Paediatric Neurology

Question 1

What is done in a headache examination?

Answer 1

Growth parameter, OFC, BP, sinuses, teeth, visual acuity, fundoscopy, visual fields, cranial bruit, focal neurological sign and cognitive/ emotional status

Question 2

What are pointers to childhood migraines?

Answer 2

Associated with abdominal pain and N/V
Focal symptoms/ signs before/ during/ after, visual disturbance, paraesthesia and weakness
Relation to stress and fatigue
Aggravated by bright light and noise

Question 3

What are features of migraine?

Answer 3

Hemicranial pain, throbbing/ pulsatile, abdo pain, N/V, relieved by rest, photophobia/ phonophobia, positive FH and aura

Question 4

What are some features of tension headaches?

Answer 4

Diffuse and symmetrical
Band-like distribution
Present most of the time and constant ache

Question 5

What are signs of raised intracranial pressure?

Answer 5

Aggravated by activities - coughing, straining and bending
Woken from sleep with headache and possible vomiting

Question 6

What are signs of analgesic overuse headache?

Answer 6

Headache is back before allowed to use another dose
Paracetamol and NSAIDs
Particular problem with compound analgesics - co-codamol

Question 7

What are the indicators for neuroimaging in headaches?

Answer 7

Features of cerebellar dysfunction, raised ICP, new focal neurological deficit, seizures, personality change and unexplained deterioration in school work

Question 8

What is the management for migraine in children?

Answer 8

Acute attack - effective pain relief or triptans
Preventative - pizotifen, propranolol, amitriptyline, topiramate and valproate

Question 9

What is the management of tension type headache in children?

Answer 9

Aim at reassurance
Acute attacks - simple analgesia
Prevention - amitriptyline
Discourage analgesics in chronic TTH

Question 10

What is a convulsion?

Answer 10

Seizure where there is prominent motor activity

Question 11

What is an epileptic seizure?

Answer 11

An electrical phenomenon
Abnormal excessive hyper synchronous discharge from a group of neurons
Paroxysmal change in motor, sensory or cognitive function
May have clinical manifestations

Question 12

Describe epilepsy

Answer 12

A tendency to recurrent, unprovoked spontaneous epileptic seizures
Recourse to EEG

Question 13

What are some non-epileptic seizures and other mimics in children?

Answer 13

Acute symptomatic seizure - due to acute insult
Reflex anoxic seizure - common in toddlers
Syncope
Parasomnias
Behavioural stereotypes
Psychogenic non-epileptic seizures (PNES)

Question 14

Describe febrile convulsions

Answer 14

A seizure occurring in infancy/ childhood, usually between 2 months and 5 years of age
Associated with fever but without evidence of intracranial infection or defined cause for the seizure
Commonest cause of acute symptomatic seizure in childhood

Question 15

What is the mechanism of epileptic fits?

Answer 15

Chemically triggered by decreased inhibition, excessive excitation and excessive influx of Na and Ca ions
Chemical stimulation produces an electrical current - summation of multitude of electrical potentials results in depolarisation of multiple neurons

Question 16

What are the types of epileptic seizures?

Answer 16

Partial and generalised seizures

Question 17

What is the differences in adult vs childhood epileptic seizures?

Answer 17

Majority are idiopathic and generalised in childhood
Seizures can be subtle and diagnosis can be challenging

Question 18

What is the role of the EEG?

Answer 18

Has limited value in deciding when the individual has epilepsy
Useful in identifying seizure types, seizure syndrome and aetiology
Sensitivity of first routine - 30-60%
Problematic false positive rates

Question 19

How is epilepsy diagnosed?

Answer 19

History, video of event occurring, ECG in convulsion seizures, EEG, MRI brain, genetics and metabolic tests

Question 20

What is the management of epilepsies in children?

Answer 20

Anti-epileptics only considered if diagnosis is clear
Aim is to control seizures
Start with one anti-epileptic
Side effects - CNS related, drowsiness, effect on learning, cognition and behavioural

Question 21

What is the drug treatment of epilepsy?

Answer 21

Sodium valproate - not in girls - Levetiracetam
Carbamazepine - focal epilepsies
Levetiracetam and lamotrigine
Other therapies - steroids, immunoglobulins and ketogenic diet
Epilepsy surgery - vagal nerve stimulator

Question 22

What can be some head size problems?

Answer 22

Macrocephaly and microcephaly

Question 23

What is part of the normal skull of the infant?

Answer 23

Metopic, coronal, sagittal and lambdoid sutures
Anterior and posterior fontanelle

Question 24

What is the order of fontanelle closure?

Answer 24

Posterior closes first at around 2-3 months after birth and anterior closes between 1-3 years of age

Question 25

How is head circumference measured?

Answer 25

Occipitofrontal circumference As routine - birth to 3 years Look at facial features, fontanelles and shape of head Measure and plot parental head sizes

Question 26

Describe microcephaly

Answer 26

OFC <2SD - mild OFC <3SD - moderate to severe Usually indicates small brain - microencephaly Timing of onset may be a clue Causes - antenatal, post-nantal, genetic and environmental

Question 27

Describe macrocephaly

Answer 27

OFC >2SD Check if the crossing centiles move upwards, sutures, fontanelles, hydrocephalus and large brain Other abnormalities - facial features, hepatosplenomegaly and bony features

Question 28

What is plagiocephaly?

Answer 28

Flat head

Question 29

What is scaphocephaly and brachycephaly?

Answer 29

Scaphocephaly - boat shaped skull Brachycephaly - short head or flat at the back

Question 30

Describe plagiocephaly

Answer 30

Flat head - deformational Asymmetry seen Positional defect More prominent in first year in life

Question 31

Describe craniosynostosis

Answer 31

Sutures have fused too early Some conditions can cause suture to close Can have characteristic head shapes - indicates the type CT scans and skull X-rays

Question 32

When is a neuromuscular disorder suspected?

Answer 32

baby can be floppy after birth, slips from hands, paucity of limb movements, alert but less motor activity, delayed motor milestones, and able to walk but lots of falls

Question 33

Describe Duchenne muscular dystrophy

Answer 33

Xp21, dystrophin gene and male infants Delayed gross motor skills, symmetrical proximal weakness, elevated creatinine kinase levels, cardiomyopathy and respiratory involvement in teens

Question 34

What are some clinical features of Duchenne muscular dystrophy?

Answer 34

Waddling gait, hold their shoulder back, side to side sway, prominent abdomen, hyperextension of knees and walking on toes Balance is poor

Question 35

What are some other neuromuscular disorders?

Answer 35

Myopathy facies - weakness, power and tone of face muscles Myotonia - inability of muscle to relax after contraction Paskowitz - high arch of the feet

Question 36

What is the anatomical approach to neuromuscular conditions?

Answer 36

Muscle - muscular dystrophies, myopathies-congenital and inflammatory, myotonic syndromes Neuromuscular junction - myasthenic syndromes Nerve - hereditary or acquire neuropathies Anterior horn cell - spinal muscular atrophy

Question 37

What are features of neuropathy?

Answer 37

Distal weakness, may have sensory symptoms + signs, reflexes lost early and fasciculations may be present

Question 38

What are features of myopathy?

Answer 38

Usually proximal weakness, pure motor, reflexes preserved until later, contractures present and may have accompanying cardiovascular problem