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Current concepts of polymicrogyria.

Barkovich AJ - Neuroradiology (2010)

Bottom Line: Polymicrogyria is one of the most common malformations of cortical development.It has been known for many years and its clinical and MRI manifestations are well described.Recent advances in imaging, however, have revealed that polymicrogyria has many different appearances on MR imaging, suggesting that is may be a more heterogeneous malformation than previously suspected.

View Article: PubMed Central - PubMed

Affiliation: Neuroradiology, University of California at San Francisco, San Francisco, CA 94143-0628, USA. Jim.Barkovich@radiology.ucsf.edu

ABSTRACT
Polymicrogyria is one of the most common malformations of cortical development. It has been known for many years and its clinical and MRI manifestations are well described. Recent advances in imaging, however, have revealed that polymicrogyria has many different appearances on MR imaging, suggesting that is may be a more heterogeneous malformation than previously suspected. The clinical and imaging heterogeneity of polymicrogyria is explored in this review.

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Grade 1 perisylvian PMG. Parasagittal (a) and axial (b) T1-weighted images show coarse polymicrogyria extending from the frontal pole to the occipital pole
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Fig6: Grade 1 perisylvian PMG. Parasagittal (a) and axial (b) T1-weighted images show coarse polymicrogyria extending from the frontal pole to the occipital pole

Mentions: Several syndromes of bilateral symmetrical polymicrogyria have been described; these should be recognized by neuroimagers. The best known and most common of these is bilateral perisylvian polymicrogyria (also called congenital bilateral perisylvian syndrome [44]). This syndrome may be sporadic or familial [15, 45]. The heterogeneous inheritance patterns suggest that mutations of several different genes can cause this malformation [45]; three locations (Xq21.33-q23 (SRPX2), 22q11.2, and Xq28) have been identified [13, 19, 20, 22]. Sporadic cases tend to present with more severe neurologic manifestations. A syndrome of developmental pseudobulbar palsy (oropharyngeal dysfunction and dysarthria, 100%), epilepsy (80–90%), mental retardation (50–80%), and, sometimes, congenital arthrogryposis has been described [44, 46–48]. Other patients are brought to attention in infancy or early childhood because of delayed development (60%), palatal dysfunction (40%), hypotonia (30%), arthrogryposis (30%), or motor deficits (25%) [47, 49]. Seizures are present in 40–60% and may be of many clinical types [47, 49]. Studies of familial cases of congenital bilateral perisylvian polymicrogyria show a lower incidence of these clinical manifestations [45, 50], possibly because patients with minimal symptoms are more readily identified and examined. A developmental reading disorder and reading impairment without severe motor or cognitive handicap may be the cause of seeking medical attention in this group [51]. Bilateral perisylvian polymicrogyria can be graded according to severity on MRI (with grade 1 the most severe and grade 4 the mildest) [50]: Grade 1, with perisylvian polymicrogyria extending to the frontal or occipital pole (Fig. 6); Grade 2, with polymicrogyria extending beyond the perisylvian region but not to either pole (most common, Fig. 7); Grade 3, with polymicrogyria of the perisylvian region only (Fig. 8); and Grade 4, with polymicrogyria restricted to the posterior perisylvian regions.Fig. 6


Current concepts of polymicrogyria.

Barkovich AJ - Neuroradiology (2010)

Grade 1 perisylvian PMG. Parasagittal (a) and axial (b) T1-weighted images show coarse polymicrogyria extending from the frontal pole to the occipital pole
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2872023&req=5

Fig6: Grade 1 perisylvian PMG. Parasagittal (a) and axial (b) T1-weighted images show coarse polymicrogyria extending from the frontal pole to the occipital pole
Mentions: Several syndromes of bilateral symmetrical polymicrogyria have been described; these should be recognized by neuroimagers. The best known and most common of these is bilateral perisylvian polymicrogyria (also called congenital bilateral perisylvian syndrome [44]). This syndrome may be sporadic or familial [15, 45]. The heterogeneous inheritance patterns suggest that mutations of several different genes can cause this malformation [45]; three locations (Xq21.33-q23 (SRPX2), 22q11.2, and Xq28) have been identified [13, 19, 20, 22]. Sporadic cases tend to present with more severe neurologic manifestations. A syndrome of developmental pseudobulbar palsy (oropharyngeal dysfunction and dysarthria, 100%), epilepsy (80–90%), mental retardation (50–80%), and, sometimes, congenital arthrogryposis has been described [44, 46–48]. Other patients are brought to attention in infancy or early childhood because of delayed development (60%), palatal dysfunction (40%), hypotonia (30%), arthrogryposis (30%), or motor deficits (25%) [47, 49]. Seizures are present in 40–60% and may be of many clinical types [47, 49]. Studies of familial cases of congenital bilateral perisylvian polymicrogyria show a lower incidence of these clinical manifestations [45, 50], possibly because patients with minimal symptoms are more readily identified and examined. A developmental reading disorder and reading impairment without severe motor or cognitive handicap may be the cause of seeking medical attention in this group [51]. Bilateral perisylvian polymicrogyria can be graded according to severity on MRI (with grade 1 the most severe and grade 4 the mildest) [50]: Grade 1, with perisylvian polymicrogyria extending to the frontal or occipital pole (Fig. 6); Grade 2, with polymicrogyria extending beyond the perisylvian region but not to either pole (most common, Fig. 7); Grade 3, with polymicrogyria of the perisylvian region only (Fig. 8); and Grade 4, with polymicrogyria restricted to the posterior perisylvian regions.Fig. 6

Bottom Line: Polymicrogyria is one of the most common malformations of cortical development.It has been known for many years and its clinical and MRI manifestations are well described.Recent advances in imaging, however, have revealed that polymicrogyria has many different appearances on MR imaging, suggesting that is may be a more heterogeneous malformation than previously suspected.

View Article: PubMed Central - PubMed

Affiliation: Neuroradiology, University of California at San Francisco, San Francisco, CA 94143-0628, USA. Jim.Barkovich@radiology.ucsf.edu

ABSTRACT
Polymicrogyria is one of the most common malformations of cortical development. It has been known for many years and its clinical and MRI manifestations are well described. Recent advances in imaging, however, have revealed that polymicrogyria has many different appearances on MR imaging, suggesting that is may be a more heterogeneous malformation than previously suspected. The clinical and imaging heterogeneity of polymicrogyria is explored in this review.

Show MeSH
Related in: MedlinePlus