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The surface of the brain normally has many ridges or folds, called gyri. In people with polymicrogyria, the brain develops too many folds, and the folds are unusually small. The name of this condition literally means too many (poly-) small (micro-) folds (-gyria) in the surface of the brain.","mondoId":"MONDO:0000087","records":[{"frequencyKind":"reported","key":"00333923192798976a871ad4af5b153b55f788b893fef8b1e6739c9cb2a1a310","raw":{"aspect":"P","body_system":"head_and_neck","disease_id":"OMIM:300388","disease_name":"Polymicrogyria, bilateral perisylvian, X-linked","evidence":"TAS","frequency_raw":null,"hpo_id":"HP:0007024","is_excluded":false,"label":"Pseudobulbar paralysis","onset_id":null,"reference":"OMIM:300388","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"range","key":"008cd9e2718a57cd7596baabf3731789f5308f89d807e057e8e05a66485166b6","raw":{"aspect":"P","body_system":"nervous_system","disease_id":"ORPHA:101070","disease_name":"Bilateral frontoparietal polymicrogyria","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0001320","is_excluded":false,"label":"Cerebellar vermis 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polymicrogyria","evidence":"TAS","frequency_raw":"HP:0040281","hpo_id":"HP:0002194","is_excluded":false,"label":"Delayed gross motor development","onset_id":null,"reference":"ORPHA:208447","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"count","key":"fff2caff99e32909d3ea812a1d2de46b1dc3f7d73f7ab3748832c437d0d06de0","raw":{"aspect":"P","body_system":"nervous_system","disease_id":"OMIM:610031","disease_name":"Cortical dysplasia, complex, with other brain malformations 7","evidence":"PCS","frequency_raw":"2/5","hpo_id":"HP:0001320","is_excluded":false,"label":"Cerebellar vermis hypoplasia","onset_id":null,"reference":"PMID:19465910","source_release":"2026-06-23"},"sourceGroups":["published"]}],"referenceLabels":{},"references":{"causeHtml":"<ul><li><a href=\"https://medlineplus.gov/genetics/condition/polymicrogyria\">Polymicrogyria</a></li></ul>","identityNote":"MONDO cross-references identify native condition entries. Ancestor membership does not transfer phenotype values between groups.","importantHtml":""},"sections":[{"bodyHtml":"<p>In most people with polymicrogyria, the cause of the condition is unknown. However, researchers have identified several environmental and genetic factors that may contribute to the development of this disorder.</p><p>Environmental causes of polymicrogyria include certain infections during pregnancy, such as cytomegalovirus and Zika virus infections. A lack of oxygen to the developing fetus, which can occur when the placenta is not working properly (placental insufficiency), may also increase the risk of developing polymicrogyria. Having twins may reduce blood flow to the developing brain, which may also increase the risk of polymicrogyria.</p><p>Researchers are investigating the various genetic causes of polymicrogyria. The condition can result from deletions or rearrangements of genetic material from several different chromosomes. Additionally, changes in one of over 50 genes can cause either isolated polymicrogyria or polymicrogyria that occurs as part of a syndrome. Genetic changes that cause disease are called pathogenic variants.</p><p>Pathogenic variants in one gene, ADGRG1 (also known as GPR56), have been found to cause a severe form of the condition called bilateral frontoparietal polymicrogyria (BFPP). The ADGRG1 gene appears to be critical for normal brain development. The ADGRG1 gene variants that cause BFPP likely disrupt the normal movement of brain cells early in development. As a result, the brain forms too many small folds.</p><p class=\"section-source\"><a href=\"https://medlineplus.gov/genetics/condition/polymicrogyria/#causes\">MedlinePlus Genetics: Causes</a></p>","key":"forms","title":"What causes Polymicrogyria?"},{"bodyHtml":"<p>Polymicrogyria can affect part of the brain or the whole brain. When the condition affects one side of the brain, researchers describe it as unilateral. When it affects both sides of the brain, it is described as bilateral. The signs and symptoms associated with polymicrogyria depend on how much of the brain, and which particular brain regions, are affected.</p><p>Researchers have identified multiple forms of polymicrogyria. The mildest form is known as unilateral focal polymicrogyria. This form of the condition affects a relatively small area on one side of the brain. It may cause minor neurological problems, such as mild seizures that can be easily controlled with medication. Some people with unilateral focal polymicrogyria do not have any problems associated with the condition.</p><p>Bilateral forms of polymicrogyria tend to cause more severe neurological problems. Signs and symptoms of these conditions can include recurrent seizures (epilepsy), delayed development, crossed eyes, problems with speech and swallowing, and muscle weakness or paralysis. The most severe form of the disorder, bilateral generalized polymicrogyria, affects the entire brain. This condition causes severe intellectual disability, problems with movement, and seizures that are difficult or impossible to control with medication.</p><p>Polymicrogyria most often occurs as an isolated feature, although it can occur with other brain abnormalities. It is also a feature of several genetic syndromes characterized by intellectual disability and multiple birth defects. These include 22q11.2 deletion syndrome, Adams-Oliver syndrome, Aicardi syndrome, Galloway-Mowat syndrome, Joubert syndrome, and Zellweger spectrum disorder.</p><p class=\"section-source\">Source: <a href=\"https://medlineplus.gov/genetics/condition/polymicrogyria\">MedlinePlus Genetics</a></p>","key":"overview","title":"Condition overview"}],"sharedSources":[],"slug":"polymicrogyria","sourceDocuments":[{"name":"Polymicrogyria","reviewed":"2009-06","sections":{"causes":{"fetchedAt":"2026-10-20T20:24:18.015690+00:00","label":"Causes","pageSha256":"33b5ba2023797d5e16229fe4fb76eebefca4d6f0c2884b3493682065bac5186f","sourceUrl":"https://medlineplus.gov/genetics/condition/polymicrogyria/#causes","status":"available"}},"source_release":"2026-10-01","url":"https://medlineplus.gov/genetics/condition/polymicrogyria"}],"sourceNote":"MedlinePlus descriptions and Causes; original HPO annotations.","takeawayHtml":"Cause explanations are from MedlinePlus Genetics. Feature values below retain their original sources and patient groups.","title":"Polymicrogyria"},"schemaVersion":1}