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Microlissencephaly (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. More about... 47


Figure 281.2. Typical appearance of patients with microlisencephaly [A,B]. MRI demonstrating lisencephaly and cerebellar dysgenesis [C].

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Behrman, 1996