Reversible Posterior Leukoencephalopathy Syndrome

Hinchey, Judith A.

doi:10.1001/archneurol.2007.62

Cited by 233 publications

(420 citation statements)

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“…Reversible posterior leukoencephalopathy syndrome was first described by Hinchey et al 1 in 1996 and is characterized by headaches, visual disturbances, seizures, altered mental status and occasional focal signs. This entity has been reported in a variety of medical conditions including abrupt rise in blood pressure.…”

Section: Discussionmentioning

confidence: 99%

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Reversible posterior leukoencephalopathy in a patient with autonomic dysreflexia: a case report

Chaves

Lee

2008

Spinal Cord

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Study design: Case report. Objectives: To report a case of reversible posterior leukoencephalopathy (RPL) in a patient with traumatic cervical spinal cord injury. Setting: Neurologic inpatient Unit, Lahey Clinic, Burlington, MA, USA. Methods: A 55-year-old woman with a residual spastic quadriparesis from a traumatic C5-C6 fracture developed, during an assisted cough maneuver, sudden severe headache followed by cortical blindness in the setting of high blood pressure. Magnetic resonance imaging (MRI) showed T2 hyperintensities in the subcortical white matter of both medial occipital lobes and left post-central gyrus.Results: Elevation of the head of the bed during subsequent cough maneuvers with occasional use of sublingual nifedipine prevented further episodes of acute arterial hypertension and development of new symptoms. Repeat MRI of the head done one month later was normal. Conclusion: RPL can occur in the setting of autonomic dysreflexia in patients with traumatic cervical cord injury. The prompt recognition of this syndrome is of importance to prevent further morbidity and mortality in patients with spinal cord injury.

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Section: Discussionmentioning

confidence: 99%

“…1 When promptly recognized and treated, symptoms and radiographic abnormalities are usually reversible. Occasionally if left untreated, this condition can lead to strokes and be fatal.…”

Section: Discussionmentioning

confidence: 99%

Reversible posterior leukoencephalopathy in a patient with autonomic dysreflexia: a case report

Chaves

Lee

2008

Spinal Cord

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“…PRES was first described in 1996 by Hinchey et at. 7 . In this landmark paper, 15 patients were described to have a reversible syndrome characterized by headache, altered mental status, seizures, and loss of vision associated with imaging findings compatible with predominantly posterior white matter disease (leukoencephalopathy).…”

Section: Introductionmentioning

confidence: 99%

Hemorrhagic stroke with intraventricular extension in the setting of acute posterior reversible encephalopathy syndrome (PRES): Case report

et al. 2009

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SummaryWe report the case of an eighteen year-old pregnant female with preeclampsia and florid signs and symptoms of posterior reversible encephalopathy syndrome (PRES) in whom intracerebral hemorrhage was evidenced following delivery. Management included blood pressure control, external ventricular drainage and lumboperitoneal shunt. To our knowledge this is the first report of intracranial hemorrhage occurring concurrently with peripartum acute PRES. This case was successfully treated with good outcome upon conclusion of management, thus making awareness of this potentially fatal complication and its suggested management for successful outcome necessary for neurosurgeons, neurologists and intensivists alike.KEY WORDS: Preeclampsia. PRES. Reversible encephalopathy. Intracranial hemorrhage. Intraventricular hemorrhage. External ventriculostomy drainage. Infarto hemorrágico con extensión intraventricular en el contexto de leucoencefalopatía aguda posterior reversible (LPR): descripción de un caso ResumenDescribimos el caso de una mujer embarazada de 18 años con preeclampsia y signos y síntomas floridos de leucoencefalopatía posterior reversible (LPR) en la que se evidenció la presencia de hemorragia cerebral tras el parto. El tratamiento de la enferma incluyó el control de la presión arterial, la utilización de drenaje ventricular externo y la colocación de una válvula lumboperitoneal. En nuestro conocimiento esta es la primera descripción en la literatura de la concurrencia de hemorragia intracraneal con la LPR. Este caso fue satisfactoriamente tratado con un buen resultado, haciendo que la sospecha y el conocimiento de esta posible fatal complicación y su correcto tratamiento sea importante para neurocirujanos, neurólogos e intensivistas.

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“…Hypotheses include cerebral ischemia from vasospasm, disordered cerebral autoregulation and endothelial dysfunction [1,2]. RPLS associated with calcineurin is thought to result from direct toxic injury to vascular endothelium, leading to production of inflammatory cytokines and capillary leakage, which triggers vasogenic cerebral edema [1,3,4]. Hypertension and high serum tacrolimus level are commonly associated with RPLS but there are reported RPLS cases with normal blood pressure and normal serum tacrolimus drug concentrations [5].…”

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confidence: 99%

“…Characteristic clinical manifestations include non-localized headache unresponsive to analgesics, altered mental status, visual disturbances and seizures [1,2]. RPLS is frequently associated with acute hypertension, preeclampsia or eclampsia, sepsis, renal failure, thrombotic thrombocytopenic purpura, hypercalcemia, hypomagnesium, autoimmune diseases, cytotoxic therapies and immunosuppressants [2].…”

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confidence: 99%

Reversible posterior leukoencephalopathy syndrome

Min

Gnann

2013

Intern Emerg Med

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A 40-year-old woman presented with headache, lethargy, dysarthria, diplopia and altered mental status. Her past medical history was significant for cystic fibrosis for which she underwent bilateral lung transplantation 3 years prior to admission. Her medications included tacrolimus and prednisone. On physical examination, she was afebrile and normotensive without neck stiffness; she was intubated and on mechanical ventilation. Her MRI brain with intravenous gadolinium showed symmetric hyperdense signals within the white matter involving parietal (Fig. 1a), occipital ( Fig. 1b) and cerebellar regions (Fig. 1c), without intracerebral space-occupying lesions. Cerebrospinal fluid (CSF) studies revealed clear and colorless fluid with normal opening pressure, cell counts, glucose and protein. Evaluation for infections, including routine blood cultures, CSF Gram's stain and cultures, VDRL, cryptococcal antigen, cytomegalovirus (CMV) polymerase chain reaction (PCR) assay, Epstein-Barr virus (EBV) PCR, human herpes virus-6 (HHV-6) PCR and JC virus PCR, was, however, unrevealing. Serum tacrolimus drug level was 5 ng/ml (normal 5-20 ng/ml). The clinical diagnosis of tacrolimus-associated reversible posterior leukoencephalopathy syndrome (RPLS) was entertained given the negative infectious diseases workup. Tacrolimus was cautiously tapered off over a week, and her neurological condition slowly improved. Repeat MRI brain imaging a week after complete discontinuation of the tacrolimus showed the resolution of the hyperintense lesions (Fig. 1d, e).RPLS, also known as posterior reversible encephalopathy syndrome (PRES), is a clinical syndrome of varying etiologies, but with similar neuroimaging findings. Characteristic clinical manifestations include non-localized headache unresponsive to analgesics, altered mental status, visual disturbances and seizures [1,2]. RPLS is frequently associated with acute hypertension, preeclampsia or eclampsia, sepsis, renal failure, thrombotic thrombocytopenic purpura, hypercalcemia, hypomagnesium, autoimmune diseases, cytotoxic therapies and immunosuppressants [2]. Because of widespread use of calcineurin inhibitors (cyclosporine and tacrolimus) in patients with solid organ transplantation (SOT) to prevent organ rejection, neurotoxicity of these agents has been increasingly reported, although the incidence of RPLS among patients with SOT is low (0.5 %) [3]. The underlying pathophysiology of RPL is poorly defined. Hypotheses include cerebral ischemia from vasospasm, disordered cerebral autoregulation and endothelial dysfunction [1,2]. RPLS associated with calcineurin is thought to result from direct toxic injury to vascular endothelium, leading to production of inflammatory cytokines and capillary leakage, which triggers vasogenic cerebral edema [1,3,4]. Hypertension and high serum tacrolimus level are commonly associated with RPLS but there are reported RPLS cases with normal blood pressure and normal serum tacrolimus drug concentrations [5]. Brain MRI, particularly fluid-attenuated inversion r...

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Reversible Posterior Leukoencephalopathy Syndrome

Cited by 233 publications

References 9 publications

Reversible posterior leukoencephalopathy in a patient with autonomic dysreflexia: a case report

Reversible posterior leukoencephalopathy in a patient with autonomic dysreflexia: a case report

Hemorrhagic stroke with intraventricular extension in the setting of acute posterior reversible encephalopathy syndrome (PRES): Case report

Reversible posterior leukoencephalopathy syndrome

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