenting with acute flaccid limb weakness.
Objectives
We report a child who presented with subacute onset of flaccid paraplegia, anesthesia, and urinary retention with positive nasopharyngeal swab for enterovirus/rhinovirus (EV/RV), who was found to be positive for MOG antibodies.
Methods
Case report detailing the clinical presentation, laboratory findings, neuroimaging results, clinical decision-making, treatment, and hospitalization course.
Results
A 4-year-old previously healthy Hispanic boy presented with back pain and lower extremity weakness and numbness in July 2020. The symptoms evolved over several hours the previous evening and were preceded by an upper respiratory infection 2 weeks prior. His exam was notable for flaccid paralysis, areflexia, and anesthesia of his lower extremities and urinary retention.
Pertinent laboratory findings included nasopharyngeal swab detection of EV/RV, CSF with 222 WBCs/mm3, glucose 59 mg/dL, protein 171 mg/dL, elevated IgG index of 0.87, and negative CSF enterovirus PCR. Spine MRI demonstrated a longitudinally extensive transverse myelitis largely restricted to the gray matter. Brain MRI showed T2 hyperintense lesion in the right pons and left occipital white matter.
Given his profound leg weakness and potential in benefitting from empirically treating an immune-mediated myelitis, he received IV methylprednisolone and plasma exchange. Serum MOG autoantibody later returned positive (1:100). He regained volitional bladder function and had antigravity lower extremity movements following these treatments.
Conclusions
The case highlights the diagnostic challenges of a child presenting with acute flaccid weakness of unclear etiology. Our patient had demographic (age, seasonality), exam (flaccid, areflexic limbs), and laboratory features (enterovirus/rhinovirus positive on nasal swab) which combined were highly suggestive AFM. However, the presence of a supratentorial brain lesion and concern for an immune-mediated myelitis led to administration of immunotherapies prior to knowledge of his serological results. More efforts are needed to better define the unique characteristics associated with these syndromes to provide diagnostic and treatment guidelines.
Presenter Of 1 Presentation
LB1225 - Acute disseminated encephalomyelitis in a pediatric patient in the setting of SARS-CoV2 with histopathology (ID 2104)
Abstract
Background
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.
Objectives
We present a case of a 5 year-old boy with ADEM in the setting of SARS-CoV with histopathology showing meningoencephalitis.
Methods
Case report featuring clinical presentation, laboratory, neuroimaging, and histopathology results, and medical decision-making, treatment, and clinical course.
Results
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.
Conclusions
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.