Primary Craniospinal Anomalies

Question 1

A 40-year-old female patient presents in neurosurgical OPD with complaints of occipital headache which is exacerbated by coughing. On examination, she has numbness in right upper
limb and downbeat nystagmus is found to be positive. MRI of brain shows cerebellar tonsillar herniation 7 mm below foramen with stasis of CSF flow at foramen magnum level and cervical syringomyelia. What is this condition called?
● A. Type 1 Chiari malformation
● B. Type 2 Chiari malformation
● C. Arnold Chiari malformation
● D. Chiari type 1.5 malformation
● E. Cerebellar tonsillar herniation syndrome

Answer 1

A. Type 1 Chiari malformation

Question 2

Chiari pseudotumor cerebri syndrome is a condition in which CSF obstruction due to Chiari leads to elevated ICP without hydrocephalus. Following are the features of this condition except?
● A. CSF shunting is an initial therapy
● B. Posterior fossa decompression is done for patients with papilledema and Chiari
● C. Papilledema is uncommon in this condition
● D. LP is relatively contraindicated
● E. None of the above

Answer 2

C. Papilledema is uncommon in this condition

Question 3

A female with obesity presents with visual field constriction and only abducens nerve palsy. On examination, there is papilledema without hydrocephalus. Following are true statements
regarding pseudotumor cerebri except?
● A. For patients with severe pseudotumor cerebri VP shunt or ONSF is the treatment of choice
● B. On LP if opening pressure is more than 25 cm of CSF, consider pseudotumor cerebri
● C. If findings are not suggestive of pseudotumor, then posterior fossa decompression is done
● D. For deterioration of vision, shunt is the only treatment
● E. None of the above

Answer 3

D. For deterioration of vision, shunt is
the only treatment

Question 4

What are the features of Chiari type 1.5?
● A. Dislocation of cerebellum below foramen magnum
● B. Cerebellar hypoplasia without cerebellar herniation and small posterior fossa
● C. Cerebellar tonsils herniated ventrally with brain stem compression
● D. Entire cervicomedullary junction situated below foramen magnum
● E. Syringohydromyelia without hindbrain herniation

Answer 4

D. Entire cervicomedullary junction situated below foramen magnum

Question 5

A neonate presents in neurosurgical emergency with sudden respiratory arrest. He/she had previous history of swallowing difficulties with poor feeding, cyanosis during feeding, and nasal regurgitation. There is also history of previous apneic spells and stridor. MRI of brain showed low caudally displaced cervicomedullary junction, small posterior fossa, and tectal beaking. What is the most likely diagnosis?
● A. Chiari malformation type 1
● B. Chiari malformation type 3
● C. Arnold–Chiari malformation
● D. Chiari type 1.5
● E. Chiari malformation type 4

Answer 5

C. Arnold–Chiari malformation

Question 6

What is the most frequently performed procedure for Chiari malformation which decompresses brain stem and reestablish normal flow of CSF at cervicomedullary junction?
● A. c1, c2, and c3 laminectomy
● B. Suboccipital craniectomy without dural patch grafting
● C. Suboccipital craniectomy with dural patch grafting but with no upper cervical laminectomy
● D. Suboccipital craniectomy with dural patch grafting and upper cervical laminectomy
● E. Suboccipital craniectomy only

Answer 6

D. Suboccipital craniectomy with dural patch grafting and upper cervical laminectomy

Question 7

All of the following are postneurulation defects except?
● A. Microcephaly
● B. Anencephaly
● C. Holoprosencephaly
● D. Lissencephaly
● E. Porencephaly

Answer 7

B. Anencephaly

Question 8

Which of the following is a postneurulation defect that is considered migration abnormality?
● A. Lissencephaly (maldevelopment of cerebral contusion)
● B. Heteropia (abnormal foci of gray matter)
● C. Schizencephaly (a cleft that communicates with ventricle)
● D. Cortical dysplasia (a cleft that does not communicate with ventricles)
● E. All of the above

Answer 8

E. All of the above

Question 9

Following are tests can differentiate hydranencephaly (absence or near-total absence of cerebrum) from maximal hydrocephalus except?
● A. EEG
● B. Occipitofrontal circumference measurement
● C. CT, MRI, or ultrasound
● D. Transillumination of skull
● E. CT brain angiography

Answer 9

B. Occipitofrontal circumference measurement

Question 10

Microcephaly is defined as head circumference more than 2 standard deviations below the mean for sex and gestational age. It is a postneurulation defect. What are the possible causes of this condition?
● A. Infection during pregnancy (rubella, toxoplasmosis, zika virus, cytomegalovirus)
● B. Severe malnutrition
● C. Maternal exposure to certain drugs such as cocaine or alcohol during pregnancy
● D. Interruption of blood supply to brain during pregnancy
● E. All of the above

Answer 10

E. All of the above

Question 11

All of the following are risk factors for macrocrania except?
● A. Lack of prenatal folic acid
● B. Maternal cold exposure from cold water
● C. Use of valproic acid
● D. Obesity
● E. Maternal cocaine abuse

Answer 11

B. Maternal cold exposure from cold water