Acute Hemorrhagic Leukoencephalitis in a Patient with Sickle Cell Disease: A Case Report

Acute hemorrhagic leukoencephalitis (AHLE), otherwise known as Hurst’s Disease, is a rare, rapidly progressive and usually fatal demyelinating condition affecting the central nervous system. It is usually preceded by an upper respiratory tract infection and is thought by many authors to be a hyperacute form of post infectious encephalomyelitis. It was recently found to be linked to Epstein-Barr virus infection. We report what we think is the first case of AHLE occurring in a child with homozygous sickle cell disease. As with our case, this is usually a fatal condition; however, there have been few recent reports of survival following aggressive, high dose corticosteroid therapy. It is therefore important to recognize this disease early so appropriate treatment can be instituted. Work done in the Department of Pathology, University Hospital of the West Indies, Kingston, Jamaica WI