Acute Hemorrhagic Leukoencephalitis: A Case and Systematic Review of the Literature

Abstract

Objectives: To present a patient with acute hemorrhagic leukoencephalitis (AHLE) and a systematic review of the literature analyzing diagnostic procedures, treatment, and outcomes of AHLE. Methods: PubMed and Cochrane databases were screened. Papers published since 01/01/2000 describing adult patients are reported according to the PRISMA-guidelines. Results: A 59-year old male with rapidly developing coma and cerebral biopsy changes compatible with AHLE is presented followed by 43 case reports from the literature including males in 67% and a mean age of 38 years. Mortality was 47%. Infectious pathogens were reported in 35%, preexisting autoimmune diseases were identified in 12%. Neuroimaging revealed uni-or bihemispheric lesions in 65% and isolated lesions of the cerebellum, pons, medulla oblongata or the spinal cord without concomitant hemispheric involvement in 16%. Analysis of the cerebrospinal fluid showed an increased protein level in 87%, elevated white blood cells in 65%, and erythrocytes in 39%. Histology (reported in 58%) supported the diagnosis of AHLE in all cases. Glucocorticoids were used most commonly (97%), followed by plasmapheresis (26%), and intravenous immunoglobulins (12%), without a clear temporal relationship between treatment and the patients' clinical course. Conclusions: Although mortality was lower than previously reported, AHLE remains a life-threatening neurologic emergency with high mortality. Diagnosis is challenging as the level of evidence regarding the diagnostic yield of clinical, neuroimaging and laboratory characteristics remains low. Hence, clinicians are urged to heighten their awareness and to prompt cerebral biopsies in the context of rapidly progressive neurologic decline of unknown origin with the concurrence of the compiled characteristics. Future studies need to focus on treatment characteristics and their effects on course and outcome.

Acute hemorrhagic leukoencephalitis in a COVID-19 patient—a case report with literature review

“…AHLE, a monophasic demyelinating disease and a hyper-acute severe form of ADEM, is a rare clinical entity with a poor prognosis. There is antecedent history of viral or bacterial infection in 50–75% of patients, with most cases following an upper respiratory tract infection [ 1 ]. ADEM is not uncommon during childhood, with hospitalization estimated at ~0.5 per 100,000 patients [ 2 , 3 ].…”

“…ADEM is not uncommon during childhood, with hospitalization estimated at ~0.5 per 100,000 patients [ 2 , 3 ]. A much lower incidence has been reported in adults [ 1 ]. In comparison to other presentations of ADEM, AHLE is much rarer, and there are only a few case reports describing this entity [ 1 ].…”

Acute haemorrhagic leukoencephalitis (Hurst disease) in severe COVID- 19 infection

“…Imaging scans play a crucial role in the early identification of AHLE. MRI could also differentiate AHLE from ADEM through demonstrating the presence of petechial haemorrhages and extensive white matter lesions in AHLE ( Grzonka et al., 2020 ). Another differential diagnosis of AHLE could be infectious encephalitis.…”

“…Another differential diagnosis of AHLE could be infectious encephalitis. In HSV-1, brain involvement is often in temporal and limbic areas ( Grzonka et al., 2020 ). Infectious encephalitis was excluded with negative CSF PCR and serology results.…”

The Role of Extracellular Vesicles in Demyelination of the Central Nervous System

“…Acute disseminated encephalomyelitis (ADEM) is also an inflammatory autoimmune disorder of the CNS myelin, but, in this case, an infectious etiology has been observed in most cases: the first symptoms are frequently preceded by viral or bacterial infections [79]. Acute hemorrhagic leukoencephalitis (AHL), a variant of ADEM, is a human autoimmune disorder probably caused by molecular mimicry stimulated by viral or bacterial pathogens [86]. On the other hand, several demyelinating CNS diseases have a defined etiology such as viral infection, immunological mechanisms, toxins, metabolic disorders or ischemia ( Figure 1).…”