Paediatric acute haemorrhagic leukoencephalitis

Abstract

We report a case of a preschool age girl, previously healthy, referred to our hospital on ventilatory support with a history of vomiting, headache, and rapid neurological worsening within 24 hours in the form of seizures, encephalopathy and loss of consciousness. On presentation, she was deeply comatose with dilated non-reactive pupils, absent brainstem reflexes and flaccid quadriplegia. Diagnosis of acute haemorrhagic leukoencephalitis was considered based on laboratory and neuroimaging findings. MRI of the brain showed fluffy white matter hyperintensities and microhaemorrhages in bilateral cerebral hemispheres and thalami. Aggressive treatment with methylprednisolone, plasmapheresis and intravenous immunoglobulin showed dramatic improvement with no neurological sequelae. Our case is unique in a way that despite the hyperacute onset and rapid deterioration, with a fulminant course in the intensive care unit, the child recovered dramatically with aggressive management.

Keywords: Neurology (drugs and medicines); Paediatric intensive care; Paediatrics (drugs and medicines).

Figure 1

The axial T2 and FLAIR images at the level of basal ganglia (A, B) and supraventricular level (C, D) does not show any abnormal signal abnormality. The diffusion-weighted images (E, F) do not reveal any diffusion abnormality. The scan was interpreted as a normal study. FLAIR, fluid-attenuated inversion recovery.

Figure 2

The axial T2 images (A) shows a subtle hyperintense signal involving the bilateral striatum (white arrows). Abnormal patchy T2 and FLAIR hyperintensities are noted at bilateral frontoparietal subcortical white matter (B, C). The diffusion images (D, E) show focal restriction at the bilateral external capsule. The postcontrast T1 image does not show any abnormal parenchymal or leptomeningeal enhancement (F). FLAIR, fluid-attenuated inversion recovery.

Figure 3

Follow-up MRI at an interval of 3 weeks. The axial T2 (A) and FLAIR (B) images show diffuse cerebral atrophy with the resolution of signal changes at the bilateral striatum and bilateral frontoparietal subcortical white matter. The diffusion images (C, D) show T2 shining through at bilateral external capsule without diffusion restriction (white arrows). FLAIR, fluid-attenuated inversion recovery.