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  • Child Neurology: Remarkable Recovery From Severe Acute Necrotizing Encephalopathy. Neurology Silverman, A., Sasaki, M., Espindola Lima, J. E., Cheronis, C., Lin, G. L., Johnson, A., Dahmoush, H., Archer, E., Grekov, K., LaRocca, T. J., Van Haren, K. 2024; 103 (8): e209877

    Abstract

    A previously healthy 6-year-old girl presented with several days of fever before a generalized seizure. Laboratory investigation revealed elevated liver enzymes, normal ammonia, and positive influenza A through respiratory PCR. Brain MRI demonstrated extensive, bilateral lesions in the cerebral and cerebellar white matter, thalami, basal ganglia, and brainstem. She was diagnosed with acute necrotizing encephalopathy, a rare parainfectious encephalitis commonly associated with influenza. Genetic variants have been implicated (e.g., RANBP2 and RNH1), but our patient's rapid genome was nondiagnostic. Her 1-month hospitalization was complicated by prolonged encephalopathy and intracranial pressure crises requiring hyperosmolar therapy, sedation, intermittent paralysis, and hypothermia. Concomitantly, she received pulse corticosteroids, plasmapheresis, and oseltamivir. Three months after illness onset, she achieved a remarkable recovery with a normal neurologic examination. Although prognosis may comprise considerable morbidity and mortality, prompt recognition, immunotherapy, and intensive care can achieve positive neurodevelopmental outcomes. Our discussion concludes with a focus on the intrinsic uncertainties of neuroprognostication in the pediatric intensive care unit.

    View details for DOI 10.1212/WNL.0000000000209877

    View details for PubMedID 39298704

  • Harnessing IgG Fc glycosylation for clinical benefit. Current opinion in immunology Archer, E. J., Gonzalez, J. C., Ghosh, D., Mellins, E. D., Wang, T. T. 2022; 77: 102231

    Abstract

    The effector activity of IgG antibodies is regulated at several levels, including IgG subclass, modifications of the Fc glycan, and the distribution of Type I and II Fcgamma receptors (FcgammaR) on effector cells. Here, we explore how Fc glycosylation, particularly sialylation and fucosylation, tunes cellular responses to immune complexes. We review the current understanding of the pathways and mechanisms underlying this biology, address FcgammaR in antigen presentation, and discuss aspects of the clinical understanding of Fc glycans in therapies and disease.

    View details for DOI 10.1016/j.coi.2022.102231

    View details for PubMedID 35797920