Paediatric neurology

Question 1

What should you look for when doing a paediatric neurological examination?

Answer 1

Muscle bulk: atrophy, hypertrophy
Tongue fasciculations
Abnormal spontaneous movements: chorea, tremor, athetosis, dystonia

Question 2

Define chorea

Answer 2

sudden jerky movements

Question 3

Define athetosis

Answer 3

abnormal muscle contraction causes involuntary writhing movements

Question 4

Define tone

Answer 4

resistance to passive movement

Question 5

Define power

Answer 5

resistance to active movement

Question 6

What is myotonia?

Answer 6

muscles do not relax, sustained contraction

congenital myotonic dystrophy = floppy baby - trinucleotide repeat expansion so worse in subsequent generations

Question 7

List some common causes of hypotonia based on the area it is caused by

Answer 7

CNS:
- chromosome disorders eg. Prader-Willi, trisomy-21
- metabolic diseases
- cerebral dysgenesis
- hypoxic-ischaemic injuries

Motor neuron:
- spinal muscular atrophies (anterior horn cell disease)

Nerve:
- congenital hypomyelinating neuropathy
- familial dysautonomia
- infantile neuraxonal degeneration

Neuromuscular junction:
- congenital and transient MG
- infantile botulism

Muscle:
- muscular dystrophies
- metabolic myopathies
- central core disease/fibre myopathies

Question 8

Name 2 causes of hypotonia that are due to primary neural tube defect

Answer 8

lissencephaly
sacral dysgenesis

Question 9

What is lissencephaly?

Answer 9

smooth brain
no grey-white differentiation
no gyri + sulci present

Question 10

What is hypertonia? What causes it?

Answer 10

due to UMN pathology
increased tone
spasticity
ankle clonus
upgoing plantar reflex/Babinski test +ve

Question 11

What is schizencephaly?

Answer 11

one side of brain hasn’t developed, therefore opposite side of body is weak

Question 12

What can cause dystonic cerebral palsy?

Answer 12

kernicterus
unconjugated hyperbilirubinaemia
jaundiced + lethargic

can cross BBB, often affects basal ganglia

Question 13

Describe the MRC grading of power

Answer 13

0 = no movement
1 = flicker
2 = movement with gravity eliminated
3 = movement against gravity but without resistance
4 = movement against resistance
5 = normal power

Question 14

What is Gower’s sign?

Answer 14

uses arms to push up from seated position to standing
proximal muscle weakness

Question 15

Name 2 signs on Duchenne’s muscular dystrophy

Answer 15

calf pseudohypertrophy
Gower’s +ve

(Becker’s = milder version of Duchenne’s)

Question 16

Biceps reflex nerve roots

Answer 16

C5, C6

Question 17

Supinator reflex nerve roots

Answer 17

C5, C6

Question 18

Triceps reflex nerve root

Answer 18

C7

Question 19

Abdominal reflexes nerve roots

Answer 19

T8-T9
T10-T11

Question 20

Knee reflex nerve roots

Answer 20

L3, L4

Question 21

Ankle reflex nerve roots

Answer 21

S1, S2

Question 22

Anal reflex nerve roots

Answer 22

S4, S5

Question 23

How are deep tendon reflexes graded?

Answer 23

0 = absent
+/- = obtainable with reinforcement
+ = normal
++ = increased
+++ = increased with clonus

Question 24

What can cause absent deep tendon reflexes?

Answer 24

(LMN pathology)
SMA, Guillain-Barre, demyelinating diseases

Question 25

What can caused increased deep tendon reflexes?

Answer 25

cerebral palsy, hemiplegia, strokes

Question 26

What should you look for on the spine in a paediatric neurological examination?

Answer 26

cutaneous abnormality - midline angioma
hairy tuft
lipoma
sinus (not dimple)
scoliosis

Question 27

List some motor deficits in children that are static or progressive

Answer 27

static motor deficit:
- dysgenesis
- ischaemia
- post-infectious
- post-haemorrhagic
- post-traumatic
- metabolic

progressive motor deficit:
- neoplastic
- demyelinating
- metabolic
- infectious
- vascular

Question 28

What signs would be found in an UMN pattern of disease?

Answer 28

increased tone
brisk reflexes with clonus
pyramidal pattern of weakness
babinski positive response

Question 29

What signs would be found in an UMN pattern of disease?

Answer 29

decreased tone
wasting
fasciculations
decreased/absent reflexes

Question 30

Define fasciculations

Answer 30

involuntary rapid muscle twitches that are too weak to move a limb