Abstract
We present the case of a previously healthy 13-year-old boy who was admitted to the emergency department with acute flaccid paralysis. Magnetic resonance imaging revealed radiological evidence of longitudinally extensive transverse myelitis. Additionally, homogeneous T2 signal increase was observed in the pons and medulla oblongata, initially indicating brainstem encephalitis. Subsequent evaluations confirmed a coexistence of diffuse midline glioma (DMG) in the brain stem alongside acute transverse myelitis (ATM). Children with ATM generally have a more favorable prognosis than adults. However, despite the implementation of advanced treatment methods, the patient’s quadriplegia did not improve and resulted in spinal cord sequela atrophy. DMG exhibits an aggressive growth pattern and lacks a known curative treatment. This case represents an exceedingly rare synchronous occurrence of aggressive conditions, underscoring the importance of raising awareness among physicians. Furthermore, we aim to discuss the radiologic differential diagnosis, as this is the first documented instance in the literature.
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References
Defresne P, Hollenberg H, Husson B, Tabarki B, Landrieu P, Huault G, Tardieu M, Sébire G (2003) Acute transverse myelitis in children: clinical course and prognostic factors. J Child Neurol 18(6):401–406. https://doi.org/10.1177/08830738030180060601
Absoud M, Greenberg BM, Lim M, Lotze T, Thomas T, Deiva K (2016) Pediatric transverse myelitis. Neurology 87(9 Suppl 2):46–52. https://doi.org/10.1212/WNL.0000000000002820
DeSanto J, Ross JS (2011) Spine infection/inflammation. Radiol Clin North Am 49(1):105–127. https://doi.org/10.1016/j.rcl.2010.07.018
Trebst C, Raab P, Voss EV, Rommer P, Abu-Mugheisib M, Zettl UK, Stangel M (2011) Longitudinal extensive transverse myelitis–it’s not all neuromyelitis optica. Nat Rev Neurol 7(12):688–698. https://doi.org/10.1038/nrneurol.2011.176
Jubelt B, Mihai C, Li TM, Veerapaneni P (2011) Rhombencephalitis/brainstem encephalitis. Curr Neurol Neurosci Rep 11(6):543–552. https://doi.org/10.1007/s11910-011-0228-5
Vecchio D, Virgilio E, Naldi P, Comi C, Cantello R (2018) MOG-antibody demyelinating diseases: a case of post-partum severe rhombencephalitis and transverse myelitis. Mult Scler Relat Disord 21:9–10. https://doi.org/10.1016/j.msard.2018.02.006
Sotoudeh H, Razaei A, Saadatpour Z, Gaddamanugu S, Choudhary G, Shafaat O, Singhal A (2021) Brainstem encephalitis. The role of imaging in diagnosis. Curr Probl Diagn Radiol 50(6):946–960. https://doi.org/10.1067/j.cpradiol.2020.09.004
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW (2016) The 2016 world health organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131(6):803–820. https://doi.org/10.1007/s00401-016-1545-1
Damodharan S, Helgager J, Puccetti D (2023) Indolent presentation of a diffuse midline glioma, H3 K27-altered. Childs Nerv Syst 39(3):833–835. https://doi.org/10.1007/s00381-022-05668-4
Solinsky R, Bunnell AE (2016) Rehabilitation of a patient with overlap of acute transverse myelitis and Bickerstaff’s brainstem encephalitis: a case report. Spinal Cord Ser Cases 7(2):15032. https://doi.org/10.1038/scsandc.2015.32
Jarius S, Kleiter I, Ruprecht K et al (2016) MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: Brainstem involvement - frequency, presentation and outcome. J Neuroinflammation 13(1):281. https://doi.org/10.1186/s12974-016-0719-z
Chan KH, Tse CT, Chung CP, Lee RLC, Kwan JSC, Ho PWL, Ho JWM (2011) Brain involvement in neuromyelitis optica spectrum disorders. Arch Neurol 68(11):1432–1439. https://doi.org/10.1001/archneurol.2011.249
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Emine Caliskan and Safiye Gunes Sager wrote the main manuscript text. Ufuk Yukselmis and Ahmet Kasim Kilic reviewed the manuscript. Hediye Pinar Gunbey prepared figures.
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Caliskan, E., Sager, S.G., Yukselmis, U. et al. Coexistence of longitudinally extensive transverse myelitis and diffuse midline glioma in the brainstem in an adolescent boy with acute flaccid paralysis. Childs Nerv Syst 40, 227–232 (2024). https://doi.org/10.1007/s00381-023-06163-0
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DOI: https://doi.org/10.1007/s00381-023-06163-0