Favourable Outcome in a 33-Year-Old Female with Acute Haemorrhagic Leukoencephalitis

Abstract

Background: Acute haemorrhagic leukoencephalitis (AHLE) is a rare and rapidly fatal disease of unknown aetiology. There is a paucity of literature on the presentation and management of this rare disease. Case Description: We report the case of a 33-year-old female presenting with headache and left-sided apraxia. Imaging revealed a right-sided white matter lesion with extensive cytotoxic oedema. Pathology was suggestive of AHLE. She underwent an open excisional biopsy and was treated with high-dose corticosteroids. Three months since symptom onset she remains clinically well with resolving apraxia and radiological appearance. Conclusion: This case may represent a milder spectrum of AHLE, which responded favourably to corticosteroids.

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

“…AHLE is a rare demyelinating condition with significant associated mortality ( Fontoura et al., 2002 ). It has previously been reported as a complication of other viral illnesses ( Solis et al., 2017 ). AHLE is thought to be the most fulminant variant of acute disseminated encephalomyelitis (ADEM) with rapid progression of white matter inflammation ( Rawal et al., 2019 ).…”

“…Although the haemorrhagic component of AHLE is usually small, we believe in our case, this was severe enough to be depicted by CT due to possible aggravation effects of anticoagulation and hypertension. The pathophysiology of AHLE is not known but cross reactivation of host myelin and viral antigens is thought to be one possible mechanism ( Fontoura et al., 2002 ; Solis et al., 2017 ; Duggal et al., 2014 ). Moreover, reported cases of COVID-19 related diffuse leukoencephalopathy and microhaemorrhages have been thought to be likely in association with hypoxaemia ( Radmanesh et al., 2020a ).…”

“…Although the AHLE diagnosis is usually confirmed at autopsy and biopsy ( Solis et al., 2017 ), MRI characteristics are useful in early detection of the condition. MRI findings show multifocal lesions ( Solis et al., 2017 ; Poyiadji et al., 2020 ). Susceptibility weighted sequences demonstrate haemorrhage.…”

“…There is white matter signal alteration with sparing of the grey matter and subcortical U-fibers, in a predominantly frontoparietal distribution. The Basal ganglia, brain stem and spinal cord may also be affected ( Solis et al., 2017 ). Some have suggested a potential role for corticosteroids or intravenous immunoglobulin as treatment ( Poyiadji et al., 2020 ; Bridwell et al., 2020 ; Pilotto et al., 2020 ).…”

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

“…However, in our review of the literature, we found an overall mortality of 46.5%, which is substantially lower. Moreover, 14% of patients made a full recovery and returned to their premorbid neurological baseline ( 3 , 5 , 7 , 14 , 18 , 35 ), and 11% survived with only minor neurological sequelae ( 2 , 12 , 15 , 23 , 38 ). The reason for the better outcome in this systematic review compared to previous studies ( 3 , 4 ) remains unclear.…”