Paediatric neurology Flashcards
What is the epidemiology of headache disorders?
In about 40% children by age 7, 75% of children by age 15
What are the different types of headache patterns and which ones are investigated?
- Isolated acute (investigated)
- Recurrent acute
- Chronic progressive (investigated)
- Chronic non-progressive
What should be asked in the history of a child with a headache disorder?
- Is there more than 1 type of headache?
- Typical episode:
□ Any warning?
□ Location?
® Headaches located at the back of the head are more concerning
□ Severity?
□ Duration?
□ Frequency?
What examination should be done on a child with headache disorder?
- Growth parameters, OFC, BP
□ The head growing too fast may mean excessive CSF and hydrocephalus
□ If the child isn’t growing well then it could be a indication of a brain tumour - Sinuses, teeth, visual acuity
- Fundoscopy
- Visual fields (craniopharyngioma)
- Cranial bruit
□ Put a stethoscope on a child’s temporal area and listen for bruits - Focal neurological signs
- Cognitive and emotional status
What is a primary headache?
Tension headache and migraine
What are the pointers to childhood migraine?
- 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
- Hemicranial pain
- Throbbing/ pulsatile
What are the pointers to tension headache in children?
- Diffuse symmetrical
- Band like distribution
- Present most of the time but there may be symptom free periods
- “constant ache”
True or false: It is rare to have mixed migraine and tension type headache
False: they are common
What are the pointers to raised intercranial pressure in children?
- Aggravated by activities that raise ICP e.g. Coughing, straining at stool, bending
- Woken from sleep with headache
What are the pointers to analgesic overuse headache in children?
- Headache is back before allowed to use another dose
- Paracetamol/ NSAIDs
- Particular problem with compound analgesics e.g. Cocodamol
What are the indications for neuroimmaging?
- 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
What is the management of migraine in children?
□ Acute attack: effective pain relief, triptans
□ Preventative (at least 1/week): Pizotifen, Propranolol, Amitriptyline, Topiramate, Valproate
What is the management of tension type headache in children?
□ 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
What is a seizure/ fit?
Any sudden attack from whatever cause
What is syncope?
Faint (a neuro-cardiogenic mechanism)
What is a convulsion?
Seizure where there is prominent motor activity
What is an epileptic siezure?
- An abnormal excessive hyper synchronous discharge from a group of (cortical) neurons
- It may have clinical manifestations
- Paroxysmal change in motor, sensory or cognitive function
- Depends on seizure’s location, degree of anatomical spread over cortex, duration
What is epilepsy?
- A tendency to recurrent, unprovoked (spontaneous) epileptic seizures
- A question that must be answered clinically, with recourse to EEG only for supportive evidence
- A seizure is not necessarily epileptic
- Consequences of misdiagnosis of epilepsy can be serious
What are the mechanisms of an epileptic fit?
□ Chemically triggered by:
® Decreased inhibition (gama-amino-butyric acid, GABA)
® Excessive excitation (glutamate and aspartate)
® 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)
What is the stepwise approach to diagnosing epilepsy?
□ 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?
What is the role of EEG in diagnosing epilepsy?
® 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
How is epilepsy dianosed in children?
® History
® Video recording of event
® ECG in convulsive seizures
◊ Can get epilepsy with arrhythmias
® 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
What should be considered when thinking about giving anti-epileptc drugs to children?
- 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
What are the antiepiletic drugs (and other therapies) that are used in children?
□ 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: Levatiracetam, Lamotrigine, Perampanel
□ Other therapies: steroids, immunoglobulins and ketogenic diet (mostly for resistant epilepsies), surgical procedures
What is a vagus nerve stimulator?
- Used in the treatment of childhood epilepsy
□ Sends tiny electrical shocks to the vagus nerve
□ 3 pieces of equipment used in VNS
® Programmable generator
® Lead with two coils at the end
® Hand held magnet
What are the different types of non-epileptic seiures and other mimics in children?
- Acute symptomatic seizures: due to acute insults e.g. Hypoxia-ischaemia, hypoglycaemia, infection, trauma
- Reflex anoxic seizure: common in toddlers
□ Triggers: pain, fright - Syncope
- Parasomnias e.g. night terrors
- Behavioural stereotypies
□ Pre-school aged children
□ Repetitive behaviours e.g. rocking in a corner or head banging - Psychogenic seizures (NEAD)
What is a febrile convulsion?
- 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
- Commonest cause of ‘acute symptomatic seizure’ in childhood
What are the different siezure types?
- Jerk/ shake: clonic, myoclonic, spasms
- Stiff: usually a tonic seizure
- Fall: Atonic/ tonic/ myoclonic
- Vacant attack: absence, complex partial seizure
What is microcephaly?
- Definition:
□ OFC <2 SD: mild
□ OFC <3 SD: moderate/ severe - Microcephaly usually indicates small brain: ‘microencephaly’
- Prenatal or postnatal onset: is it crossing centiles downwards?
- Timing of onset may be a clue
- Multiple causes: antenatal, postnatal, genetic and environmental
What is macrocephaly?
- Definition: OFC > 2SD
- Is it crossing centiles upwards?
- Sutures?
- Fontanelles?
- Familial?
- Hydrocephalus?
- Large brain?
- Development normal?
- Other physical abnormalities – facial features, hepatosplenomegaly, bony deformities etc
What should you think about when there are head shape problems in a child?
- Often in first year of life
- Timing of onset?
- Is it getting better or worse?
- Plagiocephaly ‘flat-head’
- Brachycephaly ‘short head or flat at back’
- Scaphocephaly ‘boat shaped skull’
- Craniosynostosis
When should you suspect neuromuscular disorders in a child?
- 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
- Not being able to hold its head up by 3 months
- Girdle sign- demonstrates pelvic girdle weakness
What is myopathic facies?
Muscle problem- the child has droopy eyelids and cannot close mouth, poor head control
What is myotonia?
difficulty in relaxing muscles (common in congenital myotonia and myotonia dystrophies)
What is Pes cavis?
high foot arch and hammer toes- Charcot-Marie-tooth disorder
What is Duchene muscular dystophy?
- 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
What is the managemnt of Duchene muscular dystrophy?
Steroids can prolong life (they can now live until late 20s to early 30s)
