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Congenital brain malformations

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classification of congenital brain malformations

Publicada em: Saúde e medicina

Congenital brain malformations

  1. 1. ImagIng of CongenItalImagIng of CongenItal BraIn malformatIonsBraIn malformatIons
  2. 2. Moment of embryology
  3. 3. NeurulationNeurulation
  4. 4. Ventral induction
  5. 5. MantlMantl ee ProliferationProliferation MigrationMigration OrganizationOrganization
  6. 6. Myelination PNS CNS
  7. 7. Normal myelination At birth 1 year6 month 2 years
  8. 8. CephalocelesCephaloceles Herniation of intracranial contents through a congenital defect in the dura and skull - Usually located at the midline.
  9. 9. Occipital Meningoencephalocele
  10. 10. Parietal Meningoencephalocele
  11. 11. Atretic occipital cephalocele
  12. 12. Chiari malformation type I
  13. 13. Basilar invagination
  14. 14. Posterior fossa decompression
  15. 15. Chiari malformation type III
  16. 16. Prosencephalon Mesencephalon Rhomboencephalon
  17. 17. Classification of holoprosencephaly
  18. 18. Classification of holoprosencephaly
  19. 19. Septo-optic Dysplasia
  20. 20. DWM DWV Persistent Blake’s pouch Mega cisterna magna Anterior membranous area anomaly Posterior membranous area anomaly Retro-cerebellar cyst Vermis Hypo-plastic Rotated upwards Hypo-plastic No or mild hypoplasia No or mild hypoplasia 4th ventricle Markedly dilated Dilated Dilated Normal Posterior fossa Expanded Normal size Normal size Normal size hydrocephalus 75 % of cases 25% of cases Present No
  21. 21. Dandy-Walker MalformationDandy-Walker Malformation
  22. 22. Dandy-Walker Variant
  23. 23. Mega Cistern Magna
  24. 24. Posterior Fossa Arachnoid Cyst
  25. 25. Corpus callosum genesis
  26. 26. Corpus callosum develpment
  27. 27. Corpus Callosum genesis • Genu  body  splenium  rostrum
  28. 28. Corpus Callosum Agenesis
  29. 29. Corpus Callosum Agenesis Absent cingulate gyrus High riding 3rd ventricle Parallel lateral ventricles Colpocephaly frontal horns small & pointed Radiating medial surface gyri
  30. 30. Partial corpus Callosum Agenesis
  31. 31. Corpus Callosum Agenesis Interhemispheric cyst
  32. 32. Corpus Callosum Agenesis Pericallosal lipoma
  33. 33. Focal cortical dysplasia
  34. 34. Focal cortical dysplasia
  35. 35. Focal cortical dyspalsia
  36. 36. Tuberous sclerosis • Cortical tubers. • White matte lesions. • Subependymal nodules. • SGCA.
  37. 37. Ganglioglioma
  38. 38. DNET
  39. 39. Type 1 lissencephaly (agyria / pachygyria complex)
  40. 40. Type 2 (cobblestone) lissencephaly
  41. 41. Heterotopia
  42. 42. Heterotopias • Normal neurons at abnormal sites • Result from arrested neuronal migration from periventricular germinal zone to the cortex. • Heterotopias are isointense to normal gray matter in all pulse sequences and do not enhance on administration of IV contrast.
  43. 43. Hetertopia • Isointense to grey matter on all pulse sequences  No enhancement.  No associated mass effect.
  44. 44. Band hetertopia
  45. 45. Sucortical curvilinear nodular hetertopia
  46. 46. Subcortical focal nodular heterotopia
  47. 47. Subependymal diffuse nodular heterotopia
  48. 48. subependymal focal nodular hetertopia
  49. 49. Polymicrogyria
  50. 50. Polymicrogyria
  51. 51. Schizencephaly • CSF cleft , lined by grey matter, between the ependymal surface of the ventricle to the pial surface of the cerebral hemisphere.
  52. 52. Vascular theory of Schizencephaly
  53. 53. Myelination disorders
  54. 54. Dys-myelination Vs hypo-myelination
  55. 55. Dys-myelination Hypo- myelination
  56. 56. Delayed myelination Vs hypomyelination • Repeat MRI after 6 months. • Delayed myelination  myelination progression. • Hypomyelination  no myelination progression. • DD in these 2 conditions is quite different.
  57. 57. Delayed myelination • Delayed myelination is a non specific finding associated with global developmental delay. Causes: • Chromosomal abnormalities (e.g. trisomy 21). • Metabolic disorders (e.g. phenylketonuria). • Acquired causes (e.g. HIE).
  58. 58. Special features
  59. 59. DD of leukodystrophies

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