Unilateral optic neuritis as presenting feature of acute hemorrhagic leukoencephalitis

Purpose We report a case of 23 year-old previously healthy female with acute hemorrhagic leukoencephalitis AHLE (Hurst disease) leading to lethal outcome within two weeks after presentation with unilateral visual loss and moderate upper respiratory tract infection with leukocytosis. Methods At presentation, VA was 0.3 RE and 1.2 LE with centrocecal defect on right eye, positive RAPD and dyschromatopsia. Optic nerve on fundus examination was normal. Neurological examination revealed right sided hyperreflexia and diminished right plantar response. Initial MRI revealed few focal periventricular hyperintensive lesions consistent with demyelination. Results Treatment started with 1g metilprednisolon i.v. for 3 days with good initial effect. Third day of treatment, VA in right eye was normal (1.2), RAPD was negative and dyschromatopsia improved. Three days later, altered consciousness with seizures developed, subsequently followed by right hemiparesis and coma. Cerebrospinal fluid pleocytosis was present. Follow- up MRI, obtained 4 days after initial one, revealed large areas of hemorrhagic necrosis in the left hemisphere.Despite continued high dose steroid treatment,antiviral prophylaxis and plasmapharesis, the disease progressed to death within days. Conclusion Acute hemorrhagic leukoencephalitis AHLE (Hurst disease) is a rare fulminant demyelinating disorder characterized by a fulminant clinical course due to hemorrhagic necrosis of the white matter associated with high rate of mortality. We present a case of this severe disease in which optic neuritis was presenting focal neurological deficit.