Case ReportsPosterior leukoencephalopathy syndrome may not be reversible
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
References (8)
- et al.
Occipital-parietal encephalopathyA new name for an old syndrome
Pediatr Neurol
(1997) - et al.
Pre-eclampsiaMore than pregnancy-induced hypertension
Lancet
(1993) - et al.
A reversible posterior leukoencephalopathy syndrome
N Engl J Med
(1996) - et al.
Neurologic complications in allogeneic bone marrow transplant patients receiving cyclosporin
Bone Marrow Transplant
(1991)
Cited by (159)
Diffusion-weighted MRI in paediatric neuroimaging
2018, Clinical RadiologyCitation Excerpt :The diffusion abnormality normally resolves once the underlying cause is controlled but occasionally may be complicated by acute ischaemia with resultant cytotoxic oedema. The presence of cytotoxic oedema is associated with a worse neurological outcome.17 DWI complements conventional imaging in the evaluation of perinatal brain injury, as diffusion abnormalities better illustrate the extent of perinatal brain injury when compared with conventional MRI,18 particularly when performed between the second and fourth days of life (Fig 9).
Posterior Reversible Encephalopathy Syndrome Associated With Dose-adjusted EPOCH (Etoposide, Prednisone, Vincristine, Cyclophosphamide, Doxorubicin) Chemotherapy
2017, Clinical Lymphoma, Myeloma and LeukemiaOculogyric crises: Etiology, pathophysiology and therapeutic approaches
2017, Parkinsonism and Related DisordersReversible posterior leukoencephalopathy syndrome in a patient presenting granulomatosis with polyangiitis
2017, Annales de Dermatologie et de VenereologieManagement and outcomes of malignant posterior reversible encephalopathy syndrome
2014, Clinical Neurology and NeurosurgeryCitation Excerpt :Although the initial description of PRES stated that ‘clinical signs and abnormalities on imaging are always reversible [8], further experience has tempered this optimism. In fact, some authors observed that PRES is often neither posterior nor reversible [14–17]. Outcomes following PRES are variable and fatalities reach 30% with hemorrhagic PRES [4,6].