The Spectrum of MR Imaging Patterns Suggestive of Pediatric Posterior Reversible Encephalopathy Syndrome in Children With Cerebral X-Linked Adrenoleukodystrophy

Kerem Ozturk, Alexander M McKinney

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish (US)
Number of pages6
JournalJournal of neuroimaging : official journal of the American Society of Neuroimaging
Issue number6
Early online dateAug 8 2020
StatePublished - Nov 2020
Externally publishedYes

Bibliographical note

Funding Information:
and Disclosure: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. This study received no specific grant from any agency in the public, commercial, or nonprofit sectors.

Publisher Copyright:
© 2020 American Society of Neuroimaging

PubMed: MeSH publication types

  • Journal Article

Fingerprint Dive into the research topics of 'The Spectrum of MR Imaging Patterns Suggestive of Pediatric Posterior Reversible Encephalopathy Syndrome in Children With Cerebral X-Linked Adrenoleukodystrophy'. Together they form a unique fingerprint.

Cite this