Microcephaly with a simplified gyral pattern

Case contributed by Dalia Ibrahim

Presentation

Microcephaly.

Patient Data

Age: 7 days
Gender: Male
  • severe microcephaly (evident on the sagittal images)
  • simplified gyral pattern (few and shallow sulci)
  • small thin corpus callosum with absent rostrum
  • rather reduced volume of the white matter with diffuse hypomyelination (the hypomyelination corresponds to the patient's young age)
  • severe microcephaly (evident on the sagittal images)
  • simplified gyral pattern (few and shallow sulci)
  • small thin corpus callosum with absent rostrum
  • rather reduced volume of the white matter with diffuse hypomyelination (the hypomyelination corresponds to the patient's young age)
  • distended dural venous sinuses (normal for age)

Comparison with normal newborn

Photo

Comparison with a normal 2 weeks old newborn.

Note the profound microcephaly and the small corpus callosum at the sagittal image.

Also, the severe simplified gyral pattern seen on the axial images.

Case Discussion

Microcephaly with a Simplified Gyral Pattern (MSG) is a congenital disorder characterized by microcephaly and shallow (reduced number of) gyri.

Lissencephaly is similar to MSG however, in lissencephaly the cortex is thick showing an abnormal architecture, both features are usually absent in MSG.

MSG may show corpus callosum hypogenesis or hypoplasia, hypomyelination, or periventricular nodular heterotopia.

Microcephaly is classified into:

  • primary: microcephaly with normal brain architecture. The cause is likely genetic. In some cases, this primary type may show a simplified gyral pattern without cortex thickening
  • secondary: microcephaly secondary to cerebral cortical malformation (e.g. holoprosencephaly or lissencephaly). The cause is likely secondary to brain insults, such as hypoxic-ischemic insult or infection, or metabolic disorder

 

The case is courtesy of Dr.Mostafa El Khashab, MD of radiodiagnosis

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