Delayed malignant cerebellar posterior reversible encephalopathy syndrome secondary to (CLAG-M) and venetoclax regimen.
Isha Snehal1, Subin Mathew2
1Neurology, 2Neurocritical Care, University of Nebraska Medical Center
Objective:
PRES (posterior reversible encephalopathy syndrome) is a heterogenous syndrome caused by multiple factors. Cerebellar PRES is commonly seen with autoimmune disease and rarely with chemotherapy.
Background:
Isolated cerebellar malignant posterior reversible encephalopathy syndrome (PRES) is rare. We report a case of delayed malignant cerebellar PRES triggered by cladribine, cytarabine, granulocyte colony-stimulating factor, mitoxantrone (CLAG-M) and venetoclax regimen.
Design/Methods:
A 73-year-old male presented with history of relapse after allogeneic stem cell transplantation for refractory myelodysplastic syndrome on maintenance tacrolimus. He was admitted for salvage therapy with (CLAG-M) + venetoclax regimen. Day 16th post salvage therapy he developed acute onset altered mental status and headache followed by fever and hypotension. Brain MRI revealed posterior bilateral cerebellar, vermian edema with tiny petechial hemorrhages, ventriculomegaly, transependymal edema and mass effect on fourth ventricular effacement. Despite external ventricular drain placement, hyperosmolar therapy and steroids, day 19th the patient suffered devastating neurological injury and family opted for hospice care. Of note, maintenance tacrolimus was held prior to admission and was started 4 days prior to onset of neurological symptoms with sub-therapeutic levels.
Conclusions:
The potential role of (CLAG-M) and venetoclax regimen in the pathogenesis of PRES needs to be considered. Cerebellar PRES is rare and can be complicated by early hydrocephalus and 4th ventricle compression. Although PRES is usually benign, prompt recognition of indicators of the malignant phenotype and aggressive management is crucial.