Multiple choice

Extensive involvement of deep white matter, with hyperintense thalamic lesion on MRI of the brain is seen in which one of the following?

  1. Alexander's disease

  2. Krabbe's ds.

  3. Canavan's ds

  4. Metachromatic leucodystrophy
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A Correct answer
Explanation

Alexander's disease (a leukodystrophy caused by GFAP mutations) classically presents with extensive white matter involvement and characteristic frontal-predominant changes, with MRI showing hyperintense lesions that commonly involve the thalami and basal ganglia. The pattern of extensive deep white matter hyperintensity with thalamic involvement is highly suggestive of Alexander disease, differentiating it from other leukodystrophies which have different distribution patterns.

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