Posterior leukoencephalopathy syndrome may not be reversible

The association of an acute reversible encephalopathy with transient occipi tal lobe abnormalities on imaging studies is well known. This condition has been called reversible posterior leukoencephalopathy syndrome. The clinica l presentation usually includes seizures, headache, altered mental status, and blindness, often associated with hypertension and immunosuppressants, T he authors discuss a two-year-old male with Down syndrome who presented 2 m onths after allogeneic bone marrow transplantation with severe oculogyric c risis, without other complaints. The patient was being treated for hyperten sion and was receiving cyclosporine for prophylaxis of graft-vs-host diseas e. A computed tomography scan of the head revealed marked bilateral lucenci es mainly involving the white matter of the occipital lobes, with a few foc i of punctate hemorrhage, The condition improved when cyclosporine was disc ontinued, but an area of leukomalacia was identified on follow-up magnetic resonance imaging. To the authors' knowledge, oculogyric crisis as a presen tation of reversible posterior leukoencephalopathy has not been previously described. Recognizing this association is important, because patients rece iving cyclosporine are often receiving other medications that can potential ly cause dystonic eye movements, possibly leading to a delay in diagnosis a nd treatment, which can result in an irreversible neurologic deficit. (C) 1 999 by Elsevier Science Inc. All rights reserved.