Polymicrogyria

Infobox_Disease
Name = PAGENAME


Caption =
DiseasesDB = 33975
ICD10 =
ICD9 = ICD9|742.2
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID =

Polymicrogyria (PMG) 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 may suffer from a wide spectrum of other problems, including: global developmental disabilities, mild to severe mental retardation, motor dysfunctions including speech and swallowing problems, respiratory problems, and seizures. Each child is unique in their presentation of the disorder, thus 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 and 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.

ee also

* Bilateral frontoparietal polymicrogyria (genetic lesion)
* Augmentative Alternative Communication
* Epilepsy Phenome/Genome Project

Further reading

* "Schuyler's Monster: A Father's Journey with His Wordless Daughter", Robert Rummel-Hudson, St. Martin's Press, 2008 (ISBN 978-0312372422)