Paediatric Neurology Flashcards
types of pathology causing neurological issues in children
• Congenital • Neurogenetic diseases and syndromes • Neurometabolic diseases and syndromes • Acquired o Infection o Ischaemia o Trauma o Tumour
describe the neurologicl consultation in children
- 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
describe a developmental hx
- 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
describe the neurological examination in children
- 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
what % of hospitalised children have a neurological conditions
25%
what percent of childre aged 10-17 have migraines?
7.7.%
what are the first and second most common cancers in children?
leukaemia
brain tumours
by age 7 and 15 what % of children have had a headache
40%
75%
most parents who seek help for a child with a headache are looking for what?
reassurance that it is not due to a serious cause usually brain tumour
4 ways to describe the onset of a headache
- Isolated acute
- Recurrent acute
- Chronic progressive
- Chronic non-progressive
draw out the different types of headache in a child
see notes
recurrent or chronic headache in children hx
Is there more than 1 type of headache? Typical episode: • Any warning • Location • Severity • Duration • Frequency
headache examination in children
- 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
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
migraine vs tension headache
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
features of raised intracranial pressure
Aggravated by activities that raise ICP e.g. coughing. Woken from sleep with headache
features of analgesic overuse headache
- Headache is back before allowed to use another dose
- Paracetamol/ NSAIDs
- Particular problem with compound analgesics e.g. Cocodamol
indications for neuroimaging in children with headaches
- 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
management of migraine in children
- Acute attack: effective pain relief, triptans
* Preventative (at least 1/week): Pizotifen, Propranolol, Amitriptyline, Topiramate, Valproate
management of TTH 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
define seizure/fit
any sudden attack from whatever cause
define syncope
faint - a neuro-cardiogenic mechanism
define convulsion
seizure where there is prominent motor activity
define epileptic seizure
an abnormal excessive hypersyncronous discharge from a group of usually cortical neurones
what do clinical features of an epileptic seiure depend on?
- Paroxysmal change in motor, sensory or cognitive function
* Depends on seizure’s location, degree of anatomical spread over cortex, duration
define epilepsy
a tendency to recurrent unprovoked epileptic seizures
examples of non-epileptic seizures in children
• 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)
define 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
types of seizure
- 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
mechanism of an epileptic seizure
• 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)
childhood vs adult onset epilepsies
- Majority are idiopathic in origin (both Focal & Generalised)
- Majority of epilepsies are generalised
- Seizures can be subtle (absences, myoclonus, drop attacks)
why can a diagnosis of epilepsy be difficult?
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
stepwise approach to a diagnosis of 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?
role of the EEG in 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
diagnosis of epilepsy in children
• 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
management of epilepsy in 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
• 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)
when to suspect a neuromuscular disorder
- 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
types of neuromuscular disorders
- Muscle: muscular dystrophies, myopathies- congenital and inflammatory, myotonic syndromes
- Neuromuscular junction: myasthenic syndromes
- Nerve: Hereditary or acquired neuropathies
- Anterior Horn Cell: Spinal muscular atrophy
DMD: gene
Xp21, dystrophin gene
DMD: signs
• 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
