PRES in Children Undergoing Hematopoietic Stem Cell or Solid Organ Transplantation

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a clinical neuroradiologic entity that is becoming increasingly well known and documented in pediatrics. It is characterized by a variable association of seizures, headache, vomiting, altered mental status, visual disturbances, and seizures, as well as imaging suggesting white-gray matter edema involving the posterior regions of the central nervous system in most cases. The pathophysiology of PRES remains unclear. Although PRES has been associated with a widespread range of clinical conditions, namely infections, adverse drug events, autoimmune diseases, and many others, its onset after hematopoietic stem cell and solid organ transplantation remains the most commonly reported. Historically, PRES has proved to be generally reversible and associated with good clinical outcomes; however, severe complications, sometimes life-threatening, can also occur. Most reported cases of childhood PRES after hematopoietic stem cell or solid organ transplantation have been case reports or series across a broad spectrum of different transplant settings, and no clear consensus exists regarding how best to manage the syndrome. Thus, in this article, we provide a comprehensive review of the pathophysiological, clinical, and diagnostic aspects of PRES in children, with a specific focus on the transplant scenario. Differential diagnoses with other neurologic complications after pediatric transplantation are reviewed, and crucial issues in the management of PRES and the development of future research are ultimately addressed.

Posterior reversible encephalopathy syndrome

“…Etiologies may be manifold; however, a clear correlation between clinical signs and symptoms, lesion site or specific trigger factors has not been observed [2, 5]. PRES has been frequently reported in patients receiving immunosuppressive medication after solid organ, bone marrow or stem cell transplantation [18, 19]. The incidence of PRES after solid organ transplantation is reported to be between 0,4 and 6%, whereas up to 8% of patients after bone marrow transplantation may be affected [18, 20].…”

Central Nervous System Complications in Children Receiving Chemotherapy or Hematopoietic Stem Cell Transplantation

“…In the setting of HSCT, PRES occurs usually within 100 days from transplantation; the use of calcineurin inhibitors for GvHD such as cyclosporine A or tacrolimus has been recognized as a major trigger for PRES. Acute GvHD, hypomagnesemia, the administration of fludarabine during conditioning and the use of umbilical cord blood stem cells are other factors associated with an increased risk of PRES in transplanted children (8). …”

“…Complete clinical recovery is frequently achieved earlier than imaging resolution. Seizures are the most common, and often the presenting, manifestation of PRES (8). They typically manifest as non-convulsive seizures with occipital onset characterized by focal signs, such as gaze deviation, rhythmic ocular movements, and visual symptoms as hallucinations often associated with altered mental status of variable degree.…”

“…A rapid diagnosis of this life-threatening event is fundamental because of the possible need of surgical posterior fossa decompression. Outcome of PRES is usually good, and the reversibility of PRES has been widely described (8, 17); however, non-reversible cases in children with hemato-oncological disorders have been described, with the presence of long-lasting neurological sequelae or epilepsy secondary to residual brain lesions (17). Supportive care is the cornerstone of treatment.…”

Das posteriore reversible Enzephalopathiesyndrom

“…Zahlreiche Publikationen liegen zu zytotoxischer oder -statischer Therapie (v. a. Immunsuppressiva nach Organ-, Knochenmarks-oder Stammzelltransplantation sowie Chemotherapeutika zur Behandlung einer onkologischen Grunderkrankung) vor [23]. Die Inzidenz der Erkrankung wird mit 0,4-6 % nach Organtransplantation und 5-8 % nach Knochenmarkstransplantation angegeben [4,29].…”