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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http://dx.doi.org/10.5281/zenodo.10814411 | DOI Listing |
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