Published in:
01-11-2010 | Clinical image
Megaloencephalic leukoencephalopathy with subcortical cyst formation (van der Knaap disease)
Published in: Pediatric Radiology | Issue 11/2010
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A child born at full term developed macrocephaly in infancy with regression of motor skills and cerebellar ataxia starting at age 3 that progressively worsened. Axial T2 weighted (T2-W) MRI (Fig. 1) shows diffuse white matter hyperintensity involving the subcortical fibers as well as sparing of the basal ganglia as thalami. Subcortical cyst formation is seen in the anterior temporal region on the sagittal T2-W image (Fig. 2, arrow). No contrast enhancement was noted.
Fig. 1
Axial T2-W image
Fig. 2
Sagittal T2-W image
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