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We compare the features in 42 patients evaluated for physical and neuroimaging characteristics of MCAP and MPPH and propose a more global view of these syndromes based on classes of developmental abnormalities that include primary MEG and growth dysregulation, developmental vascular anomalies (primarily capillary malformations), distal limb anomalies (such as syndactyly and polydactyly), cortical brain malformations (most distinctively polymicrogyria, PMG), and variable connective tissue dysplasia. Based on these classes of developmental abnormalities, we propose that MCAP diagnostic criteria include progressive MEG with either vascular anomalies or syndactyly. In parallel, we propose that MPPH diagnostic criteria include progressive MEG and PMG, absence of the vascular anomalies and syndactyly characteristic of MCAP, and absence of brain heterotopia. \u00a9 2012 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.a.34402","type":"journal-article","created":{"date-parts":[[2012,1,6]],"date-time":"2012-01-06T19:52:49Z","timestamp":1325879569000},"page":"269-291","source":"Crossref","is-referenced-by-count":196,"title":["Megalencephaly\u2010capillary malformation (MCAP) and megalencephaly\u2010polydactyly\u2010polymicrogyria\u2010hydrocephalus (MPPH) syndromes: Two closely related disorders of brain overgrowth and abnormal brain and body morphogenesis"],"prefix":"10.1002","volume":"158A","author":[{"given":"Ghayda M.","family":"Mirzaa","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert 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