Malformations of Cortical Development: Difference between revisions

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! Group !! Affected step of development !! MCDs resulting from the disturbance !! Short definition of the MCD
! Group
! Affected step of development
! MCDs resulting from the disturbance
! Short definition of the MCD
|-
|-
| Group I || Progenitor cell proliferation and apoptosis ||
| rowspan="4" | Group I
* Microcephaly
| rowspan="4" | Progenitor cell proliferation and apoptosis
* Macrocephaly
| Microcephaly
* Hemimegalencephaly
| Abnormally small head and brain
* Focal cortical dysplasia ||
* Abnormally small head and brain
* Abnormally big head and brain
* Overgrowth of (part of) a cerebral hemisphere
* Disturbed lamination and dysmorphic neurons
|-
|-
| Group II || Neuronal migration ||
| Macrocephaly
* Lissencephaly type I
| Abnormally big head and brain
* Periventricular heterotopia (PH)
* Subcortical band heterotopia/double cortex ||
* Absence of normal convolutions/folds
* Neurons accumulating at the ventricles underneath a normal cortex
* Band of grey matter located between the lateral ventricular wall and the cortex
|-
|-
| Group III || Neuronal organisation ||
| Hemimegalencephaly
* Cobblestone lissencephaly/lissencephaly type II
| Overgrowth of (part of) a cerebral hemisphere
* Polymicrogyria
|-
* Schizencephaly ||
| Focal cortical dysplasia
* Overmigration of neurons to localize on the surface of a brain with reduced gyri
| Disturbed lamination and dysmorphic neurons
* Too many (usually small) folds/convolutions
|-
* Fluid-filled cleft from ventricle(s) to pia lined by heterotopic grey matter
| rowspan="3" | Group II
| rowspan="3" | Neuronal migration
| Lissencephaly type I
| Absence of normal convolutions/folds
|-
| Periventricular heterotopia (PH)
| Neurons accumulating at the ventricles underneath a normal cortex
|-
| Subcortical band heterotopia/double cortex
| Band of grey matter located between the lateral ventricular wall and the cortex
|-
| rowspan="3" | Group III
| rowspan="3" | Neuronal organisation
| Cobblestone lissencephaly/lissencephaly type II
| Overmigration of neurons to localize on the surface of a brain with reduced gyri
|-
| Polymicrogyria
| Too many (usually small) folds/convolutions
|-
| Schizencephaly
| Fluid-filled cleft from ventricle(s) to pia lined by heterotopic grey matter
|}
|}

Revision as of 09:58, 22 May 2024

Categories

  • group I: abnormal cell proliferation or apoptosis
  • group II: abnormal neuronal migration
  • group III: abnormal cortical organization
Group Characteristics Conditions
Group I abnormal proliferation of neuronal and glial cells
  • Microcephaly
  • macrocephaly
  • FCDs without and with balloon cells (Taylor dysplasia types IIa and IIb),
  • hemimegalencephaly,
  • tuberous sclerosis,
  • dysembryoplastic neuroepithelial tumors (DNETs), gangliogliomas, gangliocytomas
Group II abnormal neuronal migration
  • Lissencephaly
  • Band Heterotopia
Group III abnormal cortical organization
  • Polymicrogyria,
  • schizencephaly,
  • type I FCDs,
  • mild MCDs


Group Affected step of development MCDs resulting from the disturbance Short definition of the MCD
Group I Progenitor cell proliferation and apoptosis Microcephaly Abnormally small head and brain
Macrocephaly Abnormally big head and brain
Hemimegalencephaly Overgrowth of (part of) a cerebral hemisphere
Focal cortical dysplasia Disturbed lamination and dysmorphic neurons
Group II Neuronal migration Lissencephaly type I Absence of normal convolutions/folds
Periventricular heterotopia (PH) Neurons accumulating at the ventricles underneath a normal cortex
Subcortical band heterotopia/double cortex Band of grey matter located between the lateral ventricular wall and the cortex
Group III Neuronal organisation Cobblestone lissencephaly/lissencephaly type II Overmigration of neurons to localize on the surface of a brain with reduced gyri
Polymicrogyria Too many (usually small) folds/convolutions
Schizencephaly Fluid-filled cleft from ventricle(s) to pia lined by heterotopic grey matter
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