neural tube defects
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Transcript of neural tube defects
‘அரி�து, அரி�து மா�னி�டரி�ய் பி�றத்தல்அரி�து. அதனி�னும்அரி�து, கூன், குருடு, செ�வி�டு
’நீங்கி� பி�றத்தல்அரி�து
NTD
Neural tube defects
• NT fail to close ~3-4w in utero due to radiation, chemicals, drugs, DM, malnutrition, hyperthermia
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.
• Spina bifida occulta• Meningocele• Meningomyelocele• Encephalocele • Anencephaly • Syringomyelia• Tethered chord
• 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
Management
• Counseling• UTI management• Surgery
Lober’s criteria
• No surgery if– Paraplegia – Kyphoscoliosis– Gross hydrocephalus– Congenital anomaly– IntraCranial birth injury– ventriculitis
Anencephaly
Antenatal screening
• ~16wks• AlfaFetoProtein• Anticholinesterase • USG
Neuronal migration disorders
• Radial glial fibre system • Reelin, mdab1.• Wide spectrum
Lissencephaly
Porencephaly
Holoprosencephaly
Schizencephaly
Neurocutaneous syndromes
• Problem in differentiation of primitive ectoderm
• NF• Tuberous sclerosis• Sturge weber • Von Hippel Lindau• Ataxia telangiectasia• Incontinentia pigmenti
Neurofibramatosis
• 1:4000, autosomal dominant
• Abnormal neural crest differentiation & migration
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
Unidentified bright objects
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
s
NF2
• Bilateral acoustic neuromas• Relative with NF2 or NF/glioma/meningioma
Shagreen patch
Tuberous sclerosis
• AD, 1:6000.
Features
• Infantile spasm • Cognitive problems• Rhabdomyoma heart• Polycystic kidney disease• Angiomyolipoma lung
Mulberry tumor
Tubers
Candle dripping appearance
Hypsarrhythmia
Management
• Seizure control• Investigate other organic problems• Manage ICT
Sturge weber syndrome
Features
• Portwine stain• Contralateral focal clonic seizuures• Contralateral hemiparesis, transient strokes• Glaucoma eye• MR
• Hemispherectomy • Measure IOT • Pulsed laser for portwine stain• Anticonvulsants
Ataxia telangiectasia
Features
• ATAXIA• Nystagmus• Oculomotor apraxia• Less elasticity• More sinopulmonary infections• IgA, IgG2 less• Lymphomas• Broken chromosomes
Thank you