Report of 3 cases of acute hemorrhagic leukoencephalitis in Mexico
DOI:
https://doi.org/10.5281/zenodo.10814411Keywords:
Acute Hemorrhagic Leukoencephalitis, Autoantibody, Aquaporin 4, Central Nervous SystemAbstract
Background: Acute hemorrhagic leukoencephalitis (AHLE) or Weston-Hurst syndrome is a rare and severe variant of acute disseminated encephalomyelitis (ADEM). It causes fulminant hemorrhagic demyelination of the central nervous system, and has high mortality and morbidity. Due to the increase in cases and to its high rate of mortality, we decided to make a series of cases and a review of the disease.
Clinical cases: We present 3 cases of Mexican patients, with clinical, radiological, and cerebrospinal fluid presentation compatible with the disease, with little response to treatment and high short-term morbidity. In 2 of the cases, there was positive evidence of aquaporin 4 antibodies (ac-AQP4), without any other apparent trigger.
Conclusions: There is little evidence on the clinical evolution and medical management of the disease. With the arrival of the COVID-19 pandemic, there was an increase in cases of these diseases of autoimmune etiology. Although these cases did not present previous infection by COVID 19, there was an increase in the immunogenicity of ab- AQP4. Because of this, we believe it is important to distinguish it, describe as many cases as possible, and provide more information about its diagnosis, treatment, and prognosis. Likewise, we carried out a brief systematic review of the bibliography.
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Nabi S, Badshah M, Ahmed S, et al. Weston-Hurst syndrome: a rare fulminant form of acute disseminated encephalomyelitis (ADEM). BMJ Case Rep. 2016;2016:bcr2016217215. doi: 10.1136/bcr-2016-217215.
Ancau M, Liesche-Starnecker F, Niederschweiberer J, et al. Case Series: Acute Hemorrhagic Encephalomyelitis After SARS-CoV-2 Vaccination. Front Neurol. 2022;12:820049. doi: 10.3389/fneur.2021.820049.
Grzonka P, Scholz MC, De Marchis GM, et al. Acute Hemorrhagic Leukoencephalitis: A Case and Systematic Review of the Literature. Front Neurol. 2020;11:899. doi: 10.3389/fneur.2020.00899.
Waak M, Malone S, Sinclair K, et al. Acute Hemorrhagic Leukoencephalopathy: Pathological Features and Cerebrospinal Fluid Cytokine Profiles. Pediatr Neurol. 2019;100:92-6. doi: 10.1016/j.pediatrneurol.2019.06.013.
Manzano GS, McEntire CRS, Martinez-Lage M, et al. Acute Disseminated Encephalomyelitis and Acute Hemorrhagic Leukoencephalitis Following COVID-19: Systematic Review and Meta-synthesis. Neurol Neuroimmunol Neuroinflamm. 2021;8(6):e1080. doi: 10.1212/NXI.0000000000001080.
Chen X, Laurent S, Onur OA, et al. A systematic review of neurological symptoms and complications of COVID-19. J Neurol. 2021 Feb;268(2):392-402. doi: 10.1007/s00415-020-10067-3.
Aguilar-Vázquez CA, Raymundo-Carrillo AD, Ruvalcaba-Sánchez ND, et al. Leucoencefalopatía hemorrágica aguda. Reporte de caso. Rev Med Inst Mex Seguro Soc. 2023;61(2):239-44.
Krupp LB, Tardieu M, Amato MP, et al; International Pediatric Multiple Sclerosis Study Group. International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions. Mult Scler. 2013;19(10):1261-7. doi: 10.1177/1352458513484547.
Zoghi A, Ramezani M, Roozbeh M, et al. A case of possible atypical demyelinating event of the central nervous system following COVID-19. Mult Scler Relat Disord. 2020;44:102324. doi: 10.1016/j.msard.2020.102324.
Cao L, Ren L. Acute disseminated encephalomyelitis after severe acute respiratory syndrome coronavirus 2 vaccination: a case report. Acta Neurol Belg. 2022;122(3):793-5. doi: 10.1007/s13760-021-01608-2.
Nagireddy RBR, Kumar A, Singh VK, et al. Clinicoradiological comparative study of Aquaporin-4-IgG seropositive neuromyelitis optica spectrum disorder (NMOSD) and MOG antibody associated disease (MOGAD): A prospective observational study and review of literature. J Neuroimmunol. 2021;361:577742. doi: 10.1016/j.jneuroim.2021.577742.
Karapanayiotides T, Geka E, Prassopoulos P, et al. Concentric demyelination pattern in COVID-19-associated acute haemorrhagic leukoencephalitis: a lurking catastrophe? Brain. 2020;143(12):e100. doi: 10.1093/brain/awaa375.
Varadan B, Shankar A, Rajakumar A, et al. Acute hemorrhagic leukoencephalitis in a COVID-19 patient-a case report with literature review. Neuroradiology. 2021;63(5):653-61. doi: 10.1007/s00234-021-02667-1.
Duignan S, Wright S, Rossor T, et al. Myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies are highly specific in children with acquired demyelinating syndromes. Dev Med Child Neurol. 2018;60(9):958-62. doi: 10.1111/dmcn.13703.
Bhatt P, Bray L, Raju S, et al. Temporal Trends of Pediatric Hospitalizations with Acute Disseminated Encephalomyelitis in the United States: An Analysis from 2006 to 2014 using National Inpatient Sample. J Pediatr. 2019;206:26-32.e1. doi: 10.1016/j.jpeds.2018.10.044.
Bonduelle T, Stricker J, Minéo JF, et al. Weston-Hurst syndrome with acute hemorrhagic cerebellitis. Clin Neurol Neurosurg. 2018;173:118-9. doi: 10.1016/j.clineuro.2018.08.007.
Bernarding J, Braun J, Koennecke HC. Diffusion and perfusion-weighted MR imaging in a patient with acute demyelinating encephalomyelitis (ADEM). J Magn Reson Imaging. 2002;15(1):96-100. doi: 10.1002/jmri.10045. PMID: 11793463.
Mondia MWL, Reyes NGD, Espiritu AI, et al. Acute hemorrhagic leukoencephalitis of Weston Hurst secondary to herpes encephalitis presenting as status epilepticus: A case report and review of literature. J Clin Neurosci. 2019;67:265-70. doi: 10.1016/j.jocn.2019.06.020.
Hardy TA, Guo Y, Flanagan EP, et al. The clinical spectrum of haemorrhagic CNS inflammatory demyelinating lesions. Mult Scler. 2022;28(11):1710-8. doi: 10.1177/13524585221094241.
Yong MH, Chan YFZ, Liu J, et al. A Rare Case of Acute Hemorrhagic Leukoencephalitis in a COVID-19 Patient. J Neurol Sci. 2020;416:117035. doi: 10.1016/j.jns.2020.117035.
Ashraf Z, Todnam N, Morgan J, et al. 42-Year-Old Man with Worsening Headache. Brain Pathol. 2019;29(2):305-306. doi: 10.1111/bpa.12706.
Ryan LJ, Bowman R, Zantek ND, et al. Use of therapeutic plasma exchange in the management of acute hemorrhagic leukoencephalitis: a case report and review of the literature. Transfusion. 2007;47(6):981-6. doi: 10.1111/j.1537-2995.2007.01227.
Ancau M, Liesche-Starnecker F, Niederschweiberer J, et al. Case Series: Acute Hemorrhagic Encephalomyelitis After SARS-CoV-2 Vaccination. Front Neurol. 2022;12:820049. doi: 10.3389/fneur.2021.820049.
Waak M, Malone S, Sinclair K, et al. Acute Hemorrhagic Leukoencephalopathy: Pathological Features and Cerebrospinal Fluid Cytokine Profiles. Pediatr Neurol. 2019;100:9-96. doi: 10.1016/j.pediatrneurol.2019.06.013.
Sureshbabu S, Babu R, Garg A, et al. Acute hemorrhagic leukoencephalitis unresponsive to aggressive immunosuppression. Clinical and Experimental Neuroimmunology. 2017;8. doi: 10.1111/cen3.12361.
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