Paediatric Neurology

Question 1

How should we approach the neurological history in childhood?

Answer 1

• History taking: interactive
• Hear what was said, not what you thought was said
• Avoid quasi-medical language
• Time course of symptoms crucial
• Distinguishing static from slowly progressive symptoms can be challenging

Question 2

How does one carry out the neurological examination in childhood?

Answer 2

• Opportunistic approach and observation skills
• Appearance
• Gait
• Head size
• Skin findings
• Real world examination (depends on age)

Question 3

List some common paeds neurology and their epidemiology:

Answer 3

• Migraine 7.7% of children 10-17
• Traumatic Brain Injury: 180-300/ 100,000 children
• Tourette syndrome: 1% of all children with high frequency in ADHD and OCD
• Epilepsy: 0.7% of all children, 1/3 will have intractable epilepsy
• Brain tumours: second most common cancer in children

Question 4

What are different types of headache patterns in paeds?

Which ones are more concerning

Answer 4

• Isolated acute
• Recurrent acute
• Chronic progressive
• Chronic non-progressive

Question 5

What headache questions do we need to ascertain?

Answer 5

• Any warning?
• Location?
• Severity?
• Duration?
• Frequency?

Question 6

What are elements of headaches examination?

Answer 6

• Growth parameters, OFC, BP
• Sinuses, teeth, visual acuity
• Fundoscopy
• Visual fields (craniopharyngioma)
• Cranial bruit
• Focal neurological signs
• Cognitive and emotional status

Question 7

Childhood migraine: SSx?

Answer 7

• Associated abdominal pain, nausea, vomiting
• Focal symptoms/ signs before, during, after attack: Visual disturbance, paresthesia, weakness
• ‘Pallor’
• Aggravated by bright light/ noise
• Relation to fatigue/ stress
• Helped by sleep/ rest/ dark, quiet room
• Family history often positive

Question 8

Tension headache: SSx?

Answer 8

• Diffuse, symmetrical
• Band-like distribution
• Present most of the time (but there may be symptom free periods)
• “Constant ache”

Question 9

List the main differences between tension and migraine:

Answer 9

write down and use previous to slides to compare

Question 10

What would suggest raised ICP?

Answer 10

• Aggravated by activities that raise ICP eg. Coughing, straining at stool, bending
• Woken from sleep with headache

Question 11

What would suggest analgesic overuse headache?

Answer 11

• Headache is back before allowed to use another dose
• Paracetamol/ NSAIDs
• Particular problem with compound analgesics eg. Cocodamol

Question 12

What are some indications for neuroimaging?

Answer 12

• Features of cerebellar dysfunction
• Features of raised intracranial pressure
• New focal neurological deficit eg. new squint
• Seizures, esp focal
• Personality change
• Unexplained deterioration of school work

Question 13

Rx for migraine?

Answer 13

• Acute attack: effective pian relief, triptans
• Preventative (at least 1/week):
  
  • Pizotifen, Propranolol, Amitryptyline, Topiramate, Valproate

Question 14

Rx for tension type headache (TTH)?

Answer 14

• Aim at reassurance: no sinister cause
• Multidisciplinary management
• Attention to underlying chronic physical, psychological or emotional problems
• Acute attacks: simple analgesia
• Prevention: Amitryptiline
• Discourage analgesics in chronic TTH

Question 15

What comes under bracket of paroxysmal disorders?

Answer 15

Fits, Faints and Funny-turns

Question 16

What is a convulsion?

Answer 16

Seizure where there is prominent motor activity

Question 17

What is epilepsy?

Answer 17

A tendency to recurrent, unprovoked (spontaneous) epileptic seizures

Question 18

What is an epileptic seizure?

Answer 18

An abnormal excessive hyper synchronous discharge from a group of (cortical) neurons

Question 19

How can we Dx epilepsy?

Answer 19

clinically, with recourse to EEG only for supportive evidence

Question 20

DDx for non-epileptic seizures and other mimics in children?

Answer 20

• Acute symptomatic seizures: due to acute insults eg. Hypoxia-ischaemia, hypoglycemia, infection, trauma
• Reflex anoxic seizure: common in toddlers
• Syncope
• Parasomnias eg. night terrors
• Behavioural stereotypies
• Psychogenic seizures (NEAD)

Question 21

What is febrile convulsion??

Answer 21

• Event occurring in infancy/ childhood, usually between 3 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 22

What are the 4 different seizure types?

Answer 22

• Jerk/ shake: clonic, myoclonic, spasms
• Stiff: usually a tonic seizure
• Fall: Atonic/ tonic/ myoclonic
• Vacant attack: absence, complex partial seizure

Question 23

Pathophysiology of epileptic fit?

Answer 23

• Chemical stimulation produces an electrical current
• Summation of a multitude of electrical potentials results in depolarization of many neurons which can lead to seizures

Question 24

What is an epileptic seizure that only affects a small area of brain vs one that affects whole brain?

Answer 24

Question 25

What are the differences between childhood and adult onset epilepsies?

Answer 25

* Majority are idiopathic in origin (both Focal & Generalised) * Majority of epilepsies are generalised * Seizures can be subtle (absences, myoclonus, drop attacks)

Question 26

What questions should we ask ourselves to diagnose epilepsy?

Answer 26

* Is the paroxysmal event epileptic in nature? * Is it epilepsy? * What seizure types are occurring? * What is the epilepsy syndrome? * What is the etiology? * What are the social and educational effects on the child?

Question 27

What is the role of the EEG?

Answer 27

* An interictal EEG has limited value in deciding when the individual has epilepsy * Useful in identifying seizure types, seizure syndrome and etiology

Question 28

Dx procedure for epilepsy?

Answer 28

* History * Video recording of event * ECG in convulsive seizures * Interictal/ ictal EEG * MRI Brain: to determine etiology eg. Brain malformations/ brain damage * Genetics: idiopathic epilepsies are mostly familial; also single gene disorders eg. Tuberous sclerosis * Metabolic tests: esp if associated with developmental delay/ regression

Question 29

When do we start Mx for epilepsy?

Answer 29

* Only be considered if diagnosis is clear even if this means delaying treatment * Role of drugs is to control seizures not stop epilepsy

Question 30

What is Rx for **epilepsy**?

Answer 30

* Sodium Valproate: first line for generalised epilepsies (not in girls- recent MHRA advice) * Carbamazepine: first line for focal epilepsies

Question 31

When would we suspect NM disorder?

Answer 31

* Baby ‘floppy’ from birth * Slips from hands * Paucity of limb movements * Alert, but less motor activity * Delayed motor milestones * Able to walk but frequent falls

Question 32

Anatomical approach: If affecting muscle: DDx? If affecting neuromuscular junction: DDx? If affecting nerve: DDx? If affecting anterior horn cells: DDx?

Answer 32

* **Muscle**: muscular dystrophies, myopathies- congenital and inflammatory, myotonic syndromes * **Neuromuscular junction**: myasthenic syndromes * **Nerve**: Hereditary or acquired neuropathies * **Anterior Horn Cel**l: Spinal muscular atrophy

Question 33

What is Duchenne muscular dystrophy?

Answer 33

* Xp21, dystrophin gene, 1/3500 male infants * Delayed gross motor skills * Symmetrical proximal weakness * Waddling gait, calf hypertrophy * Gower’s sign positive * Elevated Creatinine Kinase levels * \>1000 in DMD * Cardiomyopathy * Respiratory involvement in teens