Polymicrogyria
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Polymicrogyria is a developmental malformation of the human brain characterized by an excessive number of small convolutions (gyri) on the surface of the brain. Either the whole surface or parts of the surface can be affected.
Clinical presentation
The diagnosis of PMG is merely descriptive and is not a disease in itself, nor does it describe the underlying etiology or cause of the brain malformation.
Polymicrogyria may in fact be just one piece of a syndrome of developmental abnormalities, because children born with it suffer from a wide spectrum of other problems, including global developmental disabilities, mild to severe mental retardation, and motor disfunctions including speech and swallowing problems, respiratory problems, and seizures. In fact, each child is unique in their presentation of the disorder. As a result, it is difficult to make a predictable prognosis for children with the diagnosis of PMG.
Diagnosis
With increased use of imaging techniques such as MRI or CT, polymicrogyria is becoming more widely diagnosed. However, polymicrogyria is often misdiagnosed as pachygyria or lissencephaly, even by experienced radiologists, since the differences between these conditions can be difficult to see on an MRI or CT scan.
Pathology
Polymicrogyria is a disorder of neuronal migration resulting in structurally abnormal cerebral hemispheres. The Latin roots of the name describe its salient feature: many [poly] small [micro] gyri (convolutions in the surface of the brain). It is also characterized by shallow sulci, a slightly thicker cortex, neuronal heterotopia and enlarged ventricles. When many of these small folds are packed tightly together, PMG may resemble pachygyria (a few "thick folds" - a mild form of lissencephaly).
Etiology
Causes are genetic, viral, or due to nutritional deficits during gestation.
See also
- Bilateral frontoparietal polymicrogyria (genetic lesion)