Presentation
Cognition deterioration for 2 months.
Patient Data
Bilateral symmetric abnormal T2/FLAIR subcortical and deep white matter hyperintensities centered on the posterior and inferior cerebrum (parietal, temporal, occipital lobes), and involving the corpus callosum (posterior body and splenium), showing thin peripheral "leading edge" enhancement. Frontal involvement is not seen.
Bilateral T2 high signal also follows white matter tracks in the thalami, posterior margins of the internal capsules, and brainstem (midbrain, pons, and medulla), some along the corticospinal tracts.
The involved cerebral areas show trizonal distribution.
The signal intensity of the cerebellum is within normal limits.
Case Discussion
The features are typical for x-linked adreno-leukodystrophy.
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