Neurology Flashcards
Why is child neurology considered dynamic?
- Brain continues to grow and function evolves
- Static lesions produce evolving features
Give examples of types of pathology that can affect children?
- Congenital anomalies
- Neurogenetic diseases and syndromes
- Neurometabolic diseases and syndromes
- Acquired: infection, ischaemia, trauma, tumour
What is included in the neurological consultation in childhood?
- History taking interactive
- Avoid medical jargon
- Time course of symptoms
- Distinguishing static from slowly progressive symptoms can be challenging
- Perinatal, developmental, family history
What is assessed in a developmental history?
- 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
What is looked at in a paediatric neurological examination?
- 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
How common are neurological problems?
- 10% of primary care consultations for children
- 25% of hospitalised children
- 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
What is the epidemiology of headache disorders?
- 40% of children by age 7
- 75% of children by age 15
What are the 4 classifications of headache?
- Isolated acute
- Recurrent acute
- Chronic progressive
- Chronic non-progressive
What do you want to know about the typical episode of chronic headache?
- Any warning?
- Location?
- Severity?
- Duration?
- Frequency?
What should you look at when examining a child due to headache?
- 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
What features would suggest childhood migraine?
- 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
What is the typical presentation of migraine?
- Hemicranial pain
- Throbbing/ pulsatile
- Abdo pain, nausea, vomiting
- Relieved by rest
- Photophobia/ phonophobia
- Visual, sensory, motor aura
- Positive family history
What is the typical presentation of tension headache?
- Diffuse, symmetrical
- Band-like distribution
- Present most of the time (but there may be symptom free periods)
- “Constant ache”
What type of headache would suggest raised intracranial pressure?
- Aggravated by activities that raise ICP eg. Coughing, straining at stool, bending
- Woken from sleep with headache
What type of headache would suggest analgesic overuse headache?
- Headache is back before allowed to use another dose
- Paracetamol/ NSAIDs
- Particular problem with compound analgesics eg. Cocodamol
What are the indications for neuroimaging in headache?
- 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
How is acute migraine treated in children?
Effective pain relief and triptans
What is the preventative treatment for migraines in children?
- Pizotifen
- Propranolol
- Amitryptiline
- Topiramate
- Valproate
How are tension type headaches treated in children?
- 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
Seizure/fit
Any sudden attack from whatever cause
Syncope
Faint ( a neuro-cardiogenic mechanism)
Convulsion
Seizure where there is prominent motor activity
What is a an epileptic seizure due to?
An electrical phenomenon coming form the brain
What does presentation of an epileptic seizure depend on?
- Seizure 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 resource to EEG only supportive evidence
What non-epileptic seizures and mimics can occur in children?
- 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: Non-epileptic attack disorder)
What is the commonest cause of acute symptomatic seizure in childhood
Febrile convulsion
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
What are the different seizure types?
- Jerk/ shake: clonic, myoclonic, spasms
- Stiff: usually a tonic seizure
- Fall: Atonic/ tonic/ myoclonic
- Vacant attack: absence, complex partial seizure
What are epileptic fits chemically triggered by?
- Decreased inhibition (gama-amino-butyric acid, GABA)
- Excessive excitation (glutamate and aspartate)
- Excessive influx of Na and Ca ions
What is the mechanism of epileptic fits?
- Chemical stimulation produces an electrical current
- Summation of a multitude of electrical potentials results in depolarisation of many neurones which can lead to seizures, can be recorded from surface electrodes (Electroencephalogram)
What is a focal seizure?
A seizure which is restricted to one hemisphere of one part of the one hemisphere
What is a generalised seizure?
Abnormally activity in both hemispheres
Why can diagnosis of childhood epilepsies be challenging?
- Non-epileptic paroxysmal disorders are more common in children
- Difficulty in explaining (Children are not young adults)
- Difficulty in interpretation (witness)
- Difficulty in interpretation and synthesising information(physician)
What type of seizures tend to occur in childhood?
Idiopathic and generalised
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 etiology?
- What are the social and educational effects on the child?
What is the effectiveness of interictal EEGs?
- An interictal EEG has limited value in deciding when the individual has epilepsy
- Sensitivity of first routine interictal EEG: 30- 60%
What are the disadvantages of using EEG for diagnosis of fits?
Problematic false positives
- Paroxysmal activity seen in 30%
- Frankly epileptiform activity in 5% of normal children
What are EEGs useful in?
Identifying seizure types, syndrome and aetiology
How are seizures/fits diagnosed?
-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
When should anti-epileptic drugs be considered for use 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
What should be considered when choosing anti-epileptics drugs?
- Age
- Gender
- Type of seizures
- Epilepsy
How are anti-epileptic drugs used in children?
Start with one AED: slow upward titration until side-effects manifest or drug is considered to be inefficient.
What are the possible side effects of anti-epileptic seizures?
- CNS related
- Drowsiness
- Effect on learning
- Cognition and behavioural effects
What is the first line treatment for generalised epilepsies?
Sodium valproate
What is the first line of treatment for focal epilepsies?
Carbamazepine
Other than AED what other therapies can be used in childhood epilepsy?
- Steroids
- Immunoglobulins
- Ketogenic diet (resistant epilepsy)
- Vagal nerve stimulation
- Surgery
What do neuromuscular disorders affect?
Disorders of the peripheral nervous system from the anterior horn cells down
When should you 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
What signs suggest a floppy baby?
- Infant exhibits weakness and flaccidity of all musculature
- Infant hangs like rag doll when lifted under abdomen
- Infant is unable to sit up or hold up head. Head drops back when infant is lifted by its hands
How do muscular dystrophies present?
- Shoulders and arm held back when walking
- Sway back
- Weak buttock muscles
- Knees bend to take weight
- Thick lower leg muscles
- Thick heel cord (may walk on toes)
- Belly sticks out
- Thin weak thighs
- Poor balance
- Foot drop and tip toe contractures
Give examples of neuromuscular conditions which affect the muscles.
- Muscular dystrophies
- Myopathies (congenital and inflammatory)
- Myotonic syndromes
Give an example of a neuromuscular conditions which affects the neuromuscular junction.
Myasthenia syndromes
Give examples of neuromuscular conditions which affect the nerves.
Hereditary or acquired neuropathies
Give an examples of a neuromuscular condition which affects the anterior horn cell.
Spinal muscular atrophy
What are the classical signs of Duchenne muscular dystrophy?
Symmetrical proximal weakness
- Waddling gait
- Calf hypertrophy
- Gower’s sign positive
Delayed gross motor skills
What investigation is done for Duchenne muscular dystrophy?
Creatinine kinase levels
-Elevated >1000 in DMD
What are the complications of Duchenne muscular dystrophy?
- Cardiomyopathy
- Respiratory involvement in teens
What is the incidence of Duchenne muscular dystrophy?
1 in 3,500 male infants
What is the genetic abnormality in Duchene muscular dystrophy?
Xp21 dystrophin gene
What is an epileptic seizure?
- 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
Neuropathy: Site of weakness
Distal weakness
Neuropathy: Reflexes
Lost early
Neuropathy: Fasciculation’s
May be present
Neuropathy: Contractures
Not a feature
Neuropathy: Myocardial dysfunction
Not a typical feature
Neuropathy:Sensory
May have concomitant sensory symptoms and signs
Myopathy: Site of weakness
Usually proximal
Myopathy: Sensory
Pure motor
Myopathy: Reflexes
Preserved till late
Myopathy: Fasciculation’s
Not typical
Myopathy: Contractures
Present
Myopathy: myocardial Dysfunction
May have accompanying cardiac dysfunction with the dystrophies
