Laminar cortical necrosis in adrenal crisis: sequential changes on MRI

Area of Science:

• Pediatric Neurology
• Neuroradiology
• Endocrinology

Background:

• Congenital adrenal hyperplasia (CAH) is a group of genetic disorders affecting the adrenal glands.
• Adrenal insufficiency in CAH can lead to life-threatening hypoadrenal crises.
• Neurological complications, such as seizures and altered consciousness, can occur during these crises.

Observation:

• Serial magnetic resonance imaging (MRI) in a child with CAH revealed progressive brain abnormalities during a hypoadrenal crisis.
• Initial findings included fronto-parietal cortical edema with diffusion-weighted imaging abnormalities.
• Computed tomography (CT) demonstrated bilateral frontal lobe hypodensities, indicating evolving injury.

Findings:

• Follow-up MRI showed extensive white matter lesions and deep cortical layer involvement.
• Development of superficial cortical lesions suggested laminar cortical necrosis, particularly in the frontal lobes.
• Later imaging revealed multi-layered cortical lesions with cavitary changes, indicating complex ischemic and hypoxic injury.

Implications:

• The unique multi-layered cortical lesions likely result from a combination of hypoglycemia and hypoxia/ischemia secondary to adrenal insufficiency.
• These findings highlight the severe neurological impact of hypoadrenal crises in CAH.
• Understanding these imaging patterns is vital for accurate diagnosis and management of neurological complications in CAH.