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Hemicraniectomy and externalized ventricular drain placement in a pediatric patient with myelin oligodendrocyte glycoprotein-associated tumefactive demyelinating disease

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Abstract

Background

Acquired demyelination of the central nervous system in children can manifest as multiple sclerosis, neuromyelitis optica, myelin oligodendrocyte glycoprotein (MOG)-associated demyelination, or as an acute monophasic illness without serum antibodies. Rarely do patients with demyelinating disease need surgical intervention for fulminant crises.

Case

We report a case of anti-MOG antibody-related tumefactive demyelination in a 10-year-old female who required urgent hemicraniectomy and external ventricular drain placement for progressive white matter edema with obstructive hydrocephalus, subfalcine, and transtentorial herniation.

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Authors and Affiliations

  1. Division of Neurosurgery, Children’s Hospital of Philadelphia, Department of Neurosurgery, University of Pennsylvania, Perelman School of Medicine, 3401 Civic Center Blvd, 6 Wood Center, Philadelphia, PA, 19104, USA

    Saurabh Sinha, Alexander Tucker, Phillip B. Storm & Shih-Shan Lang

  2. Division of Neurology, Children’s Hospital of Philadelphia, Department of Neurology, University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, USA

    Brenda Banwell

  3. Department of Anesthesiology and Critical Care Medicine, Children’s Hospital of Philadelphia, University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, USA

    Jimmy Huh

Authors
  1. Saurabh Sinha
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  2. Brenda Banwell
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  3. Alexander Tucker
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  4. Phillip B. Storm
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  5. Jimmy Huh
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  6. Shih-Shan Lang
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Correspondence to Shih-Shan Lang.

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Supplementary information

Supplementary Figure 1

A) Low magnification (10X) and B) high magnification (100X) shows loss of myelin around blood vessels consistent with demyelinating process. Luxol fast blue and cresyl violet stain. (JPG 609 kb)

Supplementary Figure 2

A) Low magnification (10X) and B) high magnification (200X) shows an abundance of CD 68(+)macrophages in the areas of demyelination and adjacent white matter. (JPG 453 kb)

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Sinha, S., Banwell, B., Tucker, A. et al. Hemicraniectomy and externalized ventricular drain placement in a pediatric patient with myelin oligodendrocyte glycoprotein-associated tumefactive demyelinating disease. Childs Nerv Syst 38, 185–189 (2022). https://doi.org/10.1007/s00381-021-05139-2

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  • DOI: https://doi.org/10.1007/s00381-021-05139-2

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