Elsevier

Pediatric Neurology

Volume 20, Issue 3, March 1999, Pages 241-243
Pediatric Neurology

Case Reports
Posterior leukoencephalopathy syndrome may not be reversible

https://doi.org/10.1016/S0887-8994(98)00148-9Get rights and content

Abstract

The association of an acute reversible encephalopathy with transient occipital lobe abnormalities on imaging studies is well known. This condition has been called reversible posterior leukoencephalopathy syndrome. The clinical presentation usually includes seizures, headache, altered mental status, and blindness, often associated with hypertension and immunosuppressants. The authors discuss a two-year-old male with Down syndrome who presented 2 months after allogeneic bone marrow transplantation with severe oculogyric crisis, without other complaints. The patient was being treated for hypertension and was receiving cyclosporine for prophylaxis of graft-vs-host disease. A computed tomography scan of the head revealed marked bilateral lucencies mainly involving the white matter of the occipital lobes, with a few foci of punctate hemorrhage. The condition improved when cyclosporine was discontinued, but an area of leukomalacia was identified on follow-up magnetic resonance imaging. To the authors’ knowledge, oculogyric crisis as a presentation of reversible posterior leukoencephalopathy has not been previously described. Recognizing this association is important, because patients receiving cyclosporine are often receiving other medications that can potentially cause dystonic eye movements, possibly leading to a delay in diagnosis and treatment, which can result in an irreversible neurologic deficit.

Introduction

The association of an acute encephalopathy and transient occipital lobe abnormalities on imaging studies is well known. This condition has been called reversible posterior leukoencephalopathy syndrome(RPLS), emphasizing the transitory nature of the dysfunction once therapy is instituted [1]; it has also been called occipitoparietal encephalopathy because involvement of the gray matter is observed on occasion [2]. The clinical presentation includes seizures, headache, altered mental status, and blindness, often secondary to hypertension or treatment with cyclosporine or both [3]. The authors describe a child with posterior leukoencephalopathy associated with hypertension and cyclosporine therapy, who presented with unusual ocular findings and progressed to irreversible leukomalacia.

Section snippets

Case report

A 2-year-old male with trisomy 21 developed a myelodysplastic syndrome associated with monosomy 7 at 7 months of age that progressed to acute myelogeneous leukemia. He was treated with a high dose of cytosine arabinoside and l-asparaginase, achieved complete remission, and proceeded to an unrelated 1-antigen, mismatched, T-cell-depleted (T10B9+C’) bone marrow transplantation after cytoreduction with hyperfractionated total body irradiation, cytosine arabinoside, and cyclophosphamide.

Discussion

The association of transient bioccipital dysfunction and hypertension or cyclosporine use has been well established. The clinical picture is characterized by an encephalopathy with headache, vomiting, confusion, seizures, and cortical blindness [1]. The neuroimaging studies usually reveal bilateral white matter edema in the posterior regions of the cerebral hemispheres [4]. On discontinuation of the immunosuppressive therapy and institution of antihypertensive medication, neurologic deficits

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