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The histopathology of polymicrogyria: a series of 71 brain autopsy studies
Author(s) -
Jansen Anna C,
Robitaille Yves,
Honavar Mrinalini,
Mullatti Nandini,
Leventer Richard J,
Andermann Eva,
Andermann Frederick,
Squier Waney
Publication year - 2016
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.12840
Subject(s) - polymicrogyria , corticogenesis , leptomeninges , maldevelopment , neuropathology , pathology , autopsy , etiology , subplate , histopathology , medicine , cortex (anatomy) , cerebral cortex , neuroscience , biology , anatomy , disease , central nervous system , epilepsy , embryonic stem cell , gene , biochemistry
Aim Polymicrogyria ( PMG ) is one of the most common forms of cortical malformation yet the mechanism of its development remains unknown. This study describes the histopathological aspects of PMG in a large series including a significant proportion of fetal cases. Method We have reviewed the neuropathology and medical records of 44 fetuses and 27 children and adults in whom the cortical architecture was focally or diffusely replaced by one or more festooning bands of neurons. Results The pial surface of the brain overlying the polymicrogyric cortex was abnormal in almost 90% of cases irrespective of the aetiology. This accords with animal studies indicating the importance of the leptomeninges in cortical development. The aetiology of PMG was highly heterogeneous and there was no correlation between cortical layering patterns and aetiology. PMG was almost always associated with other brain malformations. Interpretation The inclusion of many fetal cases has allowed us to examine the early developmental stages of PMG . The study indicates the significance of surface signals responsible for human corticogenesis and the complex interaction between genetic and environmental factors leading to this common endpoint of cortical maldevelopment.