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Polymicrogyria
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Polymicrogyria
Polymicrogyria (PMG) is a condition that affects the development of the human brain by multiple small gyri (microgyri) creating excessive folding of the brain leading to an abnormally thick cortex. This abnormality can affect either one region of the brain or multiple regions.
The time of onset has yet to be identified; however, it has been found to occur before birth in either the earlier or later stages of brain development. Early stages include impaired proliferation and migration of neuroblasts, while later stages show disordered post-migration development.
The symptoms experienced differ depending on what part of the brain is affected. There is no specific treatment to get rid of this condition, but there are medications that can control the symptoms such as seizures, delayed development or weakened muscles as some of the noted effects.
Significant technological advances have been made within the past few decades that have allowed more extensive studies to be made regarding syndromes from conditions such as polymicrogyria. Research, imaging, and analysis has shown that distribution of polymicrogyria does not always appear to be random, which revealed different types polymicrogyria. A summary of clinical manifestations of each syndrome can be found below, in the section labelled "Clinical presentation".[citation needed]
The main patterns of polymicrogyria are: perisylvian (61%), generalised (13%), frontal (5%), and parasagittal parieto-occipital (3%) and 11% is associated with gray matter heterotopia (grey matter is located in the white matter instead of usual location in the cerebral cortex).
BFP appears to be a symmetrical polymicrogyria that extends anteriorly from the frontal poles to the posterior precentral gyrus, and inferiorly to the frontal operculum. Patients who had polymicrogyria distribution similar to this also experienced similar symptoms including delayed motor and language developments, spastic hemiparesis or quadriparesis, and forms of mild intellectual disability.[citation needed]
BFPP was one of the first discovered forms of polymicrogyria to have a gene identified linking to the syndromes caused. This gene is called GPR56. Symmetrical distribution is also evident in this form, but more distinctly, patients with BFPP were found to have atrophy of the cerebellum and brain stem, as well as bilateral white matter abnormalities. BFPP is characterized by esotropia, global development delay, pyramidal signs, cerebral signs, and seizures. Esotropia is also known as dysconjugate gaze, and is a common feature of severe static encephalopathy. This differentiates BFPP from the other bilateral polymicrogyria syndromes.[citation needed]
BPP is similar to the other types of polymicrogyria in that it is usually symmetrical, but BPP can vary among patients. BPP is characterized by its location; the cerebral cortex deep in the sylvian fissures is thickened and abnormally infolded, as well as the sylvian fissures extending more posteriorly up to the parietal lobes and more vertically oriented. BPP has been classified into a grading system consisting of four different grades that describe the variations in severity:[citation needed]
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Polymicrogyria
Polymicrogyria (PMG) is a condition that affects the development of the human brain by multiple small gyri (microgyri) creating excessive folding of the brain leading to an abnormally thick cortex. This abnormality can affect either one region of the brain or multiple regions.
The time of onset has yet to be identified; however, it has been found to occur before birth in either the earlier or later stages of brain development. Early stages include impaired proliferation and migration of neuroblasts, while later stages show disordered post-migration development.
The symptoms experienced differ depending on what part of the brain is affected. There is no specific treatment to get rid of this condition, but there are medications that can control the symptoms such as seizures, delayed development or weakened muscles as some of the noted effects.
Significant technological advances have been made within the past few decades that have allowed more extensive studies to be made regarding syndromes from conditions such as polymicrogyria. Research, imaging, and analysis has shown that distribution of polymicrogyria does not always appear to be random, which revealed different types polymicrogyria. A summary of clinical manifestations of each syndrome can be found below, in the section labelled "Clinical presentation".[citation needed]
The main patterns of polymicrogyria are: perisylvian (61%), generalised (13%), frontal (5%), and parasagittal parieto-occipital (3%) and 11% is associated with gray matter heterotopia (grey matter is located in the white matter instead of usual location in the cerebral cortex).
BFP appears to be a symmetrical polymicrogyria that extends anteriorly from the frontal poles to the posterior precentral gyrus, and inferiorly to the frontal operculum. Patients who had polymicrogyria distribution similar to this also experienced similar symptoms including delayed motor and language developments, spastic hemiparesis or quadriparesis, and forms of mild intellectual disability.[citation needed]
BFPP was one of the first discovered forms of polymicrogyria to have a gene identified linking to the syndromes caused. This gene is called GPR56. Symmetrical distribution is also evident in this form, but more distinctly, patients with BFPP were found to have atrophy of the cerebellum and brain stem, as well as bilateral white matter abnormalities. BFPP is characterized by esotropia, global development delay, pyramidal signs, cerebral signs, and seizures. Esotropia is also known as dysconjugate gaze, and is a common feature of severe static encephalopathy. This differentiates BFPP from the other bilateral polymicrogyria syndromes.[citation needed]
BPP is similar to the other types of polymicrogyria in that it is usually symmetrical, but BPP can vary among patients. BPP is characterized by its location; the cerebral cortex deep in the sylvian fissures is thickened and abnormally infolded, as well as the sylvian fissures extending more posteriorly up to the parietal lobes and more vertically oriented. BPP has been classified into a grading system consisting of four different grades that describe the variations in severity:[citation needed]
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