What is periventricular GREY matter?
What is periventricular GREY matter?
Periventricular nodular heterotopia (PVNH), also known as subependymal grey matter heterotopia, is a brain malformation of cortical development. PVNH is characterized by an abnormal clumping of grey matter (nerve cells) around the deep fluid chambers inside the brain called the ventricles.
Is GREY matter Heterotopia hereditary?
Subcortical Band Heterotopia SBH cortical malformations always have a genetic origin, and abnormalities in the DCX and LIS1 genes account for the majority of the SBH cases.
Does gray matter heterotopia cause seizures?
Clinical context Developmental, cognitive and motor impairments can occur, and are more severe in those with bilateral or extensive heterotopia or other brain abnormalities. Seizures are common, seen in 80-90% of cases. There is a female predominance in cases of bilateral periventricular nodular heterotopia.
What is subependymal GREY matter Heterotopia?
Subependymal heterotopia consists of small foci of gray matter that are located in a subependymal location in close proximity to the ventricular wall. Subcortical heterotopia occurs as masses of gray matter within the deep and subcortical white matter.
Are Heterotopias bad?
At the other extreme, heterotopia can result in severe seizure disorder, loss of motor skills, and mental retardation. Fatalities are practically unknown, other than the death of unborn male fetuses with a specific genetic defect.
Can heterotopia cause seizures?
Periventricular heterotopia is a condition in which the nerve cells (neurons) do not migrate properly during early development of the fetal brain. People with this condition typically develop recurrent seizures ( epilepsy ) beginning in mid-adolescence.
What is periventricular region?
The periventricular area is located deep within the brain, and therefore is very susceptible to reduced blood flow in the small blood vessels that originate at the surface of the brain.
Can heterotopia cause headaches?
Epilepsy in periventricular nodular heterotopia is common but headache is rare [3] . Asymmetric ventricu- lomegaly is a common radiological feature and is thought to be a structural consequence of the presence of hetero- topia, rather than a disorder of ependymal compliance or CSF dynamics [4] .
What causes heterotopic gray matter?
It is believed that gray matter heterotopia are caused by arrested migration of neurons to the cerebral cortex; that is, when neurons that are supposed to form part of the cerebral cortex. fail to climb to the end of their ladder correctly and are permanently situated in the wrong location.
What causes periventricular heterotopia?
In most cases, periventricular heterotopia is caused by mutations in the FLNA gene. This gene provides instructions for producing the protein filamin A, which helps build the network of protein filaments (cytoskeleton ) that gives structure to cells and allows them to change shape and move.
Where is periventricular white matter?
Periventricular white matter refers to white matter located immediately adjacent to the fluid-filled ventricles of the brain.
Where is periventricular located?
What happens if the periventricular white matter is damaged?
The periventricular white matter that surrounds two horseshoe shaped cavities in the brain is primarily responsible for the transmission of nerve impulses that control motor function. Damage in this area can result in spasticity and intellectual impairment.
What is the difference between periventricular heterotopia and PNH?
Periventricular heterotopia is easily diagnosed as normal-appearing gray matter in the form of nodules or short bands in an ectopic, periventricular position; PNH, most often bilateral, consists of nodules of gray matter located along the lateral ventricles with a total failure of migration of some neurons.
What is Periventricular leukomalacia (PVL)?
Periventricular Leukomalacia, or PVL. When a person suffers a periventricular leukomalacia injury, these functions are impaired. PVL is a strikingly common causal factor among children with Cerebral Palsy that leads to intellectual impairment and spasticity that require therapy and treatment.
What is the difference between gray matter and white matter?
Gray matter has neural cell bodies, which can initiate nerve impulses, while white matter transports impulses between gray matter cells. The periventricular white matter that surrounds two horseshoe shaped cavities in the brain is primarily responsible for the transmission of nerve impulses that control motor function.
