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Malformations

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Pediatric Neuropathology

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Abstract

The brain and upper spinal cord form from the neural plate during primary neurulation beginning at 22 days’ gestation; the sacral spinal cord forms from the tail bud during secondary neurulation [1]. The various classifications of neural tube defects (NTDs) is complex and somewhat contradictory [2]. Practically, they can be considered as “open” (e.g., anencephaly, craniorachischisis, myelomeningocele) or “closed” (e.g., encephaloceles, meningoceles, split spinal cord). There are several pathoetiologies [3]. Folic acid supplementation before and during early pregnancy prevents most NTDs [3,4]. A mutant mouse model for NTD, Splotch, is being used to define the mechanism of teratogenesis by folate insufficiency [5]. A second mutant, curly tail, has an NTD that responds to myoinositol [6].

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2 Malformations

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(2007). Malformations. In: Pediatric Neuropathology. Springer, Tokyo. https://doi.org/10.1007/978-4-431-49898-8_2

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