Congenital CNS Anomalies

Question 1

What is the most common congenital CNS anomaly?

Answer 1

Neural tube defect (Spina bifida)

Question 2

At what gestational weeks do the rostral and caudal neuropores close?

Answer 2

Rostral: Day 25, Caudal: Day 27

Question 3

What is the most common type of neural tube defect?

Answer 3

Spina bifida

Question 4

Which embryonic structure releases factors that induce neural plate formation?

Answer 4

Notochord

Question 5

What is the primary cause of congenital hydrocephalus?

Answer 5

Aqueductal stenosis

Question 6

Which brain vesicle gives rise to the cerebral hemispheres?

Answer 6

Telencephalon

Question 7

What are the three primary brain vesicles?

Answer 7

Prosencephalon, Mesencephalon, Rhombencephalon

Question 8

Which neural tube defect results from failure of the rostral neuropore to close?

Answer 8

Anencephaly

Question 9

What is the commonest location of spina bifida?

Answer 9

Lumbar spine

Question 10

What maternal vitamin deficiency is strongly associated with neural tube defects?

Answer 10

Folic acid deficiency

Question 11

What are the key features of spina bifida occulta?

Answer 11

Cutaneous stigmata (hair tuft, pigmented patch, dimple), no neurological dysfunction

Question 12

Which congenital spine anomaly is associated with recurrent meningitis?

Answer 12

Congenital dermal sinus

Question 13

What are the main types of spina bifida cystica?

Answer 13

Meningocele, Myelomeningocele, Myeloschisis

Question 14

Which type of spina bifida involves herniation of meninges and CSF but no neural tissue?

Answer 14

Meningocele

Question 15

What condition is characterized by a defect in both meninges and spinal cord herniation?

Answer 15

Myelomeningocele

Question 16

Which neural tube defect presents with a sac-like swelling containing brain tissue?

Answer 16

Encephalocele

Question 17

What congenital CNS anomaly is associated with hindbrain herniation?

Answer 17

Chiari malformation

Question 18

Which Chiari malformation is most commonly associated with myelomeningocele?

Answer 18

Chiari Type II

Question 19

What is the hallmark of Dandy-Walker malformation?

Answer 19

Agenesis/hypoplasia of cerebellar vermis with enlarged posterior fossa

Question 20

Which neural tube defect has the highest mortality rate?

Answer 20

Anencephaly

Question 21

What embryological process leads to neural tube formation?

Answer 21

Neurulation

Question 22

Which structure gives rise to the cerebellum?

Answer 22

Rhombencephalon (Metencephalon)

Question 23

Which congenital anomaly presents with a ‘sunsetting’ sign in the eyes?

Answer 23

Hydrocephalus

Question 24

What is the commonest congenital cause of hydrocephalus?

Answer 24

Aqueductal stenosis

Question 25

Which syndrome includes café-au-lait spots, neurofibromas, and Lisch nodules?

Answer 25

Neurofibromatosis type 1 (NF-1)

Question 26

What genetic disorder is associated with bilateral vestibular schwannomas?

Answer 26

Neurofibromatosis type 2 (NF-2)

Question 27

Which neurocutaneous disorder is characterized by port-wine stains and glaucoma?

Answer 27

Sturge-Weber syndrome

Question 28

Which condition is caused by premature fusion of cranial sutures?

Answer 28

Craniosynostosis

Question 29

What is the most common type of craniosynostosis?

Answer 29

Sagittal craniosynostosis

Question 30

What is the recommended daily allowance of folic acid for neural tube defect prevention?

Answer 30

400 mcg/day

Question 31

Which congenital anomaly results from failure of corpus callosum formation?

Answer 31

Agenesis of the corpus callosum

Question 32

Which neural tube defect is associated with tethered cord syndrome?

Answer 32

Spina bifida occulta

Question 33

What congenital condition involves CSF obstruction at the foramina of Luschka and Magendie?

Answer 33

Dandy-Walker malformation

Question 34

Which phakomatosis is associated with hemangioblastomas and renal cell carcinoma?

Answer 34

Von Hippel-Lindau syndrome

Question 35

What embryologic abnormality results in colpocephaly?

Answer 35

Abnormal neuronal proliferation

Question 36

What congenital anomaly presents with a 'cracked pot' sound (Macewen sign)?

Answer 36

Hydrocephalus

Question 37

What is the major cause of congenital CNS anomalies in low-income countries?

Answer 37

Maternal folate deficiency

Question 38

What is a common prenatal diagnostic marker for neural tube defects?

Answer 38

Elevated alpha-fetoprotein (AFP)

Question 39

Which neural tube defect has the worst prognosis?

Answer 39

Anencephaly

Question 40

What is the treatment for myelomeningocele?

Answer 40

Surgical repair

Question 41

Which neural tube defect is most associated with hydrocephalus?

Answer 41

Myelomeningocele

Question 42

What CNS congenital anomaly is frequently associated with clubfoot?

Answer 42

Spina bifida

Question 43

Which syndrome involves subependymal giant cell astrocytomas?

Answer 43

Tuberous sclerosis

Question 44

Which congenital CNS anomaly is characterized by absence of cerebral hemispheres?

Answer 44

Hydranencephaly

Question 45

What is the treatment of choice for hydrocephalus?

Answer 45

CSF diversion (shunt or endoscopic third ventriculostomy)

Question 46

Which congenital CNS anomaly is associated with infanticide in some cultures?

Answer 46

Myelomeningocele

Question 47

What is the best imaging modality for diagnosing craniosynostosis?

Answer 47

CT scan

Question 48

What congenital anomaly presents with 'rocker-bottom feet' as an associated feature?

Answer 48

Meningocele

Question 49

What is the gold standard diagnostic tool for Dandy-Walker malformation?

Answer 49

MRI

Question 50

What is the most critical prevention method for neural tube defects?

Answer 50

Folic acid supplementation