(microbrain or lissencephaly type IV) results from severe cerebral and
cerebellar hypoplasia. Microcephaly is severe. These patients usually
die during the neonatal period. Craniofacial dysmorphism, genitalia anomalies,
and arthrogryposis may occur. Microlissencephaly results from an abnormal
neuronal and glial proliferation. An autosomal recessive inheritance has
been suggested. The diagnosis is established by MRI of the brain. The
MRI of the brain shows a smooth cerebral surface, agenesis of the corpus
callosum, and cerebellar dysgenesis.
Typical appearance of patients with microlisencephaly [A,B]. MRI demonstrating
lisencephaly and cerebellar dysgenesis [C].