Classification system for malformations of cortical development

Changed by Amir Rezaee, 7 Jun 2015
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Updates to Article Attributes

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Classification system for malformations of cortical development organises a myriad of conditions according to one of three major underlying  mechanisms:

  1. abnormal cell proliferation
  2. abnormal neuronal migration
  3. abnormal cortical organisation

As is to be expected a number of conditions fall outside these three groupings and are placed in a miscellaneous group: those associated with other diseases or yet to be classified 2. The classification present below is adapted from Barkovic et al, 2004 2.

Abnormal cell proliferation or apoptosis 

These conditions include those due to disorders of neuronal and/or glial proliferation or apoptosis. They can be further divided into two groups; those with abnormalities of brain size due to abnormalities in proliferation or apoptosis, and; those with abnormal cells (neoplastic or non-neoplastic) 2.

Abnormal brain size
Abnormal cell proliferation

Non-neoplastic

Neoplastic

Abnormal neuronal migration

Abnormal cortical organisation

Not otherwise classified

  • malformations secondary to inborn errors of metabolism
    • mitochondrial and pyruvate metabolic disorders 
    • peroxisomal disorders 
  • other unclassified malformations
    • sublobar dysplasia 

See also

For other congenital central nervous system classifications see:

  1. classification system for midline abnormalities of the brain
  2. classification system for malformations of the cerebellum
  3. classification system for malformations of the brainstem
  • -</ol><p>As is to be expected a number of conditions fall outside these three groupings and are placed in a miscellaneous group: those associated with other diseases or yet to be classified <sup>2</sup>. The classification present below is adapted from Barkovic et al, 2004 <sup>2</sup>.</p><h4>Abnormal cell proliferation</h4><p>These conditions include those due to disorders of neuronal and/or glial proliferation or apoptosis. They can be further divided into two groups; those with abnormalities of brain size due to abnormalities in proliferation or apoptosis, and; those with abnormal cells (neoplastic or non-neoplastic) <sup>2</sup>.</p><h5>Abnormal brain size</h5><ul>
  • +</ol><p>As is to be expected a number of conditions fall outside these three groupings and are placed in a miscellaneous group: those associated with other diseases or yet to be classified <sup>2</sup>. The classification present below is adapted from Barkovic et al, 2004 <sup>2</sup>.</p><h4>Abnormal cell proliferation or apoptosis </h4><p>These conditions include those due to disorders of neuronal and/or glial proliferation or apoptosis. They can be further divided into two groups; those with abnormalities of brain size due to abnormalities in proliferation or apoptosis, and; those with abnormal cells (neoplastic or non-neoplastic) <sup>2</sup>.</p><h5>Abnormal brain size</h5><ul>
  • -<a href="/articles/microcephaly">microcephaly</a> with normal to thin cortex </li>
  • -<li><a href="/articles/microlissencephaly">microlissencephaly</a></li>
  • +<a href="/articles/microcephaly">microcephaly</a><ul>
  • +<li>with normal to simplified cortical pattern </li>
  • +<li><a href="/articles/microlissencephaly">microcephaly with lissencephaly</a></li>
  • +</ul>
  • +</li>

References changed:

  • 5. Barkovich AJ, Guerrini R, Kuzniecky RI et-al. A developmental and genetic classification for malformations of cortical development: update 2012. Brain. 2012;135 (5): 1348-69. <a href="http://dx.doi.org/10.1093/brain/aws019">doi:10.1093/brain/aws019</a> - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3338922">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/22427329">Pubmed citation</a><span class="auto"></span>

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