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

摘要

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 Introduction Acute hemorrhagic leukoencephalitis (AHLE) is a rare, fulminant, demyelinating condition of the central nervous system (CNS) which is usually fatal. The condition was first described by Weston Hurst [1] in 1941 and later by Adams [2] in 1949. Since these first descriptions, there has been less than 100 adult [345678] and 10 pediatric [910] cases of AHLE reported in the literature. So far, only 4 children with the condition have survived and of these only one has definitive pathological confirmation of the diagnosis.It is widely hypothesized that because AHLE shares many clinical features with acute disseminated encephalomyelitis (ADEM), the two are part of a spectrum of diseases rather than two distinct entities. Both conditions are commonly preceded by upper respiratory tract infections, infection with Herpes simplex virus (HSV), Epstein-Barr virus (EBV), influenza virus, rubella, measles, mumps or by vaccination. We report a fatal case of pediatric AHLE in a sickler who was diagnosed with infectious mononucleosis some 4 years previously. To our knowledge, this is the first reported case of AHLE occurring in a person with sickle cell disease. Case Report This 10 year old girl with sickle cell disease (Hb SS) presented with a one week history of a “flu-like” illness which resolved. One day prior to presentation she developed severe headache associated with fever and one episode of vomiting. She was treated with acetaminophen but was found unresponsive some eight hours later. On examination, she was unresponsive with a Glasgow Coma Score of 10/15 (eye response-5; verbal response-1; motor response -4) with fluctuations in consciousness noted during examination. She was also febrile with a temperature of 100.4 F. There was generalized hypotonia and hyporeflexia associated with marked neck stiffness. Kernig’s and Brudzinsky’s signs were negative. There was no obvious hemiparesis. Her pupils were small and sluggishly reactive to light. Fundoscopy revealed a normal appearing optic disc on the right while the left was not properly visualized. Laboratory results at the time revealed a polymorphonuclear leukocytosis. A lumbar puncture was not performed.A computed tomography (CT) scan of the brain was done which revealed bilateral frontal lobe white matter changes which were thought to be due to either cerebritis or an infiltrative disease such as lymphoma or a high grade glioma. There was evidence of marked cerebral edema with early coning. (Figure 1).