Laminar cortical necrosis in adrenal crisis: Sequential changes on MRI

Abstract

We describe the serial magnetic resonance imaging (MRI) findings in a six-year-old girl with congenital adrenal hyperplasia, who presented with seizures and unconsciousness during a hypoadrenal crisis. Initial neuroimaging revealed the presence of brain edema with high signal changes in the fronto-parietal cortex on diffusion-weighted MRI. The brain edema worsened four days into admission, and by day 14 low-density areas were seen over the frontal lobes bilaterally using computed tomography (CT). Follow-up MRI at between one and two months of admission revealed extensive white matter lesions with high intensity on T2-weighted images (T2WI) and fluid-attenuated inversion recovery (FLAIR) images, which extended into deep cortical layers. Additionally, linear lesions with high signal change on T1-weighted imaging developed in the superficial cortical layers, with frontal predominance. This layer appeared isointense on T2WI and high intensity on FLAIR images, suggesting laminar cortical necrosis. Two months later, linear, cavitary lesions appeared in the middle cortical layers between the aforementioned superficial laminar abnormality and deep cortex/white matter lesions. The high-intensity signals in the deep cortical layers remained contiguous with the white matter lesions. This unique type of multi-layered cortical lesion may have resulted from a complex combination of hypoglycemia and hypoxia/ischemia in the setting of adrenal insufficiency.