Presentation
Recurrent convulsions and disturbance of consciousness with prior one week history of upper respiratory tract infection symptoms.
Patient Data
Bilateral asymmetrical hypo-densities of cerebral white matter associated with punctate hemorrhagic foci affecting the right basal ganglia and periventricular white matter, as well as hypo-density involving the deep white matter of both cerebellar hemispheres.
Bilateral asymmetrical multiple lesions involve subcortical and periventricular cerebral white matter, as well as deep cerebellar white matter. Some lesions show restricted diffusion, and there are multiple foci of hemorrhage affecting the right basal ganglia and periventricular white matter. No significant enhancement is observed in the post-contrast study. Additionally, a left-sided internal-type porencephalic cyst is noted.
Case Discussion
Acute hemorrhagic leukoencephalitis (AHLE) is a rare demyelinating disease characterized by an acute, rapidly progressive, fulminant inflammation of the white matter that was first described by Hurst in 1941.
Acute disseminated encephalomyelitis (ADEM) and AHLE share many histopathologic features and autoimmune response mechanisms. Therefore, AHLE is regarded as the most severe subtype of ADEM.
The prognosis is poor, with death from brain edema within one week of onset of the encephalopathy, although favorable neurologic outcomes have been published with early and aggressive treatment.
Treatment: combinations of corticosteroids, immunoglobulin, cyclophosphamide, and plasma exchange.
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