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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Armangue T, Olive-Cirera G, Martinez-Hernandez E, Sepulveda M, Ruiz-Garcia R, Munoz-Batista M, Arino H, Gonzalez-Alvarez V, Felipe-Rucian A, Jesus Martinez-Gonzalez M, Cantarin-Extremera V, Concepcion Miranda-Herrero M, Monge-Galindo L, Tomas-Vila M, Miravet E, Malaga I, Arrambide G, Auger C, Tintore M, Montalban X, Vanderver A, Graus F, Saiz A, Dalmau J, Spanish Pediatric anti MOGSG (2020) Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study. Lancet Neurol 19:234–246. https://doi.org/10.1016/S1474-4422(19)30488-0
Waters P, Fadda G, Woodhall M, O'Mahony J, Brown RA, Castro DA, Longoni G, Irani SR, Sun B, Yeh EA, Marrie RA, Arnold DL, Banwell B, Bar-Or A, Canadian pediatric demyelinating disease N (2019) serial anti-myelin oligodendrocyte glycoprotein antibody analyses and outcomes in children with demyelinating syndromes. JAMA Neurol. https://doi.org/10.1001/jamaneurol.2019.2940
Hacohen Y, Absoud M, Deiva K, Hemingway C, Nytrova P, Woodhall M, Palace J, Wassmer E, Tardieu M, Vincent A, Lim M, Waters P (2015) Myelin oligodendrocyte glycoprotein antibodies are associated with a non-MS course in children. Neurol Neuroimmunol Neuroinflamm 2:e81. https://doi.org/10.1212/NXI.0000000000000081
Hochmeister S, Gattringer T, Asslaber M, Stangl V, Haindl MT, Enzinger C, Hoftberger R (2020) A fulminant case of demyelinating encephalitis with extensive cortical involvement associated with anti-MOG antibodies. Front Neurol 11:31. https://doi.org/10.3389/fneur.2020.00031
Hoftberger R, Guo Y, Flanagan EP, Lopez-Chiriboga AS, Endmayr V, Hochmeister S, Joldic D, Pittock SJ, Tillema JM, Gorman M, Lassmann H, Lucchinetti CF (2020) The pathology of central nervous system inflammatory demyelinating disease accompanying myelin oligodendrocyte glycoprotein autoantibody. Acta Neuropathol 139:875–892. https://doi.org/10.1007/s00401-020-02132-y
Hacohen Y, Banwell B (2019) Treatment approaches for MOG-Ab-associated demyelination in children. Curr Treat Options Neurol 21:2. https://doi.org/10.1007/s11940-019-0541-x
Gonzalez Sanchez JJ, Nora JE, de Notaris M, Arboix JR, Garcia CG, Rodriguez EF (2010) A case of malignant monophasic multiple sclerosis (Marburg's disease type) successfully treated with decompressive hemicraniectomy. J Neurol Neurosurg Psychiatry 81:1056–1057. https://doi.org/10.1136/jnnp.2007.142133
Ragel BT, Fassett DR, Baringer JR, Browd SR, Dailey AT (2006) Decompressive hemicraniectomy for tumefactive demyelination with transtentorial herniation: observation. Surg Neurol 65:582–583. https://doi.org/10.1016/j.surneu.2005.08.015
Vakharia K, Kamal H, Atwal GS, Budny JL (2018) Transtentorial herniation from tumefactive multiple sclerosis mimicking primary brain tumor. Surg Neurol Int 9:208. https://doi.org/10.4103/sni.sni_131_18
Beez T, Munoz-Bendix C, Ahmadi SA, Steiger HJ, Beseoglu K (2019) From decompressive craniectomy to cranioplasty and beyond-a pediatric neurosurgery perspective. Childs Nerv Syst 35:1517–1524. https://doi.org/10.1007/s00381-019-04303-z
Beez T, Munoz-Bendix C, Steiger HJ, Beseoglu K (2019) Decompressive craniectomy for acute ischemic stroke. Crit Care 23:209. https://doi.org/10.1186/s13054-019-2490-x
Hohenhaus M, Shah MJ, Eckenweiler M, van Velthoven V (2015) Decompressive hemicraniectomy in the management of acute stroke in children with Moyamoya disease: the difficult balance between the spontaneous postoperative revascularization process and the need for cranial reconstruction. Childs Nerv Syst 31:2369–2373. https://doi.org/10.1007/s00381-015-2802-7
Shah S, Murthy SB, Whitehead WE, Jea A, Nassif LM (2013) Decompressive hemicraniectomy in pediatric patients with malignant middle cerebral artery infarction: case series and review of the literature. World Neurosurg 80:126–133. https://doi.org/10.1016/j.wneu.2013.06.001
Payne ET, Rutka JT, Ho TK, Halliday WC, Banwell BL (2007) Treatment leading to dramatic recovery in acute hemorrhagic leukoencephalitis. J Child Neurol 22:109–113. https://doi.org/10.1177/0883073807299971
Omay SB, Carrion-Grant GM, Kuzmik GA, Fu M, Grant R, Schindler JL, Diluna ML, Duncan CC, Bulsara KR (2013) Decompressive hemicraniectomy for ischemic stroke in the pediatric population. Neurosurg Rev 36:21–24; discussion 24-25. https://doi.org/10.1007/s10143-012-0411-4
Kenning TJ, Gandhi RH, German JW (2009) A comparison of hinge craniotomy and decompressive craniectomy for the treatment of malignant intracranial hypertension: early clinical and radiographic analysis. Neurosurg Focus 26:E6. https://doi.org/10.3171/2009.4.FOCUS0960
Kenning TJ, Gooch MR, Gandhi RH, Shaikh MP, Boulos AS, German JW (2012) Cranial decompression for the treatment of malignant intracranial hypertension after ischemic cerebral infarction: decompressive craniectomy and hinge craniotomy. J Neurosurg 116:1289–1298. https://doi.org/10.3171/2012.2.JNS111772
Layard Horsfall H, Mohan M, Devi BI, Adeleye AO, Shukla DP, Bhat D, Khan M, Clark DJ, Chari A, Servadei F, Khan T, Rubiano AM, Hutchinson PJ, Kolias AG (2020) Hinge/floating craniotomy as an alternative technique for cerebral decompression: a sco** review. Neurosurg Rev 43:1493–1507. https://doi.org/10.1007/s10143-019-01180-7
Park YS, Kogeichi Y, Haku T, Kim TK, Yokota H, Nakagawa I, Motoyama Y, Nakase H (2021) Hinge and floating decompressive craniotomy for infantile acute subdural hematoma: technical note. Childs Nerv Syst 37:295–298. https://doi.org/10.1007/s00381-020-04942-7
Structural brain differences in school-age children with and without single-suture craniosynostosis (2017) J Neurosurg Pediatr 19:479–489. https://doi.org/10.3171/2016.9.PEDS16107
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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