How is Hemimegalencephaly treated?

How is Hemimegalencephaly treated?

Because the seizures associated with hemimegalencephaly are difficult to treat with anticonvulsant medications, a surgery called hemispherectomy is often the most successful treatment.

What does Megalencephaly mean?

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 Megalencephaly capillary malformation syndrome?

Description. Collapse Section. Megalencephaly-capillary malformation syndrome (MCAP) is a disorder characterized by overgrowth of several tissues in the body. Its primary features are a large brain (megalencephaly) and abnormalities of small blood vessels in the skin called capillaries (capillary malformations).

Is Hemimegalencephaly hereditary?

Total hemimegalencephaly: Includes enlargement of cerebellum and sometimes brain stem and can be isolated or syndromic. Hemimegalencephaly is caused by a spontaneous mutation which occurs on the mTOR pathway at approximately the third week of gestation. It is genetic but is not hereditary.

Can Hemimegalencephaly cause death?

Although there have been a few reports of medical treatment, the main treatment is radical: remove or disconnect the affected side. However, it has a high mortality, and there have been reports of a vegetative state and seizures resuming, this time in the healthy hemisphere.

What is M-CM illness?

Macrocephaly-capillary malformation (M-CM) is a multiple malformation syndrome causing body and head overgrowth and abnormalities of the skin, vascular system, brain and limbs. The disorder has recently (June 2012) been attributed to a genetic mutation in a gene called PIK3CA.

How many cases of Hemimegalencephaly are there?

Hemimegalencephaly (HME) is a rare congenital malformation of the brain characterized by overgrowth of one hemisphere. [1] Its prevalence ranges from 1 to 3 cases/1000 epileptic children and 1–14% among those with cortical developmental abnormalities.

Does polymicrogyria affect life expectancy?

The quality of life and life expectancy for people with BPP are not well-described but may depend on severity of symptoms, whether complications develop, and whether other birth defects or an underlying syndrome are present.

Is the prognosis of megalencephaly the same as autism?

The prognosis of megalencephaly depends heavily on the underlying cause and associated neurological disorders. Because the majority of megalencephaly cases are linked with autism, the prognosis is equivalent to the corresponding condition.

What are the signs and symptoms of megalencephaly?

Symptoms of megalencephaly include delayed development, seizures, and corticospinal (brain cortex and spinal cord) dysfunction. Megalencephaly affects males more often than females. Unilateral megalencephaly or hemimegalencephaly is a rare condition that is characterized by the enlargement of one side of the brain.

Is there a cure or treatment for megalencephaly?

There is no standard treatment for megalencephaly. Treatment will depend upon the disorder with which the megalencephaly is associated and will address individual symptoms and disabilities. There is no standard treatment for megalencephaly.

What is the long-term outlook for megalencephaly?

If you have megalencephaly, your long-term outlook depends on the severity of your condition. In some cases, your symptoms may be very mild. They may require little to no treatment. They may have little impact on your life. In other cases, your symptoms may be severe.