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Intracranial Calcifications with Dandy Walker Malformation

Question:
Sakthi Seethalakshmi P, Kumaravel KS, Sampathkumar P, Babu T, Rani SR
Department of Pediatrics, Government Mohan Kumaramangalam Medical College, Salem, Tamilnadu, India.

Address for Correspondence: Kumaravel KS, 191A, Shankar Nagar, Salem 7, Tamil Nadu - 636007. Email: Kumaravelks@Rediffmail.Com

A male child born of non-consanguineous marriage was admitted with severe microcephaly and seizures. There were no ocular, hematological, abdominal or cardiovascular abnormalities. CT brain showed cerebellar vermian agenesis, Dandy-Walker Malformation (DWM), lissencephaly, agenesis of corpus callosum, intracerebral-periventricular and basal ganglia calcification (Fig1,2). TORCH infection was suspected but the serology for all TORCH components, repeated twice was negative. Maternal TORCH serology was also negative.

What is the diagnosis?