Microcephaly with simplified gyral pattern

Case contributed by Ammar Haouimi
Diagnosis probable



Patient Data

Age: 1 month
Gender: Female

The CT scan demonstrates:

  • relatively severe microcephaly with normal appearance of the cranial sutures

  • simplified gyral pattern (the width of the gyri is equal or greater than the depth of the sulci)

  • hypoplastic corpus callosum (fully formed but diffusely thin)

  • reduction of the white matter volume

  • enlarged pericerebral spaces mainly arround the frontal and parietal lobes

  • diffuse hypomyelination corresponding to the patient's young age

  • normal appearance of the posterior fossa structures

Case Discussion

CT features most consistent with microcephaly with simplified gyral pattern, group I.A according to the Classification system for malformations of cortical development.

Microcephaly can be divided into:

  • congenital (primary or true) microcephaly: the brain never forms normally. the cause is genetic and usually associated with simplified gyral pattern with normal cortex thickness

  • acquired (or secondary) microcephaly. usually associated with cerebral cortical malformations (such as lissencephaly or holoprosencephaly) and result from brain injury (such as hypoxic-ischemic insult, cerebral infection, or metabolic disease)

Case courtesy Dr M. Slougui, MD, CHU Constantine, Algeria

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