Neuro

Question 1

What are the causes of polymicrogyria

Answer 1

1. In utero infection especially CMV 2. In utero Ischaemia 3. Genetic causes

Question 2

What are the clinical manifestations of polymicrogyria

Answer 2

Developmental delay Quadriparesis or Intractable seizures

Question 3

What are the two forms of lissencephaly

Answer 3

Type 1 - classical Type 2 - cobblestone

Question 4

What is type 2 lissencephaly associated with?

Answer 4

Cerebellar and ocular malformation and muscular dystrophy - walker Warburg syndrome

Question 5

The classic form of lissencephaly is characterised by …..

Answer 5

Hour glass or figure of 8

Question 6

The congenital CC anomalies are often associated with other midline structures including ……….. Or ………

Answer 6

Midline lipoma Interhemispheric cyst

Question 7

Schizencephaly is associated with …

Answer 7

Septo- optic dysplasia which is agenesis of septum pellucidum and optic nerve hypoplasia

Question 8

Causes of cortical cleft interruption (4)

Answer 8

Schizencephaly- lined by grey matter Porencephaly - replacement of cortex by cystic structures Encephalomalacia Surgical resection cavity

Question 9

In babies , chiari 1 is associated with ……. , ……… (2)

Answer 9

Sleep apnoea Feeding problems

Question 10

NF1 manifestations

Answer 10

Cafe au lait Peripheral nerve sheath tumour CNS malignancies - optic nerve glioma Bone abnormalities - sphenoid wing dysplasia

Question 11

NF1 association with brain neoplasms

Answer 11

Optic nerve glioma Juvenile pilocystic astrocytoma Brain stem glioma

Question 12

Bone manifestations of NF1

Answer 12

Sphenoid wing dysplasia - pulsating enophthalmus or exophthalmos Posterior vertebral body scalloping Rib notching Focal gigantism Cervical kyphoscoliosis Neural foramina enlargement

Question 13

Eye manipulation of NF1

Answer 13

Hamartomas of the iris known as Lisch nodules

Question 14

Extra cranial manifestations of NF1

Answer 14

Wilm’s tumour Rhabdomyosarocoma AML - renal Leiomyosarcoma

Question 15

Manifestations of NF2

Answer 15

MISME Multiple inherited Schwannoma ( bilateral is diagnostic) Meningioma Ependymoma

Question 16

Characteristic of Sturge Weber

Answer 16

Facial port wine stain Occular abnormalities Failure of normal cortical venous development.

Question 17

The port wine stain involves the forehead and upper eyelid corresponding to the region innervated by ………….

Answer 17

Ophthalmic branch of trigeminal nerve

Question 18

Classical triad of TS

Answer 18

Nodular rash in nasolabial fold Seizure Mental retardation

Question 19

Extra cranial manifestations of TS

Answer 19

Renal : multiple angiomyolipoma Cardiac : Rhabdomyoma Lung : LAM

Question 20

When I say molar tooth configuration, you say….

Answer 20

Joubert syndrome and related disorder