Acute hemorrhagic leukoencephalitis (AHLE): An exceptional clinical presentation with the association of scrub typhus and opercular syndrome

Abstract

Introduction

Acute hemorrhagic leukoencephalitis (AHLE) is a rare and severe form of acute disseminated encephalomyelitis (ADEM), characterized by rapid progression and inflammation of the brain's white matter. It's often preceded by an upper respiratory infection, bacterial or viral illness, or even vaccination. An opercular syndrome is characterized by the triad of facial weakness, dysarthria, and difficulty in swallowing due to lesions in the operculum, the part of the brain that covers the insula. When AHLE is associated with opercular syndrome and scrub typhus, it presents a unique and challenging clinical condition. Here, we are presenting a case of AHLE (a subtype of ADEM), which is very rare but is also associated with scrub typhus and opercular syndrome (a rare syndrome). In this way, the presenting case becomes the rarest of rare or most exceptional.

Case report

A complex clinical case involving a 12-year-old girl, who had the symptoms of fever, vomiting, headache, focal seizure, and altered sensorium for the last 25 days, she was initially diagnosed as tuberculous meningitis (TBM) in an outside hospital, but there was no significant improvement with standard anti-tubercular therapy (ATT). Due to no notable improvement, she was brought to our institute (IMS, BHU) for further evaluation suggests a need for a comprehensive approach to uncover any underlying conditions that may be contributing to her symptoms. The additional and necessary investigations, such as fever workup, repeat CSF analysis, routine blood tests, and MRI brain, were crucial steps in this process. The serum of the patient was positive for IgM scrub typhus suggesting an active infection. The presence of lymphocytic pleocytosis, elevated protein, and reduced sugar in the CSF indicate inflammation and dysfunction within the central nervous system (CNS). The MRI findings of extensive subcortical and white matter changes with haemorrhage further suggest AHLE. Symptoms and MRI features also projected the diagnosis of opercular syndrome. CSF neuro-infection panel was negative. Stopping ATT and initiating pulse steroid therapy along with doxycycline was a prudent decision. Doxycycline is the treatment of choice for scrub typhus, and the addition of pulse steroid therapy aims to reduce inflammation and prevent further neurological damage.

Conclusion

AHLE (Acute Hemorrhagic Leukoencephalitis) is indeed a severe and often fatal condition, and its association with scrub typhus and opercular syndrome presents a unique and challenging clinical scenario. Given its rarity and the potential for misdiagnosis or lack of recognition, highlighting such cases can be crucial in increasing awareness among healthcare professionals. Moreover, documenting such rare occurrences can contribute significantly to the medical literature, providing valuable insights into the clinical features, pathophysiology, and management strategies of these conditions. This, in turn, can guide future research and clinical practice, potentially leading to advancements in diagnosis and treatment.