Cyclophosphamide-induced reversible posterior leukoencephalopathy syndrome

Abstract

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical radiological syndrome, characterised by acute headache, altered consciousness, seizures and hypertension. The most frequent causes are hypertensive encephalopathy, eclampsia and some immunosuppressive therapies. The pathogenesis remains unclear, but it appears to be related to altered cerebral circulation, producing oedema that can be seen on MRI, and it resolves in 2 or 3 weeks. In the present report, a possible first reported case of cyclophosphamide-induced RPLS in a 27-year-old man with high blood pressure (HBP) and glomerulonephritis caused by Goodpasture syndrome, treated with cyclophosphamide during the last month and prednisone for glomerulonephritis resulting from Goodpasture syndrome without other immunosuppressive drugs, is described.Symptoms appeared during a hypertensive crisis, but when cyclophosphamide was replaced by rituximab and hypertension was controlled, the patient did not have neurological symptoms. Almost all reported cases induced by immunosuppressive therapy or other causes were associated with hypertension as well.

Cyclophosphamide-induced posterior reversible encephalopathy syndrome (PRES): a case report

“…In these cases, breakdown of the blood–brain barrier due to endothelial dysfunction caused by certain drugs or underlying conditions such as fluid overload or renal insufficiency have been postulated [ 3 ]. Abnormal endothelial activation, dysfunction and leukocyte tracking have recently been reported to cause cerebral and systemic hypoperfusion, which may be causative factors for PRES in SLE [ 6 , 10 ]. In general, patients with SLE who developed PRES had a high disease activity index at the time of its occurrence [ 2 , 5 , 11 ].…”

“…Cyclophosphamide use has been reported in several cases of PRES, but mostly in combination with other cytotoxic agents for treatment of haematological malignancies [ 2 , 12 ]. Furthermore, PRES attributed to cyclophosphamide is confounded by the presence of fluid overload, hypertension, and/or renal failure, all of which could contribute to endothelial dysfunction [ 2 , 6 ]. In contrast, our patient did not have any such inciting factors apart from cyclophosphamide therapy, thus making it the most likely causative factor.…”

“…Cyclophosphamide is an alkylating agent that is widely used in the treatment of selected malignant processes and autoimmune diseases. However, reports of cyclophosphamide-induced PRES are rare [ 5 , 6 ]. We report the case of a 33-year-old normotensive patient with lupus nephritis who developed PRES following intravenous cyclophosphamide therapy.…”

Successful treatment of a Caucasian case of multifocal Castleman’s disease with TAFRO syndrome with a pathophysiology targeted therapy - a case report

“…Moreover this case points out another rare condition, that is Posterior Reversible Encephalopathy Syndrome (PRES) [ 22 , 23 ], a poorly understood and described clinical-radiological syndrome whose pathogenesis has been ascribed to altered cerebral circulation and endothelial dysfunction. Many immunosuppressive drugs, such as intravenous immunoglobulin, ciclosporin A, tacrolimus, interferon α and, as recently reported, cyclophosphamide, may be responsible for this syndrome.…”

Posterior reversible encephalopathy syndrome revealing renal artery stenosis in a child

“…Neuro-imaging studies show abnormalities predominating in the parieto-occipital lobes 1. Hypertension is the most frequent cause and the main conditions associated with PRES are haematological and auto-immune diseases, haemolytic–uremic syndrome, chronic renal failure, hepatitis C, HIV infection, blood transfusion and immunosuppressive drug therapy 2 3…”