비전형적인 중상으로 발현한 급성 출혈성 백질뇌염

Abstract

Acute disseminated encephalomyelitis (ADEM) and acute hemorrhagic leukoencephalitis (Hurst’s disease) are rare autoimmune demyelinating disorders, which show a monophasic illness with preceding infection. We report a 42-yearold woman presented with multiphasic and progressive neurologic deterioration without definite evidences of infection. She developed hypesthetic ataxia, followed by ipsilateral weakness after a weak, and finally encephalopathy after a month. In contrast to the first MRI showing a small longitudinal lesion, the next images revealed massive bilateral frontal lesions with hemorrhagic necrosis and biopsy unveiled inflammatory demyelination.