Paediatric neurology Flashcards

1
Q

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

A

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

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2
Q

Define chorea

A

sudden jerky movements

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3
Q

Define athetosis

A

abnormal muscle contraction causes involuntary writhing movements

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4
Q

Define tone

A

resistance to passive movement

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5
Q

Define power

A

resistance to active movement

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6
Q

What is myotonia?

A

muscles do not relax, sustained contraction

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

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7
Q

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

A

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

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8
Q

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

A

lissencephaly
sacral dysgenesis

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9
Q

What is lissencephaly?

A

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

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10
Q

What is hypertonia? What causes it?

A

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

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11
Q

What is schizencephaly?

A

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

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12
Q

What can cause dystonic cerebral palsy?

A

kernicterus
unconjugated hyperbilirubinaemia
jaundiced + lethargic

can cross BBB, often affects basal ganglia

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13
Q

Describe the MRC grading of power

A

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

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14
Q

What is Gower’s sign?

A

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

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15
Q

Name 2 signs on Duchenne’s muscular dystrophy

A

calf pseudohypertrophy
Gower’s +ve

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

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16
Q

Biceps reflex nerve roots

17
Q

Supinator reflex nerve roots

18
Q

Triceps reflex nerve root

19
Q

Abdominal reflexes nerve roots

A

T8-T9
T10-T11

20
Q

Knee reflex nerve roots

21
Q

Ankle reflex nerve roots

22
Q

Anal reflex nerve roots

23
Q

How are deep tendon reflexes graded?

A

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

24
Q

What can cause absent deep tendon reflexes?

A

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

25
What can caused increased deep tendon reflexes?
cerebral palsy, hemiplegia, strokes
26
What should you look for on the spine in a paediatric neurological examination?
cutaneous abnormality - midline angioma hairy tuft lipoma sinus (not dimple) scoliosis
27
List some motor deficits in children that are static or progressive
static motor deficit: - dysgenesis - ischaemia - post-infectious - post-haemorrhagic - post-traumatic - metabolic progressive motor deficit: - neoplastic - demyelinating - metabolic - infectious - vascular
28
What signs would be found in an UMN pattern of disease?
increased tone brisk reflexes with clonus pyramidal pattern of weakness babinski positive response
29
What signs would be found in an UMN pattern of disease?
decreased tone wasting fasciculations decreased/absent reflexes
30
Define fasciculations
involuntary rapid muscle twitches that are too weak to move a limb