Paediatric Neurology

Question 1

types of pathology causing neurological issues in children

Answer 1

• Congenital
• Neurogenetic diseases and syndromes
• Neurometabolic diseases and syndromes
• Acquired
o Infection
o Ischaemia
o Trauma
o Tumour

Question 2

describe the neurologicl consultation in children

Answer 2

• 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
• Perinatal, developmental, family history

Question 3

describe a developmental hx

Answer 3

• Motor milestones: gross and fine motor skills
• Speech and language development
• Early cognitive development
• Play esp. symbolic play and social behaviour
• Self-help skills
• Vision and hearing assessment

Question 4

describe the neurological examination in children

Answer 4

• Opportunistic approach and observation skills
• Appearance
• Gait
• Head size
• Skin findings
• Real world examination (depends on age)
• Synthesis of history and clinical findings into a differential diagnosis and investigation plan

Question 5

what % of hospitalised children have a neurological conditions

Answer 5

25%

Question 6

what percent of childre aged 10-17 have migraines?

Answer 6

7.7.%

Question 7

what are the first and second most common cancers in children?

Answer 7

leukaemia

brain tumours

Question 8

by age 7 and 15 what % of children have had a headache

Answer 8

40%

75%

Question 9

most parents who seek help for a child with a headache are looking for what?

Answer 9

reassurance that it is not due to a serious cause usually brain tumour

Question 10

4 ways to describe the onset of a headache

Answer 10

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

Question 11

draw out the different types of headache in a child

Answer 11

see notes

Question 12

recurrent or chronic headache in children hx

Answer 12

Is there more than 1 type of headache?
Typical episode:
• Any warning
• Location
• Severity
• Duration
• Frequency

Question 13

headache examination in children

Answer 13

• Growth parameters, OFC, BP
• Sinuses, teeth, visual acuity
• Fundoscopy
• Visual fields (craniopharyngioma)
• Cranial bruit
• Focal neurological signs
• Cognitive and emotional status
• The diagnosis of headache aetiology is clinical

Question 14

pointers to childhood migraine

Answer 14

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

Question 15

migraine vs tension headache

Answer 15

Migraine Tension Headache
Hemicranial pain Diffuse, symmetrical
Throbbing/pulsatile Band-like distribution
Abdo pain, N+V Present most of the time but there may be
symptom free periods
Relieved by rest Constant ache
Photophobia/phonophobia
Visual, sensory, motor aura
Positive family history

Question 16

features of raised intracranial pressure

Answer 16

Aggravated by activities that raise ICP e.g. coughing. Woken from sleep with headache

Question 17

features of analgesic overuse headache

Answer 17

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

Question 18

indications for neuroimaging in children with headaches

Answer 18

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

Question 19

management of migraine in children

Answer 19

• Acute attack: effective pain relief, triptans

* Preventative (at least 1/week): Pizotifen, Propranolol, Amitriptyline, Topiramate, Valproate

Question 20

management of TTH in children

Answer 20

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

Question 21

define seizure/fit

Answer 21

any sudden attack from whatever cause

Question 22

define syncope

Answer 22

faint - a neuro-cardiogenic mechanism

Question 23

define convulsion

Answer 23

seizure where there is prominent motor activity

Question 24

define epileptic seizure

Answer 24

an abnormal excessive hypersyncronous discharge from a group of usually cortical neurones

Question 25

what do clinical features of an epileptic seiure depend on?

Answer 25

* Paroxysmal change in motor, sensory or cognitive function | * Depends on seizure’s location, degree of anatomical spread over cortex, duration

Question 26

define epilepsy

Answer 26

a tendency to recurrent unprovoked epileptic seizures

Question 27

examples of non-epileptic seizures in children

Answer 27

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

Question 28

define febrile convulsion

Answer 28

An 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

Question 29

types of seizure

Answer 29

* Distinguishing seizure types can be challenging * Jerk/ shake: clonic, myoclonic, spasms * Stiff: usually a tonic seizure * Fall: Atonic/ tonic/ myoclonic * Vacant attack: absence, complex partial seizure

Question 30

mechanism of an epileptic seizure

Answer 30

• Chemically triggered by: o Decreased inhibition (gamma-amino-butyric acid, GABA) o Excessive excitation (glutamate and aspartate) o Excessive influx of Na and Ca ions • Chemical stimulation produces an electrical current • Summation of a multitude of electrical potentials results in depolarization of many neurons which can lead to seizures, can be recorded from surface electrodes (Electroencephalogram)

Question 31

childhood vs adult onset epilepsies

Answer 31

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

Question 32

why can a diagnosis of epilepsy be difficult?

Answer 32

o Non-epileptic paroxysmal disorders are more common in children o Difficulty in explaining (Children are not young adults) o Difficulty in interpretation (witness) o Difficulty in interpretation and synthesising information(physician

Question 33

stepwise approach to a diagnosis of epilepsy

Answer 33

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

Question 34

role of the EEG in epilepsy

Answer 34

* An interictal EEG has limited value in deciding when the individual has epilepsy * Sensitivity of first routine interictal EEG: 30- 60% * Problematic false positive rates: paroxysmal activity seen in 30%, frankly epileptiform activity in 5% of normal children * Useful in identifying seizure types, seizure syndrome and aetiology

Question 35

diagnosis of epilepsy in children

Answer 35

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

Question 36

management of epilepsy in children

Answer 36

• Anti-epileptic drugs (AED) should only be considered if diagnosis is clear even if this means delaying treatment • Role of AED is to control seizures, not cure the epilepsy • Start with one AED: slow upward titration until side-effects manifest or drug is considered to be inefficient. • Age, gender, type of seizures and epilepsy should be considered in selecting AEDs • S/Es: CNS related can be detrimental; Drowsiness, effect on learning, cognition and behavioural • Sodium Valproate: first line for generalised epilepsies (not in girls- recent MHRA advice) • Carbamazepine: first line for focal epilepsies • Several new AEDs with more tolerability and fewer side effects: Levetiracetam, Lamotrigine, Perampanel • Other therapies: steroids, immunoglobulins and ketogenic diet (mostly for resistant epilepsies)

Question 37

when to suspect a neuromuscular disorder

Answer 37

* 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 38

types of neuromuscular disorders

Answer 38

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

Question 39

DMD: gene

Answer 39

Xp21, dystrophin gene

Question 40

DMD: signs

Answer 40

``` • Delayed gross motor skills • Symmetrical proximal weakness o Waddling gait, calf hypertrophy o Gower’s sign positive • Elevated Creatinine Kinase levels o >1000 in DMD • Cardiomyopathy • Respiratory involvement in teens ```