Adrenoleukodystrophy: early frontal lobe involvement on computed tomography.

Area of Science:

• Neurology
• Radiology
• Genetics

Background:

• Adrenoleukodystrophy (ALD) is a rare genetic disorder affecting the adrenal glands and white matter of the brain.
• ALD is characterized by progressive demyelination, leading to neurological dysfunction.
• Early diagnosis and understanding of imaging findings are crucial for managing ALD.

Observation:

• A teenage boy presented with subacute onset of dementia and behavioral changes.
• Computed tomography (CT) scans showed bilateral, isolated, symmetric low-density lesions in the frontal lobes.
• No contrast enhancement was observed in these lesions, with minimal calcification noted.

Findings:

• The CT findings demonstrated an unusual pattern of early frontal lobe demyelination in a patient with ALD.
• The isolated, symmetric, and non-enhancing nature of the lesions is a key characteristic.
• This presentation differs from typical ALD imaging patterns, which often involve posterior white matter.

Implications:

• Recognizing this atypical CT presentation can aid in the early diagnosis of ALD.
• Understanding these specific imaging findings is important for neurologists and radiologists.
• This case contributes to the literature on the diverse neuroimaging manifestations of ALD.