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Subsequently, the head fails to grow, while the face continues to develop at a normal rate, producing a child with a small head and a receding forehead, and a loose, often wrinkled scalp.

As the child grows older, the smallness of the skull becomes more obvious, although the entire body also is often underweight and dwarfed.

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A homozygous mutation in one of the microcephalin genes causes primary microcephaly.

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