Paediatric Neurology Flashcards
Cerebral palsy - state the following:
- Pathophysiology
- Investigations
- Management (MDT members involved in care)
Pathophysiology:
- Umbrella term for a permanent neuro-developmental problem (non-progressive condition) caused by damage to the brain tissue
- Huge variety in type and severity of symptoms
Investigations:
Typically clinically
- May need to rule out other conditions
- CT / MRI can help support diagnosis or identify another neurological cause
Management:
MDT approach
- Physical therapy / occupational therapy
- SALT team / dieticians
- Surgery
- Paediatrician for regular reviews
- Social workers
List some potential causes of cerebral palsy
- Antenatal
- Perinatal
- Post natal
Antenatal:
- Maternal infection
- Trauma during pregnancy
Perinatal:
- Hypoxia during birth
- Prematurity
Post natal:
- Meningitis
- Severe jaundice
- Head trauma
State some different types of cerebral palsy, for each type:
- which area of brain affected
- briefly describe presentation
Spastic:
- Damage to upper motor neurones
- Leading to hypertonia
Dyskinetic:
- Damage to the basal ganglia (initiate and control movements)
- Problems controlling muscle tone, with hypotonia and hypertonia
Ataxic:
- Damage to the cerebellum
- Leading to uncoordinated movements
Mixed:
- Mix of spastic, dyskinetic and ataxic features
State some patterns of cerebral palsy
Monoplegia: one limb affected
Hemiplegia: one side of body affected
Diplegia: all limbs affected, mostly legs
Quadriplegia: all limbs affected more severely, often with seizures, speech disturbance and other impairments
State some complications / associated conditions of cerebral palsy
- Learning disability
- Hearing and visual impairment
- Muscle contractures / difficulties moving
- Epilepsy
- Kyphoscoliosis
- GORD
For the following MDT members, suggest how they help in managing cerebral palsy
- Physical therapy / occupational therapy
- SALT team / dieticians
- Surgery
- Paediatrician for regular reviews
- Social workers
Physical therapy / occupational therapy:
- Stretch and strengthen muscles
- Maximise function
- Manage daily activities
SALT team / dieticians:
- Help if uncoordinated swallowing, may need NG or PEG
- Ensure that their dietary requirements are being met
Surgery:
- Release contracture (tenotomy)
Paediatricians:
- Regular reviews
- Medicines e.g. muscle relaxants, anti-epileptic drugs
Social workers:
- Social support
Febrile convulsions - state the following:
- Pathophysiology
- Most common age
- Presentation (including any red flags)
- Investigations
- Management
Pathophysiology:
- Subtype of seizure that occurs in children with a high fever
- No underlying neurological pathology or epilepsy
- Either simple febrile seizure or complex febrile seizure
Most common age:
- By definition, only occur between 6 months old and 5 years
Presentation:
- Simple or complex febrile seizure alongside a fever
- Tonic clonic pattern, either focal or generalised
- Can last < or > 15 minutes
Investigations:
Diagnosis of exclusion = need to rule out other pathology e.g. EEG, CT scan
Management:
Key: identify source of infection
- Control fever with Paracetamol and Ibuprofen
- Complex febrile seizures may require further investigations (simple generally reassurance)
Outline the difference between a simple febrile seizure and complex febrile seizure
Simple febrile seizure:
- Generalised
- Tonic clonic
- < 15 minutes in duration
- Only occur once during a period of high temp
Complex febrile seizure:
- Focal / partial
- > 15 minutes in duration
- Can occur multiple times during a period of high temp
State some differential diagnoses for febrile seizures
- Meningitis / encephalitis (other neurological infections)
- Epilepsy
- SOL or intracranial haemorrhage
- Syncopal episode
- Electrolyte disturbance
- Trauma / abuse
Suggest some advice which can be given to parents regarding further febrile seizures once child has been discharged
- Stay with child
- Lay them on the floor, pillow under their head, away from objects
- Don’t put anything in their mouth
- Call an ambulance if not stopped within 5 minutes
Outline the prognosis for febrile convulsions
Generally cause no lasting damage
Risk of developing epilepsy:
Normal population = 2%
- Simple febrile seizure = 2-7.5%
- Complex febrile seizure = 10-20%
Epilepsy - state the following:
- Pathophysiology
- Investigations
- Management
Pathophysiology:
- Umbrella term for tendency to have seizures
- Seizures are transient episodes of abnormal electrical brain activity
Investigations:
- EEG
- Consider MRI brain
- Consider to exclude other pathology e.g. ECG, blood glucose
Management:
- Generally safety advice to parents (including advice on status epilepticus)
- Anti-epileptics (ideally 1 drug)
Suggest some situations to be cautious of with patients with epilepsy
- Swimming
- Baths (showers suggested)
- At heights
- Traffic
- Driving if older
Outline how to manage status epilepticus
ABCDE approach
A - Secure airway
B - high flow oxygen
C - cannula, check cardiac and resp function
D - check glucose
E - x
IV Lorazepam, repeat after 10 mins if seizure continues
If persist, Phenytoin or Phenobarbitol
Then escalate to ITU with intubation
Suggest 2 drugs that can be used in the community for treatment epileptic seziures (before coming to hospital)
Buccal Midazolam
Rectal Diazepam
State different types of seizures
Generalised tonic-clonic:
- LOC
- Tense muscles (tonic)
- Jerking movements (clonic)
+/- tongue biting, incontinence, groaning
- Prolonged post-ictal period
Focal:
- Start in temporal lobes
- Issues with language, memory or emotions
- Hallucinations, deja vu, memory flashbacks
Absence:
- Patient becomes expressionless and unaware of surroundings
Atonic:
- Drop attacks, brief lapses in muscle tone
- Less than 3 minutes
Myoclonic:
- Brief muscle contractions
- Awake during attack
Infantile spasms (West syndrome):
Rare (poor prognosis)
- Clusters of full body spasms
- Starts around 6 months old
Describe a generalised tonic-clonic seizure and how it is managed
Presentation:
- LOC
- Tense muscles (tonic)
- Jerking movements (clonic)
+/- tongue biting, incontinence, groaning
- Prolonged post-ictal period
Management:
- Sodium valproate
- Lamotrigine / Carbamazepine
Describe a focal seizure and how it is managed
Presentation:
- Start in temporal lobes
- Issues with language, memory or emotions
- Hallucinations, deja vu, memory flashbacks
Management:
- Lamotrigine / Carbamazepine
- Sodium valproate
Describe an absence seizure and how it is managed
Presentation:
- Patient becomes expressionless and unaware of surroundings
Management:
- Sodium valproate
Describe an atonic seizure and how it is managed
Presentation:
- Drop attacks, brief lapses in muscle tone
- Less than 3 minutes
Management:
- Sodium valproate
- Lamotrigine
Describe a myotonic seizure and how it is managed
Presentation:
- Brief muscle contractions
- Awake during attack
Management:
- Sodium valproate
- Lamotrigine
Suggest some causes of subdural haemorrhage in the following age groups
- Neonatal
- Infant
Neonatal:
- Traumatic birth
- Clotting issues e.g. thrombocytopenia, vitamin K deficiency, inherited haemophilia
- Maternal use of Aspirin (cross placenta)
Infants:
- Head trauma
- Clotting issues e.g. thrombocytopenia, vitamin K deficiency, inherited haemophilia
- CNS infections e.g. congenital toxoplasmosis, bacterial meningitis
- Connective tissue disorders e.g. Ehlers-Danlos syndrome
Always consider non-accidental injuries & shaken baby syndrome
Subarachnoid haemorrhage - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management
Pathophysiology:
- Bleeding in the subarachnoid space
- Between the pia mater and the arachnoid membrane (into CSF fluid)
Presentation:
Younger
- Irritability
- Lethargy
- Vomiting / seizures
- Poor feeding
- Increased / decreased tone
- Impaired consciousness
Older children
- Similar to adults e.g. thunderclap headache, N&V, decreased consciousness, seizures and focal neurological deficits
Investigations:
- CT head
- If positive CT head, digital subtraction angiography
Management:
- Vasospasm prophylaxis
- May not need surgery
- Surgery within 3 days
State some causes of subarachnoid haemorrhage in children
Trauma
Non-accidental injury (20%)
Ruptured aneurysm (small %)
Vascular malformation
Paediatric tumors
State some differential diagnoses for headaches in children
Primary headaches:
- Tension headache
- Migraine
Secondary headaches:
- Raised ICP
- Brain tumour
- Meningitis / encephalitis
- ENT infection
Other:
- Headache from analgesia
- Carbon monoxide poisoning
- Secondary to vision issues
List some subtypes of migraines
- Migraine with aura
- Migraine without aura
- Abdominal migraine
- Hemiplegic migraine
- Silent migraine (no headache, just aura)
State some ENT infections that could cause a headache
- Otitis media
- Sinusitis
- Tonsilitis
- Viral URTI
State the mangement steps for migraine in children, including 3 options for migraine prophylaxis
- Rest in a dark quiet room
- Fluids
- Analgesia (Paracetamol or Ibuprofen)
- Sumatriptan
- Anti-emetics e.g. Domperidone
Prophylaxis:
- Propranolol
- Pizotifen (serotonin receptor blocker)
- Topiramate (anti-epileptic = teratogenic)
Extradural haemorrhage - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management
Pathophysiology:
- Accumulation of blood between the inner table of the cranium and dura
- Usually the result of a tear to middle meningeal artery
Presentation:
Younger
- Irritability
- Lethargy
- Vomiting / seizures
- Poor feeding
- Increased / decreased tone
- Impaired consciousness
Older children
- Similar to adults e.g. headache followed by lucid interval, N&V, decreased consciousness, seizures and focal neurological deficits
Investigations:
- CT head
Management:
- Surgery to remove haematoma +/- craniotomy to relieve pressure
State some causes of an extradural haemorrhage
- Head injury
- Non-accidental injury
Spinal muscular atrophy - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management
Pathophysiology:
- Autosomal recessive condition
- Causes progressive loss of neurones, leading to muscular weakness
- Affects LMNs in the spinal cord
Presentation:
- Muscle weakness / lethargy (reduced power)
- Fasciculations
- Reduced tone
- Reduced / absent reflexes
Investigations:
- Molecular genetic testing (consider in any hypotonic or floppy baby)
Management:
MDT approach
- Physiotherapy to improve muscle strength and respiratory function
- Consider non-invasive ventilation if required
- Type 1 (severe) may need a tracheostomy or PEG
State the 4 types of spinal muscular atrophy and a very brief description of when they start and prognosis
Type 1: most severe
- Develops within months of life
- Death by 2 years
Type 2:
- Onset in first 1-2 years
- Can’t walk but survive into adulthood
Type 3:
- Onset after 1 year only
- Can walk but life significantly affected, can lose ability to walk over time
- Respiratory muscles less affected
- Can expect a normal life expectancy
Type 4: least severe
- Onset not until early 20’s
- Can walk but life significantly affected
- Respiratory muscles not affected
- Can expect a normal life expectancy
State the type of spinal cord injury most common in paediatrics
Larger head to body ratio
Increased elasticity of spinal ligament capsules
< 8 years = most commonly above C4 (car crashes, falls and child abuse
> 8 years, injuries at C5 to C8 (car crashes, sports injuries)
= predispose them to hyper mobility of the spine without apparent bony injury
State some causes of raised ICP in children
- Traumatic brain injury
- Brain tumours
- Intracranial infections
- Intracranial haemorrhage
- Severe hypertension
- Hydrocephalus
- Hepatic encephalopathy
- Obstruction to venous CNS outflow
State some signs and symptoms of raised ICP
Symptoms:
- Irritability
- Altered consciousness
- Blurred / altered vision
Signs:
- Severe hypertension
- Focal neurological signs
- Bulging fontanelle
- Unequal pupils
- Abnormal eye movement
- Seizures
- Reduced GCS</= 8
- Papilloedema (late sign)
State how raised ICP is managed
- Regular neurological observations
- Mannitol or sodium chloride
- Elevate head to 30° in midline position
- CT scan after they have been stabilised
NO lumbar puncture
State the 3 most common forms of muscular dystrophy
- Duchenne
- Becker’s
- Myotonic
Muscular dystrophy - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management
Pathophysiology:
- X-linked recessive mutation in the dystrophin protein (which joins muscles)
- Initially causes muscle regeneration
- However over time, there is muscle atrophy (with cell death and fat accumulation in the muscle)
- Also affects the heart due to dystrophin
Presentation:
- Progressive proximal muscle weakness
- Waddling gait
- Calf pseudohypertrophy
- Gower’s sign
- Wheelchair in later stages
Investigations:
- Creatine kinase
- Muscle biopsy
- Genetic testing
Management:
- MDT approach, mainly PT/OT
- Oral corticosteroids
- Creatine supplements
List some causes of visual field defects in children
- Tumor
- Head injury
- Meningitis
- Stroke
- Cerebral palsy
- Glaucoma
- Medications e.g. antiepileptics
State some potential underlying causes for a floppy baby (trauma, congenital and other)
Trauma:
- Brain damage / cerebral malformation e.g. HIE
- Head trauma
- Spinal cord injury
- Intracranial haemorrhage
Congenital:
- Muscular disorders
- Neuromuscular disorders e.g. myasthenia gravis, spinal muscular atrophy
- Connective tissue disorders
- Chromosomal disorders
Other:
- Severe infections
- Hypoglycaemia
Erbs palsy - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management
Pathophysiology:
- Injury to the C5/C6 nerves in the brachial plexus during birth
- Associated with shoulder dystocia and large birth weight or traumatic / instrumental delivery
Presentation:
‘Waiter’s tip’ appearance
- Internally rotated shoulder (poor external rotation)
- Extended elbow (poor elbow flexion)
- Flexed wrist (poor wrist extension)
- Flexed fingers (poor finger extension)
Investigations:
- Nerve conduction studies
Management:
- Early referral to a BPBI clinic
- PT / OT input
- May require reconstructive surgery
State the prognosis for Erb’s palsy
Up to 80% recover fully
Lack of full recovery by 3 months = likely to have residual impairment
