Paediatric Neurology Flashcards

1
Q

Cerebral palsy - state the following:
- Pathophysiology
- Investigations
- Management (MDT members involved in care)

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

List some potential causes of cerebral palsy
- Antenatal
- Perinatal
- Post natal

A

Antenatal:
- Maternal infection
- Trauma during pregnancy

Perinatal:
- Hypoxia during birth
- Prematurity

Post natal:
- Meningitis
- Severe jaundice
- Head trauma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

State some different types of cerebral palsy, for each type:
- which area of brain affected
- briefly describe presentation

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

State some patterns of cerebral palsy

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

State some complications / associated conditions of cerebral palsy

A
  • Learning disability
  • Hearing and visual impairment
  • Muscle contractures / difficulties moving
  • Epilepsy
  • Kyphoscoliosis
  • GORD
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

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

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Febrile convulsions - state the following:
- Pathophysiology
- Most common age
- Presentation (including any red flags)
- Investigations
- Management

A

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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Outline the difference between a simple febrile seizure and complex febrile seizure

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

State some differential diagnoses for febrile seizures

A
  • Meningitis / encephalitis (other neurological infections)
  • Epilepsy
  • SOL or intracranial haemorrhage
  • Syncopal episode
  • Electrolyte disturbance
  • Trauma / abuse
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Suggest some advice which can be given to parents regarding further febrile seizures once child has been discharged

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Outline the prognosis for febrile convulsions

A

Generally cause no lasting damage

Risk of developing epilepsy:
Normal population = 2%
- Simple febrile seizure = 2-7.5%
- Complex febrile seizure = 10-20%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Epilepsy - state the following:
- Pathophysiology
- Investigations
- Management

A

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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Suggest some situations to be cautious of with patients with epilepsy

A
  • Swimming
  • Baths (showers suggested)
  • At heights
  • Traffic
  • Driving if older
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Outline how to manage status epilepticus

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Suggest 2 drugs that can be used in the community for treatment epileptic seziures (before coming to hospital)

A

Buccal Midazolam
Rectal Diazepam

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

State different types of seizures

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Describe a generalised tonic-clonic seizure and how it is managed

A

Presentation:
- LOC
- Tense muscles (tonic)
- Jerking movements (clonic)
+/- tongue biting, incontinence, groaning
- Prolonged post-ictal period

Management:
- Sodium valproate
- Lamotrigine / Carbamazepine

18
Q

Describe a focal seizure and how it is managed

A

Presentation:
- Start in temporal lobes
- Issues with language, memory or emotions
- Hallucinations, deja vu, memory flashbacks

Management:
- Lamotrigine / Carbamazepine
- Sodium valproate

19
Q

Describe an absence seizure and how it is managed

A

Presentation:
- Patient becomes expressionless and unaware of surroundings

Management:
- Sodium valproate

20
Q

Describe an atonic seizure and how it is managed

A

Presentation:
- Drop attacks, brief lapses in muscle tone
- Less than 3 minutes

Management:
- Sodium valproate
- Lamotrigine

21
Q

Describe a myotonic seizure and how it is managed

A

Presentation:
- Brief muscle contractions
- Awake during attack

Management:
- Sodium valproate
- Lamotrigine

22
Q

Suggest some causes of subdural haemorrhage in the following age groups
- Neonatal
- Infant

A

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

23
Q

Subarachnoid haemorrhage - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management

A

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

24
Q

State some causes of subarachnoid haemorrhage in children

A

Trauma
Non-accidental injury (20%)
Ruptured aneurysm (small %)
Vascular malformation
Paediatric tumors

25
Q

State some differential diagnoses for headaches in children

A

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

26
Q

List some subtypes of migraines

A
  • Migraine with aura
  • Migraine without aura
  • Abdominal migraine
  • Hemiplegic migraine
  • Silent migraine (no headache, just aura)
27
Q

State some ENT infections that could cause a headache

A
  • Otitis media
  • Sinusitis
  • Tonsilitis
  • Viral URTI
28
Q

State the mangement steps for migraine in children, including 3 options for migraine prophylaxis

A
  • 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)

29
Q

Extradural haemorrhage - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management

A

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

30
Q

State some causes of an extradural haemorrhage

A
  • Head injury
  • Non-accidental injury
31
Q

Spinal muscular atrophy - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management

A

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

32
Q

State the 4 types of spinal muscular atrophy and a very brief description of when they start and prognosis

A

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

33
Q

State the type of spinal cord injury most common in paediatrics

A

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

34
Q

State some causes of raised ICP in children

A
  • Traumatic brain injury
  • Brain tumours
  • Intracranial infections
  • Intracranial haemorrhage
  • Severe hypertension
  • Hydrocephalus
  • Hepatic encephalopathy
  • Obstruction to venous CNS outflow
35
Q

State some signs and symptoms of raised ICP

A

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)

36
Q

State how raised ICP is managed

A
  • Regular neurological observations
  • Mannitol or sodium chloride
  • Elevate head to 30° in midline position
  • CT scan after they have been stabilised
    NO lumbar puncture
37
Q

State the 3 most common forms of muscular dystrophy

A
  • Duchenne
  • Becker’s
  • Myotonic
38
Q

Muscular dystrophy - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management

A

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

39
Q

List some causes of visual field defects in children

A
  • Tumor
  • Head injury
  • Meningitis
  • Stroke
  • Cerebral palsy
  • Glaucoma
  • Medications e.g. antiepileptics
40
Q

State some potential underlying causes for a floppy baby (trauma, congenital and other)

A

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

41
Q

Erbs palsy - state the following:
- Pathophysiology
- Presentation
- Investigations
- Management

A

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

42
Q

State the prognosis for Erb’s palsy

A

Up to 80% recover fully

Lack of full recovery by 3 months = likely to have residual impairment