Megalencephalic leukoencephalopathy with subcortical cysts

Case contributed by Bhuwan Bhatta
Diagnosis certain

Presentation

Headache

Patient Data

Age: 20 years
Gender: Male

Diffuse areas of hypodensities with finger-like projections were noted in the deep-white matter and subcortical areas involving the bilateral cerebral hemisphere with sparing cerebellar white matter fibers.

Well-defined hypodense areas of near CSF attenuation with thin imperceptible walls are noted in the subcortical region of bilateral anterior frontal and anterior temporal lobes, the largest measuring 8.2 x 2.5 cm in the right anterior frontal lobe.

Macrocephaly (head circumference measures 60.4 cm, i.e., above the 90th percentile for height) with prominent B/L cortical sulci, Sylvian fissures, and basal cisterns.

Persistent cavum septum, cavum pellucidum, and cavum vergae were noted.

Case Discussion

The patient is a known case of megalencephalic leukoencephalopathy with subcortical cysts. He had a positive family history of this disease. A CT head was done for a new-onset headache, which did not show acute findings.

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