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