Abstract

Background:Posterior reversible encephalopathy syndrome (PRES) is a disorder of reversible subcortical vasogenic brain edema with acute neurological symptoms in patients of renal failure, hypertension and on cytotoxic drugs. Established diagnostic criteria have been lacking and diagnosis can be made on exclusion based on clinical criteria, serum markers, CSFand imaging. The treatment is symptomatic depending on underlying condition. The overall prognosis is favourable but some patients may have long‐term sequale.Aims:To study the clinico‐radiologic profile and outcomes of PRES in hematological disorders.Methods:We retrospectively reviewed clinical and neuroimaging findings of patients diagnosed with PRES from January 2016 ‐ October 2018. PRES was diagnosed based on clinical signs and MRI findings.Results:There were total of 16 patients diagnosed with PRES. There were 7 females and 9 males with age ranging from 2–25 years. Maximum (12/16) patients were ALL on induction chemotherapy (BFM protocol), rest were AML on consolidation high dose cytarabine (HiDAC), thalassemia post HSCT and aplastic anaemia. None of the patients had prior history of seizures, hypertension or evidence of CNS disease. Most patients had hypertension and generalised tonic clonic seizures (GTCS) as presenting complaints. CSF examination done in all was inconclusive. The most common MRI finding (12/16) was bilateral symmetrical subcortical white matter hyperintensity on T2 and FLAIR images in occipital and parietal lobes. Others had frontal lobe, temporal lobe, thalamus and cerebellar involvement. For management cytotoxic drugs werestopped, antihypertensive (amlodipine, labetalol) and anti‐epileptics were started. Most patients experienced normalisation of blood pressure in an average of 8.6 days (range 4–15 days). Two patients expired during the course due to fungal pneumonia and intra‐cerebral haemorrhage respectively. Patients were continued on antiepileptics for 6–12 months after last seizure episode. Antihypertensives were used for period of one month and then tapered off successfully. Most patients (14/16) were discharged and are under our follow up with no active PRES related complications except one patient who has persistence of neurological deficit in the form of right sided hemiparesis.Summary/Conclusion:Amongst patients with hematological malignancy, PRES is especially seen in ALL where steroids are the backbone of induction chemotherapy. Close blood pressure monitoring in settings of induction chemotherapy and immunosuppressive drugs like calcineurin inhibitors should always be prescribed. Early recognition of PRES may facilitate appropriate treatment in a timely manner, conferring a better prognosis. Future studies should be done to address questions like duration of interruption of drug implicated in PRES, if the implicating drug has to be eliminated permanently, optimal duration of anti‐hypertensives and anti‐epileptics.image

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