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The Spectrum of MR Imaging Patterns Suggestive of Pediatric Posterior Reversible Encephalopathy Syndrome in Children With Cerebral X‐Linked Adrenoleukodystrophy
Author(s) -
Ozturk Kerem,
McKinney Alexander M.
Publication year - 2020
Publication title -
journal of neuroimaging
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.822
H-Index - 64
eISSN - 1552-6569
pISSN - 1051-2284
DOI - 10.1111/jon.12767
Subject(s) - medicine , posterior reversible encephalopathy syndrome , fluid attenuated inversion recovery , adrenoleukodystrophy , magnetic resonance imaging , neuroimaging , white matter , radiology , pediatrics , psychiatry , peroxisome , receptor
ABSTRACT BACKGROUND AND PURPOSE Children receiving chemotherapy, or immunosuppression have an increased risk for pediatric posterior reversible encephalopathy syndrome (pPRES); pPRES is scantly described in cerebral X‐linked adrenoleukodystrophy (cALD) patients, for which hematopoietic stem cell transplantation improves outcomes. This study aimed to describe distinctive lesion patterns, distribution, and evolution of neuroimaging findings in PRES in a single‐center pediatric cohort of cALD. METHODS We retrospectively identified all clinically acquired brain MRIs of children with cALD at a tertiary care university hospital between 1995 and 2020. We reviewed clinical features, conventional MRI, and diffusion‐weighted imaging findings of patients with gray matter and white matter (WM) changes suggestive of concurrent PRES‐cALD. Associations between the distinctive anatomic features, distribution, and abnormal signal intensity on MRI were examined with regard to the etiology and clinical outcome. RESULTS Our search revealed a series of eight pediatric cALD patients presenting with seizures, headache, or altered mental status with MRI findings suggestive of both PRES and cALD simultaneously. In each, the cortical‐subcortical vasogenic edema on fluid‐attenuated inversion recovery was consistent with pPRES, overlying the periventricular WM (PVWM) involvement typical of cALD. Of these 8 patients, the cortical‐subcortical lesions on FLAIR were completely reversible on follow‐up MRI in 7, but only partially reversible in 1. CONCLUSIONS It is crucial to recognize that pPRES can occur in cALD, notably, the cortical edema and leptomeningeal enhancement can accelerate the diagnosis of superimposed pPRES, while the PVWM lesions of cALD remain following the resolution of pPRES.

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