Polymicrogyria

Shain C, et al. Polymicrogyria-associated epilepsy: a multicenter phenotypic study from the Epilepsy Phenome/Genome Project. Epilepsia. 2013 Aug;54(8):1368-75.

Polymicrogyria (PMG) is an epileptogenic malformation of cortical development. We describe the clinical epilepsy and imaging features of a large cohort with PMG-related epilepsy.

Participants with PMG had both focal and generalized onset of seizures. Our data confirm the involvement of known topographic patterns of PMG and suggest that more extensive distributions of PMG present with an earlier age of seizure onset and increased prevalence of developmental delay prior to seizure onset.

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Barkovich AJ. Current concepts of polymicrogyria. Neuroradiology. 2010 Jun;52(6):479-87.

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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Turkelson SL, Martin C. Management of the child with polymicrogyria. J Neurosci Nurs. 2009 Oct;41(5):251-60.

Polymicrogyria is one of several neuronal migrational defects. This disorder is derived from a malformation in cortical development characterized by many small gyri, shallow sulci, and abnormal cortical layering. The potential causes of polymicrogyria include genetic disorders, intrauterine cytomegalovirus infection, and fetal vascular supply disruption. A wide variation in the extent of findings exists, ranging from minor deficits to profound neurological dysfunction. Seizures, feeding problems, elimination, physical mobility, and psychosocial issues must be managed for children with polymicrogyria. Children with this disorder require collaborative care from healthcare practitioners and parents to achieve their highest level of health. The incidence of neuronal migrational defects is approximately 1 in 2,500 live births (L. Villard et al., 2002). The diagnosis of these disorders has increased with improvement of magnetic resonance imaging (MRI) technology (A. J. Barkovich, R. Hevner, & R. Guerrini, 1999).

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Chang B, et al. Polymicrogyria Overview. 2005 Apr 18 [updated 2007 Aug 6]. In: Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2017.

Polymicrogyria is characterized by stable neurologic deficits, i.e., a “static encephalopathy.” The mildest form, unilateral focal polymicrogyria, may have minimal neurologic manifestations. In more severe forms, focal, motor, sensory, visual, or cognitive problems may be present, depending on the brain region affected. In the most widespread form, bilateral generalized polymicrogyria, severe intellectual disability, cerebral palsy, and refractory epilepsy may be present.

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