Congenital malformation of nervous system

Arini Nurlela 03008040

Learning Points

Know about the normal embryo development Know about NTD Know about Spina Bifida

Normal Development

19 Days

20 Days

23 Days

Neural Tube Defect

Neural Tube Defect

Neural tube defects (NTDs) are a group of disorders that arise early in fetal development and can cause life-long complications of varying severity. During the first 3-4 weeks of development, specific cells in an embryo curl up and their edges fuse together to form a narrow tube that becomes the foundation of the spinal cord, brain, and the bone and tissues that surround it. This neural tube fusing process usually is complete by 28 days of gestation before many women even know that they are pregnant. If the tube does not close properly along its entire length, then a NTD will form at the open location. most common birth defects, occurring in approximately one in 1.000 live births in the United States

Causing

Neural Tube Defect (cont)

Treatment Treatments of NTDs depends on the severity of the complication.

Discussion

Spina Bifida

OCCULTA

SPINA BIFIDA

MENINGOCELE

CYSTICA

MYELOMENINGOCELE

Tatyana McFadden

CONCLUSION

Spina bifida is the most common of a group of birth defects known as neural tube defects, which affect the central nervous system (brain and spinal cord).
Spina bifida begins in the womb, when the tissues that fold to form the neural tube do not close or do not stay closed completely. This causes an opening in the vertebrae, which surround and protect the spinal cord. This occurs just a few weeks (21 to 28 days) after conception,usually before the woman knows that she is pregnant. Spina bifida malformations fall into two catagories,there are spina bifida occulta, spina bifida cystica (meningocele and myelomeningocele). Myelomeningocele is the most significant form and it is this that leads to disability in most affected individuals. The only known way to prevent spina bifida and other neural tube defects is for the mother to have adequate folic acid levels before and during early pregnancy. This does not work in all cases, but studies have shown that as many as 70% of cases of severe spina bifida could be prevented by adequate folic acid intake.

References
1.Markum A.H. Buku Ajar Ilmu Kesehatan Anak, Jakarta : EGC, 2002.

2. Kent, George C.; Robert K. Carr. Comparative Anatomy of the Vertebrates: Ninth Edition. New York, NY, USA: McGraw-Hill Higher Education. pp. 408.2001
3. Sadler,T.W.Embriologi Kedokteran Langman.Edisi 10.Jakarta : EGC,2009. 4. Menkes,John H; Till,Kenneth.Text Book of Child Neurology. Philadelphia : Lippincott Williams and Wilkins,1995 5. Holmes,Lewis B. Journal of Pediatrics Vol. 120, Issue 6, Pages 918-919.1992 6. http://www.nlm.nih.gov/medlineplus/spinabifida.html.Accessed June 15 ,2011

Days of gestation 0-18 18 22-23 24-26 26-28

Event Three germ layers elaborate and early neural plate forms Neural plate and groove development Optic vesicles appear Anterior neuropore closed Posterior neuropore closed,ventral horns form

Effect of toxic stimulus No effect /death Anterior midline defects induction hydrocephalus Anencephaly Cranium bifidum,spina bifida cystica,spina bifida occulta

28-32
32 33-35

Anterior and posterior nerve roots form

Cerebellar primordium,vascular circulation Prosencephalon claves to form paired telencephalon. Five cerebral vesicles, choroid plexi, dorsal root ganglion develop Region of olfactory bulb appears in fore brain Differentiation of cerebral cortex, meninges, ventricular foramina, CSF circulation Corpus callosum Primary fissures of cerebral cortex ,spinal cord ends at L3 level Neuronal proliferation in cerebral cortex ends Secondary and tertiary sulci Neuronal migration, glial cell production. Myelin formation, axosomatic and axodendritic synaptic connections, spinal cord ends L1-L2 level Microcephaly,proliferation syndrome,migration anomalies Holoprosencephaly

41 56

Arhinencephaly Dandy-walker syndrome

70-100 70-150 140-175 7-9 months 175 days-4 years postnatally

Agenesis of corpus calllosum Lissencephaly, pachygyria Defects of cellular architectonic , myelin defects Destructive pathologic changes first noted