Classification system for malformations of cortical development

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 Barkovich et al, 2004 2.

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

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

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

Malformations of the central nervous system
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Article Information

rID: 15853
Synonyms or Alternate Spellings:
  • Disorders of cortical formation

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Cases and Figures

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    Case 1: microcephaly
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    Tuberous sclerosi...
    Case 2: hamartomas of tuberous sclerosis
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    Case 3: focal cortical dysplasia with balloon cells
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    Case 4: gangliocytoma
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    Case 5: DNET
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    Case 6: band heterotopia
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    Case 7: heterotopia - subependymal
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    Case 8: schizencephaly
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    Case 9: cortical dysplasia without balloon cells
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    Case 10: ganglioglioma
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    Case 11: hemimegalancephaly
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