Paediatric Neurology 2

Question 1

What is nusinersen for?

Answer 1

• SMA

- promotes more production of SMN2 protein

Question 2

What is cerebellopontine angle syndrome and what is the cause?

Answer 2

• Unilateral impairment CN 5, 7, 8 2ndary vestibular schwannoma
• Unilateral hearing loss, nystagmus/vertigo, Bell’s palsy

Question 3

What are the possible causes of Horner syndrome?

Answer 3

• Brachial plexus injury
• Lesions in midbrain, brainstem, upper spinal cord, neck, midline fossa or orbit (e.g. tumour)
• Post thoracic surgery, e.g. CHD
• Neuroblastomas.

Question 4

What are the typical findings in SMA?

Answer 4

Tongue fasciculations and hypotonia

Question 5

What portion of the tongue is affected in Bell’s Palsy?

Answer 5

• Anterior 2/3, taste

Question 6

How does Botox work?

Answer 6

• Inhibits the release of acetylcholine

- Decreases endplate potential which causes impaired neuromuscular and autonomic transmission

Question 7

Which epilepsy syndrome presents with episodic eye deviation progressing to altered awareness, then followed by pallor and vomiting ~20‐30 minutes?

Answer 7

• Benign occipital epilepsy

Question 8

What do you think of in a child who is ‘floppy-strong’?

Answer 8

• Chromosomal issues e.g. trisomy 21
• Metabolic syndromes
• CNS problems

Question 9

What do you think of in a child who is ‘floppy-weak’?

Answer 9

• Neuromuscular disease

Question 10

Which antiepileptic is predominantly excreted unchanged in the urine?

Answer 10

• Gabapentin
• Renal elimination and a half life of 5-7 hours. It does not have significant pharmacokinetic drug interactions and is not significantly protein bound
• Levetiracetam also 90% renal

Question 11

Diplopia with two images most separated when looking to the right. When looking to the right with the left eye covered, the more medial image disappears. The nerve involved is the:

Answer 11

R abducens
R eye can’t move right - because MEDIAL image disappears (i.e. right lateral rectus muscle is broken) which is innervated by the right abducens nerve (CN VI)

Question 12

A seven-year-old boy presents to you with a six-month history of toe walking which worsens over the course of the day. He has left equinovarus foot deformity on examination and increased tone in his lower limbs. The most likely diagnosis is:

Answer 12

Segawa disease (Dopa responsive dystonia)

Question 13

What is the typical presentation of Guillian Barre?

Answer 13

Guillain-Barre syndrome present with ascending paralysis, loss of reflexes, loss of sensation, and weakness, but no urinary retention.

Question 14

How might poliomyelitis present?

Answer 14

Poliomyelitis is a disease of the anterior horn motor neurons of the spinal cord and brain stem caused by poliovirus. Flaccid asymmetric weakness and muscle atrophy due to loss of motor neurons and denervation of their associated skeletal muscles. Reflexes are initially brisk and then absent. Fasciculations are often observed.

Question 15

What is the typical presentation of transverse myelitis?

Answer 15

Inflammation of spinal cord with loss of function over hours-weeks. Sudden onset of lower back pain, muscle weakness, abnormal sensation with possible progression to paralysis, urinary retention, and loss of bowel control. Reflexes will be reduced.

Question 16

What are some NON protein bound psychotropic drugs?

Answer 16

• lithium (0%)
• methylphenidate (10 – 30%)
• venlafaxine (25 – 30%)
• gabapentin (0 – 3%)
• topiramate

Question 17

When do 99% of normal children walk by and what factors predict a good outcome?

Answer 17

• “sitting on air” posture (hips flexed and abducted and knees extended, when held in vertical suspension), predicts a good outcome
• 5% never crawl
• 18 months is when you expect walking to have happened
• Idiopathic delay is more common in females
• family history of shuffling, a non-revealing prenatal and perinatal history, a developmental pattern of delayed learning to sit, and the presence of shuffling favour a normal outcome

Question 18

What increases the half life of lamotrigine? Decreases?

Answer 18

Increases - valproate. Decreases - Carbamazepine

Question 19

What is the potency of dexamethasone vs. methylpred vs. pred vs. hydrocortisone?

Answer 19

If hydrocortisone = 1
pred =4
Methylpred = 5
Dex = 30

Question 20

What cranial nerves control which eye movements?

Answer 20

• Opening eyes - levator palpebrae, oculomotor (III)
• Closing eyes - facial nerve (VII)
• Cross eyes downwards - superior oblique, trochlear (IV)
• Lateral/out - abducens (VI)
• III does everything else!!

Question 21

What’s the difference between a pituitary adenoma and craniopharyngioma presentation-wise?

Answer 21

• adenoma causes overgrowth

- craniopharyngioma causes pituitary dysfunction/growth failure and headaches.

Question 22

A 12-year-old girl presents with a one-year history of an increasing unsteady gait and slurred speech. There is no family history of note. On examination she has extensor plantar responses with absent ankle and knee reflexes. Her examination is otherwise normal. The most likely diagnosis is:

Answer 22

Freiderich’s ataxia

Question 23

What are Piaget’s developmental stages?

Answer 23

Sensorimotor Stage: Birth through about 2 years. Children learn about the world through their senses and the manipulation of objects.

Preoperational Stage: Ages 2 through 7. Developing memory and imagination. They are also able to understand things symbolically, and to understand the ideas of past and future.

Concrete Operational Stage: Ages 7 through 11. Awareness of external events, as well as others’ feelings. Less egocentricity, beginning to understand that not everyone shares their thoughts, beliefs, or feelings.

Formal Operational Stage: Ages 11 and older. Use logic to solve problems, view the world, and plan for the future.

Question 24

What are Erikson’s developmental stages?

Answer 24

1. Trust vs. Mistrust Hope 0 - 1½
2. Autonomy vs. Shame Will 1½ - 3
3. Initiative vs. Guilt Purpose 3 - 5
4. Industry vs. Inferiority Competency 5 - 12
5. Identity vs. Role Confusion Fidelity 12 - 18

Question 25

Which antiepileptics are highly protein bound?

Answer 25

• Valproate 95%
• Phenytoin 90%
• Benzos 85%