Classification system for malformations of cortical development
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Classification system for malformations of cortical development organises a myriad of conditions according to one of three major underlying mechanisms:
- abnormal cell proliferation
- abnormal neuronal migration
- abnormal cortical organization
As is to be expected a number of conditions fall out side these three groupings and are placed in an miscelaneous 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
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
- microcephaly with normal to thin cortex
- microlissencephaly
- microcephaly with extensive polymicrogyria
- macrocephalies (megalencephaly/macrocephaly)
Abnormal cell proliferation
Non-neoplastic
- cortical hamartomas of tuberous sclerosis
- cortical dysplasia with balloon cells
- hemimegalencephaly
- focal cortical dysplasia
Neoplastic
Abnormal neuronal migration
-
lissencephaly type I - subcortical band heterotopia spectrum
(aka(a.k.a. band heterotopia): undermigration - lissencephaly type II (cobblestone complex): overmigration
- heterotopia
- subependymal heterotopia
- subcortical heterotopia (not includint band heterotopia)
- marginal glioneuronal heterotopia
Abnormal cortical organizationorganisation
-
polymicrogyria and schizencephaly
- bilateral polymicrogyria syndromes
- schizencephaly
- polymicrogyria or schizencephaly as part of multiple congenital anomaly/mental retardation syndromes
- cortical dysplasia without balloon cells
- microdysgenesis
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:
-</ol><p>As is to be expected a number of conditions fall out side these three groupings and are placed in an miscelaneous group: those associated with other diseases or yet to be classified <sup>2</sup>. The classification present below is adapted from Barkovic <em>et al</em>, 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 out side these three groupings and are placed in an miscelaneous 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>
-<li>macrocephalies (<a href="/articles/megalencephaly">megalencephaly</a> / <a href="/articles/macrocephaly">macrocephaly</a>)</li>- +<li>macrocephalies (<a href="/articles/megalencephaly">megalencephaly</a>/<a href="/articles/macrocephaly">macrocephaly</a>)</li>
-<li><a title="Focal cortical dysplasia" href="/articles/focal-cortical-dysplasia">focal cortical dysplasia</a></li>- +<li><a href="/articles/focal-cortical-dysplasia">focal cortical dysplasia</a></li>
-<a href="/articles/lissencephaly-type-i-subcortical-band-heterotopia-spectrum">lissencephaly type I - subcortical band heterotopia spectrum</a> (aka <a href="/articles/band-heterotopia">band heterotopia</a>): undermigration</li>- +<a href="/articles/lissencephaly-type-i-subcortical-band-heterotopia-spectrum">lissencephaly type I - subcortical band heterotopia spectrum</a> (a.k.a. <a href="/articles/band-heterotopia">band heterotopia</a>): undermigration</li>
-</ul><h4>Abnormal cortical organization</h4><ul>- +</ul><h4>Abnormal cortical organisation</h4><ul>