Acute haemorragic leukoencephalitis of Hurst variety(AHLE) -MRI

Case Report: 39 yr old male with monoparesis of LLL with no definite preceding viral illness , shows on MRI, multiple white matter lesions of varying sizes with subtle restricted diffusion in one lesion/multiple microhges with basal ganglia/ brainstem/ corpus callosum involvement with frontoparietal lesions perpendicular to calloso septal interface , and normal cervical cord / optic nerves/ chiasm suggesting Acute haemorragic leukoencephalitis of Hurst variety(AHLE) or Western -Hurst syndrome (Contrast study not done due to un affordability)

Teaching points by Dr MGK Murthy,

MRI tech-Mr Harinath

1.Rare form of Acute disseminated encephalomyelitis (ADEM) and occurs in young adults (Cf classical ADEM -children)

2.Etiology is possibly autoimmune cross reaction to myelin antigens

3.Preceeding viral/ bacterial illness (2-12days)may be present in 50% cases

4.Punctate necrotising (tumefactive) hgic lesions of white matter hall mark including basal ganglia and typically absent in cord (unlike other ADEM). Imaging is due to necrotising venulitis with perivascular haemorrhages

5.Histopathology shows fibrinoid venous necrosis, with classical hemorrhages around the necrotic venules resembling “ring and ball”

6. Prognosis poor despite initial response to steroids and may lead to fatal outcome