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