Paediatric neurology Flashcards
What should you look for when doing a paediatric neurological examination?
Muscle bulk: atrophy, hypertrophy
Tongue fasciculations
Abnormal spontaneous movements: chorea, tremor, athetosis, dystonia
Define chorea
sudden jerky movements
Define athetosis
abnormal muscle contraction causes involuntary writhing movements
Define tone
resistance to passive movement
Define power
resistance to active movement
What is myotonia?
muscles do not relax, sustained contraction
congenital myotonic dystrophy = floppy baby - trinucleotide repeat expansion so worse in subsequent generations
List some common causes of hypotonia based on the area it is caused by
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
Name 2 causes of hypotonia that are due to primary neural tube defect
lissencephaly
sacral dysgenesis
What is lissencephaly?
smooth brain
no grey-white differentiation
no gyri + sulci present
What is hypertonia? What causes it?
due to UMN pathology
increased tone
spasticity
ankle clonus
upgoing plantar reflex/Babinski test +ve
What is schizencephaly?
one side of brain hasn’t developed, therefore opposite side of body is weak
What can cause dystonic cerebral palsy?
kernicterus
unconjugated hyperbilirubinaemia
jaundiced + lethargic
can cross BBB, often affects basal ganglia
Describe the MRC grading of power
0 = no movement
1 = flicker
2 = movement with gravity eliminated
3 = movement against gravity but without resistance
4 = movement against resistance
5 = normal power
What is Gower’s sign?
uses arms to push up from seated position to standing
proximal muscle weakness
Name 2 signs on Duchenne’s muscular dystrophy
calf pseudohypertrophy
Gower’s +ve
(Becker’s = milder version of Duchenne’s)
Biceps reflex nerve roots
C5, C6
Supinator reflex nerve roots
C5, C6
Triceps reflex nerve root
C7
Abdominal reflexes nerve roots
T8-T9
T10-T11
Knee reflex nerve roots
L3, L4
Ankle reflex nerve roots
S1, S2
Anal reflex nerve roots
S4, S5
How are deep tendon reflexes graded?
0 = absent
+/- = obtainable with reinforcement
+ = normal
++ = increased
+++ = increased with clonus
What can cause absent deep tendon reflexes?
(LMN pathology)
SMA, Guillain-Barre, demyelinating diseases
What can caused increased deep tendon reflexes?
cerebral palsy, hemiplegia, strokes
What should you look for on the spine in a paediatric neurological examination?
cutaneous abnormality - midline angioma
hairy tuft
lipoma
sinus (not dimple)
scoliosis
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
What signs would be found in an UMN pattern of disease?
increased tone
brisk reflexes with clonus
pyramidal pattern of weakness
babinski positive response
What signs would be found in an UMN pattern of disease?
decreased tone
wasting
fasciculations
decreased/absent reflexes
Define fasciculations
involuntary rapid muscle twitches that are too weak to move a limb
