Primary Intracranial Anomalies

Question 1

What is the Galassi type of a small, biconvex arachnoid cyst located in anterior temporal tip causing no mass effect and communicating with subarachnoid space on water-soluble contrast CT cisternogram (WS-CTC)?
● A. 0
● B. I
● C. II
● D. III
● E. IV

Answer 1

B. I

Question 2

“Bobble-head doll syndrome” is repetitive anteroposterior head movements usually associated with expansile lesions in the region of the third ventricle. What is the recommended treatment for suprasellar arachnoid cysts?
● A. Endoscopic third ventriculostomy
● B. Ventriculoperitoneal shunt
● C. Bilateral external ventricular drains
● D. Neuroendoscopic ventriculocystocisternostomy
● E. Biopsy followed by radiotherapy

Answer 2

D. Neuroendoscopic ventriculocystocisternostomy

Question 3

Excluding suprasellar cysts, what is the definitive treatment for intracranial arachnoid cysts?
● A. Needle aspiration
● B. Burr hole evacuation
● C. Craniotomy, excising cyst wall and fenestrating it into basal cisterns
● D. Endoscopic cyst fenestration through a burr hole
● E. Shunting of cyst into peritoneum or into vascular system

Answer 3

E. Shunting of cyst into peritoneum or into vascular system

Question 4

Which one is true about hypothalamic hamartoma (HH)?
● A. HH is the least common cause of precocious puberty
● B. Pedunculated and sessile are the two subtypes
● C. Stereotactic radiosurgery used for large sessile type or for near-total resection
● D. Precocious puberty failing to respond to medicine is not an indication of surgery
● E. Endoscopic app used for lesion > 1.5 cm in diameter

Answer 4

B. Pedunculated and sessile are the two subtypes

Question 5

Which one is false about agenesis of corpus callosum?
● A. It may present as an incidental finding
● B. Patient presents with hydrocephalus and seizures
● C. Failure of comissuration occurs at > 3 weeks of conception
● D. There is separation of lateral ventricles
● E. There may be absence of anterior or posterior CC

Answer 5

C. Failure of comissuration occurs at < 3 weeks of gestation

Question 6

Craniofacial dysmorphic syndromes are due to mutations in which gene?
● A. FGFR
● B. p53
● C. Chromosome 19
● D. WNT signaling pathway
● E. EFGR

Answer 6

A. FGFR

Question 7

Bilateral arachnoid cyst may occur in which of the following?
● A. Hunter syndrome
● B. Hurler syndrome
● C. Neurofibromatosis type 2
● D. Neurofibromatosis type 1
● E. von Hippel lindau syndrome

Answer 7

B. Hurler syndrome

Question 8

Which of the following is the most common craniosynostosis (CSO) affecting a single suture producing a palpable keel-like sagittal ridge and dolichocephaly (elongated skull with high forehead/frontal bossing)?
● A. Sagittal CSO
● B. Coronal CSO
● C. Metopic CSO
● D. Lambdoid CSO
● E. Multiple synostoses

Answer 8

A. Sagittal CSO

Question 9

Which is true about plagiocephaly?
● A. Premature ossification of lambdoid and sagittal suture
● B. It is always bilateral
● C. Positional plagiocephaly is associated with anterior displacement of ear
● D. Posterior plagiocephaly is associated with anterior displacement of ear
● E. Children can be managed conservatively for up to 1 year

Answer 9

C. Positional plagiocephaly is associated
with anterior displacement of ear

Question 10

Which of the following is the only group of encephaloceles which does not produce a visible soft tissue mass and may present as CSF leak or recurrent meningitis?
● A. Occipital
● B. Cranial vault
● C. Frontoethmoidal
● D. Basal
● E. Posterior fossa

Answer 10

D. Basal

Question 11

Mastoid process and air cells form at what age?
● A. 2 years and 5 years
● B. 2 years and 6 years
● C. 3 years and 6 years
● D. 2 years and 7 years
● E. 1 year

Answer 11

B. 2 years and 6 years

Question 12

In which of the following vermis is intact?
● A. Dandy–Walker malformation (DWM) and Dandy–Walker variant (DWV)
● B. Black pouch cyst
● C. Retrocerebellar arachnoid cyst and cisterna magna
● D. Joubert’s syndrome
● E. All of the above

Answer 12

C. Retrocerebellar arachnoid cyst and cisterna magna

Question 13

What is the rate of occurrence of hydrocephalus in DWM?
● A. 2 to 4%
● B. 75 to 95%
● C. 50 to 70%
● D. 10%
● E. 30 to 40%

Answer 13

B. 75 to 95%

Question 14

A mother brought her 2 months old child with pointed shaped head. What is this type of craniosynostosis?
● A. Sagittal CSO
● B. Coronal CSO
● C. Metopic CSO
● D. Lambdoid CSO
● E. Multiple synostoses

Answer 14

C. Metopic CSO

Question 15

Hypoplasia of the optic nerves and optic chiasm is seen in which of the following?
● A. Hurler syndrome
● B. de Morsier syndrome
● C. Hypothalamic hamartomas
● D. DWV
● E. BPC

Answer 15

B. de Morsier syndrome

Question 16

Arachnoid cyst is a congenital fluid containing abnormality of the arachnoid membrane which is most common in the middle fossa, CPA, suprasellar region, and posterior fossa. According to the classification scheme of Galassi et al for middle fossa cysts all of the following are true except?
● A. Type 1 is a small biconvex cyst located at temporal tip
● B. Type 2 involves proximal and intermediate segments of the middle fossa
● C. Type 3 involves the entire sylvian fissure
● D. Type 4 involves agenesis of the temporal horn
● E. None of the above

Answer 16

D. Type 4 involves agenesis of the tempo-
ral horn

Question 17

What are the different treatment options of arachnoid cyst?
● A. Drainage by needle aspiration or burr hole evacuation
● B. Craniotomy with excision of cyst wall and fenestrating it into basal cisterns
● C. Shunting of cyst into peritoneum
● D. Endoscopic cyst fenestration through burr hole
● E. All of the above

Answer 17

E. All of the above

Question 18

Anterior fontanelle (the largest fontanelle, diamond shaped, 4 × 2.5 cm in size) and posterior fontanelle (small, irregular in shape) normally close, respectively, by what age?
● A. 2 years and 3 to 4 months
● B. 2.5 years and 2 to 3 months
● C. 2 years and 1 to 2 months
● D. 2.5 years and 4 to 5 months
● E. None of the above

Answer 18

B. 2.5 years and 2 to 3 months

Question 19

Diploe appears in skull bone by the age of 2 years and reach maximum (when diploic veins form) by what age?
● A. 25 years
● B. 30 years
● C. 35 years
● D. 40 years
● E. None of the above

Answer 19

C. 35 years

Question 20

Craniosynostosis is premature ossification of a cranial suture. The diagnosis of craniosynostosis is aided by all of the following except?
● A. On palpation by palpating bony prominence or gentle firm pressure with thumbs fail to cause relative movement of bones
● B. Plain skull X-ray will show lack of normal lucency or beaten copper calvaria
● C. Plain CT of brain demonstrates thickening or ridging at synostosis
● D. Technetium bone scan should be used in questionable cases
● E. MRI demonstrates thickening of bone and ridging

Answer 20

E. MRI demonstrates thickening of bone and ridging

Question 21

The most common craniosynostosis (CSO) affecting a single suture which produces a palpable ridge and reduced biparietal diameter is known as dolichocephaly or scaphocephaly. CSO of which suture causes this abnormality?
● A. Sagittal suture
● B. Coronal suture
● C. Lambdoid suture
● D. Both coronal and sagittal sutures
● E. None of the above

Answer 21

A. Sagittal suture

Question 22

Coronal synostosis can accompany with Crouzon’s syndrome or Apert’s syndrome with unilateral coronal synostosis producing anterior plagiocephaly. Which of the following are the signs in case of unilateral coronal synostosis?
● A. Forehead on the affected side flattened or concaved with compensatory prominence of contralateral forehead
● B. Harlequin eye sign on the affected side (supraorbital margin is higher than the normal side)
● C. Orbit rotates out on the affected side producing amblyopia
● D. Deviation of nose toward the normal side (the root of the nose tends to rotate toward the deformity)
● E. All of the above

Answer 22

E. All of the above

Question 23

It is important to distinguish lazy lambdoid (positional flattening) from posterior plagiocephaly. What are the distinguishing features between the two?
● A. Posterior plagiocephaly produces a trapezoid-shaped deformity, while the other produces a parallelogram
● B. The ipsilateral ear is displaced posteriorly in posterior plagiocephaly, while it is displaced anteriorly in the other one
● C. There is contralateral frontal bossing in posterior plagiocephaly, while it is ipsilateral frontal bossing in postural plagiocephaly
● D. Postural plagiocephaly is associated with decreased mobility, abnormal posture, or intentional positioning
● E. All of the above

Answer 23

E. All of the above

Question 24

Encephalocele (cranium bifidum) is a defect in the fusion of the cranial bone through which either meninges and CSF herniate (meningocele) or meninges and cerebral tissue protrude (encephalocele). What is the most common type of encephalocele?
● A. Occipital
● B. Cranial vault
● C. Frontoethmoidal
● D. Basal
● E. Posterior fossa

Answer 24

A. Occipital

Question 25

Dandy Walker malformation is a congenital defect with classic triad of absence or hypoplasia of cerebellar vermis, cystic dilatation of the 4th ventricle, and enlarged posterior fossa. All of the following are included in the differential diagnosis except?
● A. Cerebellar hamartomal cyst
● B. Persistent Blake’s pouch
● C. Epidermoid cyst
● D. Retrocerebellar arachnoid cyst
● E. Joubert’s syndrome

Answer 25

A. Cerebellar hamartomal cyst