PRES-like leukoencephalopathy presenting with status epilepticus associated with Brentuximab Vedotin treatment

Alessandro  Orsini; Sayla  Bernasconi; Maria Cristina  Bianchi; Ilaria  Trivelli; Maria Cristina  Menconi; Margherita  Nardi; Andrea  Santangelo; Gabriella  Casazza; Niccolò  Carli; Maria Grazia  Esposito; Diego Peroni; Pasquale Striano; Thomas Foiadelli; Alice Bonuccelli

doi:10.23750/abm.v92iS4.12665

PRES-like leukoencephalopathy presenting with status epilepticus associated with Brentuximab Vedotin treatment

Authors

Alessandro Orsini Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy
Sayla Bernasconi Pediatric Neurology Unit, Dinogmi, Giannina Gaslini’s Istitute, University of Genoa, Italy
Maria Cristina Bianchi Neuroradiology Unit, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy
Ilaria Trivelli Neuroradiology Unit, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy
Maria Cristina Menconi Pediatric Oncohematology Unit, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy
Margherita Nardi Pediatric Oncohematology Unit, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy
Andrea Santangelo Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy
Gabriella Casazza Pediatric Oncohematology Unit, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy
Niccolò Carli Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa
Maria Grazia Esposito Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Pisa, Italy
Diego Peroni Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Pisa, Italy
Pasquale Striano Pediatric Neurology Unit, Dinogmi, Giannina Gaslini’s Istitute, University of Genoa, Italy
Thomas Foiadelli Pediatric Clinic, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia
Alice Bonuccelli Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy

Keywords:

Atypical PRES, Brentuximab-Vedotin, Status Epilepticus, leukoencephalopathy

Abstract

Posterior Reversible Encephalopathy Syndrome (PRES) is characterized by acute neurological symptoms with typical imaging features, primarily in the territories of the brain supplied by the posterior circulation, probably due to vasogenic edema. Both clinical and imaging features are generally reversible. We report a 13-year-old girl affected by Nodular Sclerosis Classical Hodgkin Lymphoma stage IIIB into complete remission, with a recurrence and autologous bone-marrow transplantation, who has been treated with an anti-CD30 monoclonal antibody, brentuximab-vedotin.

The girl has suddenly presented a convulsive status epilepticus, that needed intubation and sedation. Therefore, an IV therapy with levetiracetam was started. Furthermore, the girl has presented high blood pressure and reduced kidney function. Brain MRI demonstrated a diffuse PRES-like disease, that went into regression after the first week. After another week, the girl presented a new prolonged generalized tonic clonic convulsive episode, that needed intubation and sedation and an association of clobazam and levetiracetam: a new brain MRI showed a recurrence of PRES-like lesions in addition to some signs of leukoencephalopathy with brain lactate accumulation on 1H-MRS, due to cerebral energetic failure. The girl also presented a refractory arterial hypertension. After 45 days of ICU hospitalization the patient has been discharged and followed up with neurological examinations. Brain MRI and brain 1H-MRS, 5 months after patient’s discharge, showed incomplete regression of cerebral white matter signal abnormalities with MRS normalization.

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