What is bilateral Perisylvian polymicrogyria?
Overview. Bilateral perisylvian polymicrogyria (BPP) is a rare neurological disorder that affects the cerebral cortex (the outer surface of the brain). BPP is a subtype of a broader condition known as polymicrogyria. The cerebral cortex of the brain normally consists of several deep folds and grooves.
Does polymicrogyria affect life expectancy?
The quality of life and life expectancy for people with bilateral perisylvian polymicrogyria 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 12).
How common is bilateral polymicrogyria?
The prevalence of isolated polymicrogyria is unknown. Researchers believe that it may be relatively common overall, although the individual forms of the disorder (such as bilateral generalized polymicrogyria) are probably rare.
Is polymicrogyria a disability?
Polymicrogyria that involves one entire hemisphere (side of the brain) is usually associated focal seizures affecting the opposite side of the body that begin in early to mid-childhood, weakness and movement problems on that side of the body, and mild to moderate intellectual disability.
What does Perisylvian mean?
perisylvian language zone the area of the brain that is responsible for language. It consists of the region around the lateral sulcus (Sylvian fissure) of the left hemisphere and includes Broca’s area and Wernicke’s area.
What is congenital bilateral Perisylvian syndrome?
Congenital bilateral perisylvian syndrome (CBPS) is an extremely rare, late migration disorder of the brain characterized by pseudobulbar palsy, mental retardation, epilepsy and bilateral perisylvian polymicrogyria. This syndrome was originally described by Graff-Radford et al. in identical twins.
Can polymicrogyria be detected before birth?
Abstract. We report a rare case of polymicrogyria diagnosed at 27 weeks’ gestation on ultrasound examination and associated with cytomegalovirus (CMV) infection. The ultrasound finding suggesting this diagnosis was the direct visibility of the overfolded cortical ribbon.
How many people in the world have polymicrogyria?
The incidences of PMG and its different forms are unknown. However, the frequency of cortical dysplasia in general has been estimated to be 1 in 2,500 newborns. PMG is one of the best-known and most common malformations of cortical development, accounting for 20% of all cases.
What is the Perisylvian region of the brain?
the area of the brain that is responsible for language. It consists of the region around the lateral sulcus (Sylvian fissure) of the left hemisphere and includes Broca’s area and Wernicke’s area. Damage to the zone causes various forms of aphasia.
What causes Perisylvian syndrome?
Associated symptoms and findings are thought to be due to improper development of the outer surface of the brain (cerebral cortex) during embryonic growth (neuronal dysmigration). In most cases, the disorder appears to occur randomly for unknown reasons (sporadically).
Can polymicrogyria be seen on ultrasound?
What is bilateral frontoparietal Polymicrogyria?
Bilateral frontoparietal polymicrogyria (BFPP) is a rare neurological disorder that affects the cerebral cortex (the outer surface of the brain). BFPP specifically affects the frontal and parietal lobes on both sides of the brain (bilateral).
Can Polymicrogyria be detected before birth?
How many cases of polymicrogyria are there?
What is bilateral perisylvian polymicrogyria (BPP)?
Bilateral perisylvian polymicrogyria (BPP) is a rare neurological disorder that affects the cerebral cortex (the outer surface of the brain). BPP is a subtype of a broader condition known as polymicrogyria.
What is congenital bilateral perisylvian syndrome?
Congenital bilateral perisylvian syndrome. Congenital bilateral perisylvian syndrome ( CBPS) is a rare neurological disease characterized by paralysis of certain facial muscles and epileptic seizures .
What are the symptoms of bilateral perisylvian PMG?
Bilateral perisylvian PMG (BPP) often results in a typical clinical syndrome that is manifested by mild mental retardation, epilepsy, and pseudobulbar palsy, which causes difficulties with expressive speech and feeding (Kuzniecky et al., 1993).
What is polymicrogyria in psychology?
Polymicrogyria (PMG) is a malformation of cortical development in which the brain surface is irregular and the normal gyral pattern replaced by multiple small, partly fused gyri separated by shallow sulci. Microscopic examination shows a simplified 4-layered or unlayered cortex.