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neural tube defects

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neural tube defects

  1. 1. ‘ ’
  2. 2. NTD Neural tube defects
  3. 3. • NT fail to close ~3-4w in utero due to radiation, chemicals, drugs, DM, malnutrition, hyperthermia
  4. 4. Development • Ectoderm + mesoderm + endoderm = central nervous system [3rd wk] • Endoderm  notochord  IV disc • Mesoderm  vertebrae, dura, brain? • Ectoderm  neural plate, neural groove, neural crest • Neural crest  PNS, Cr nerve ganglia, myelin sheath.
  5. 5. • Spina bifida occulta • Meningocele • Meningomyelocele • Encephalocele • Anencephaly • Syringomyelia • Tethered chord
  6. 6. • Low sacral region – a) Bowel & Bladder incontinence b) Anesthesia in perianal region c) No motor impairment • Mid lumbar region – a) Flaccid paralysis of lower limbs b) Absence of DTR c) No response to pain , touch • Cervical/thoracic No deficit
  7. 7. Management • Counseling • UTI management • Surgery
  8. 8. Lober’s criteria • No surgery if – Paraplegia – Kyphoscoliosis – Gross hydrocephalus – Congenital anomaly – IntraCranial birth injury – ventriculitis
  9. 9. Anencephaly
  10. 10. Antenatal screening • ~16wks • AlfaFetoProtein • Anticholinesterase • USG
  11. 11. Neuronal migration disorders • Radial glial fibre system • Reelin, mdab1. • Wide spectrum
  12. 12. Lissencephaly
  13. 13. Porencephaly
  14. 14. Holoprosencephaly
  15. 15. Schizencephaly
  16. 16. Neurocutaneous syndromes • Problem in differentiation of primitive ectoderm • NF • Tuberous sclerosis • Sturge weber • Von Hippel Lindau • Ataxia telangiectasia • Incontinentia pigmenti
  17. 17. Neurofibramatosis • 1:4000, autosomal dominant
  18. 18. • Abnormal neural crest differentiation & migration
  19. 19. 2 of the 7 signs • Cafeau-lait spots >5, >5mm, • >2 Iris lisch nodules • Optic glioma • Axillary freckling • >1 NF / plexiform NF, • Scoliosis, sphenoid dysplasia • Relative with NF1
  20. 20. Unidentified bright objects
  21. 21. Neurologic complications • Areas of dysmyelination or focal areas of increased water content (UBOs) • Seizures, Hydrocephalus, macrocephaly • TIA, FND, learning problems, • precocious puberty, hypertension • Pheochromocytoma, meningioma,glioma • Scoliosis
  22. 22. s
  23. 23. NF2 • Bilateral acoustic neuromas • Relative with NF2 or NF/glioma/meningioma
  24. 24. Shagreen patch
  25. 25. Tuberous sclerosis
  26. 26. • AD, 1:6000.
  27. 27. Features • Infantile spasm • Cognitive problems • Rhabdomyoma heart • Polycystic kidney disease • Angiomyolipoma lung
  28. 28. Mulberry tumor
  29. 29. Tubers Candle dripping appearance
  30. 30. Hypsarrhythmia
  31. 31. Management • Seizure control • Investigate other organic problems • Manage ICT
  32. 32. Sturge weber syndrome
  33. 33. Features • Portwine stain • Contralateral focal clonic seizuures • Contralateral hemiparesis, transient strokes • Glaucoma eye • MR
  34. 34. • Hemispherectomy • Measure IOT • Pulsed laser for portwine stain • Anticonvulsants
  35. 35. Ataxia telangiectasia
  36. 36. Features • ATAXIA • Nystagmus • Oculomotor apraxia • Less elasticity • More sinopulmonary infections • IgA, IgG2 less • Lymphomas • Broken chromosomes
  37. 37. Thank you

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