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What causes Hemimegalencephaly?

What causes Hemimegalencephaly?

Hemimegalencephaly (HME), or unilateral megalencephaly, is a rare congenital disorder affecting all or a part of a cerebral hemisphere. It causes severe seizures, which are often frequent and hard to control.

What is Megalencephalic?

Megalencephaly, also called macrencephaly, is a condition in which an infant or child has an abnormally large, heavy, and usually malfunctioning brain. By definition, the brain weight is greater than average for the age and gender of the child.

What is a functional hemispherectomy?

A functional hemispherectomy is an evolution of the anatomic hemispherectomy in which less brain is removed, however the diseased hemisphere of the brain is completely disconnected from the normal hemisphere of the brain.

Which side of your brain is bigger?

While the two sides of the brain are roughly equal in size, in most people these parts of the left hemisphere are slightly larger. But like handedness, size discrepancy and language dominance are reversed in a minority; for about 30 percent of lefties, the right hemisphere rules in these regards.

What happens if you remove the right side of your brain?

When the right side of the brain is removed, the left side of the body is affected. Typically, this comes in the form of paralysis of the arm and often weakness in the leg. For children who have had strokes prior to the surgery, many come out as they went in.

What are the traits of a left brained person?

The left brain is more verbal, analytical, and orderly than the right brain. It’s sometimes called the digital brain. It’s better at things like reading, writing, and computations.

Which is the correct description of hemimegalencephaly?

Hemimegalencephaly is a malformation of cortical development, where there is an abnormally large and malformed cerebral hemisphere, with abnormal cortical formation, white matter volume and ventricular morphology. The other hemisphere is usually normal, but may be smaller in size.

What does it mean when an infant has megalencephaly?

Megalencephaly is characterized by an abnormally large, heavy, and poorly functioning brain. The head of an infant affected by megalencephaly is abnormally large in the infant’s early years especially.

How is hemimegalencephaly related to Sotos syndrome?

Hemimegalencephaly may also occur in association with Sotos syndrome and Alexander disease. These syndromes arise as a result of complex genetic activities such as single or multiple gene mutations. The mutations causing these disorders can be inherited or occur randomly during fetal development.

When do seizures in association with HME occur?

Seizures in association with HME often begin in early infant life including an association with infantile spasms. Hemimegalencephaly may occur as an isolated or sporadic brain malformation or it may be associated with other neurodevelopmental syndromes. Thus, when detected, HME should prompt a search for other syndromic diagnoses.