Paeds Neuro + misc Flashcards
How old does a child need to be to get a intramedullary nail?
4
What is a reflex anoxic seizure?
Episodes of tonic clonic fitting due to cardiac asystole secondary to vagal inhibition
What are the triggers of reflex anoxic seizure?
Pain
Head trauma
Cold food (ice cream)
Fright
Fever
What feature of a reflex anoxic seizure differentiates it from epilepsy?
No tongue biting
What is a febrile convulsion?
A seizure + fever in the absence of CNS infection
What is the age group that can be affected by febrile convulsions?
6m to 6y
Commonest form of childhood seizure up to age 2y
Give 4 features of simple febrile seizures
Generalised tonic clonic seizure
<15 mins
Complete recovery within 1h
DONT recur within 24h/ same febrile illness
What is a generalised tonic clonic seizure?
Muscle stiffening followed by rhythmical jerking or shaking of the limbs, which may be asymmetrical
Rolling back of eyes
Staring
Loss of consciousness
Give 4 features of complex febrile seizures
Partial (Focal) seizure
> 15 mins
Recurrence within 24h/ in same illness
Incomplete recovery wthin 1h
What is a partial onset/ focal seizure?
movement limited to 1 side of body/ 1 limb
What investigations should be done following a febrile seizure?
Exclude RFs: CNS infection
Hx of illness + identify cause
No other main Ix (don’t do an EEG)
Consider: blood glucose, urine dip + MC+S if no clear focus
How should febrile convulsion be managed during the seizure?
Start timer
Protect from injury + do not restrain
Check airway + recovery position when stopped
If a febrile convulsion lasts longer than 5 minutes, what should be done?
Call an ambulance
OR
If drugs are available: buccal midazolam or PR diazepam
If ongoing after 10 mins, repeat dose + cal ambulance
Which children should be admitted to hospital following a febrile convulsion? (5)
1st febrile seizure
<18 months old
Diagnostic uncertainty about cause
Features of recurrent Complex febrile seizure
Currently on Abx (may mask CNS infection)
What advice should be provided following a febrile seizure?
Risk of developing epilepsy is low
1/3 will have another febrile seizure (risk reduces with age)
Antipyretics don’t prevent recurrence
Ensure child completes immunisations (even if these were followed by seizure)
What system is used to classify seizures?
International League Against Epilepsy 2017
What are the different types of seizure?
Generalised
Non-motor: absence
Motor: Tonic clonic, Myoclonic, Atonic, Tonic
Focal
Aware: simple partial. Conscious, aware + remember
Impaired awareness: complex partial. Consciousness affected, may hear but not understand/ respond
What is the alternate name for an absence seizure? What are these characterised by?
Generalised non-motor seizure
person becomes blank + unresponsive for a few seconds
What is the difference in symptoms between tonic-clonic and myoclonic?
Tonic clonic: violent muscle contractions, eyes rolling back, tongue biting, incontinence and both aura + post-ictal phenomena
Myoclonic = brief arrhythmic muscular jerking movement
What is the most common form of childhood epilepsy?
Benign Rolandic Epilepsy - myoclonic seizures in 3-12y
What are the 3 different types of myoclonic epilepsy
Benign rolandic (3-12y)
Juvenile myoclonic epilepsy (12-18y)
Progressive myoclonic epilepsy
What is the other name for benign rolandic seizures?
Sylvian seizures
What are the signs and symptoms of benign rolandic epilepsy?
Often occur at night
Typically partial seizures involving face + UL with hypersalivation
What is seen on EEG in benign rolandic epilepsy?
Centrotemporal spikes
What is the prognosis for benign rolandic epilepsy?
seizures stop by adolescence
What are the signs and symptoms of juvenile myoclonic epilepsy?
Myoclonic seizures involving upper body just after waking up + beginning after puberty
What is progressive myoclonic epilepsy?
Rare syndromes of combination of myoclonic + tonic-clonic seizures, with patient deteriorating over time
For what type of epilepsy is treatment not usually given?
Benign Rolandic Epilepsy
What type of antiepileptic exacerbates myoclonic seizures?
Lamotrigine
What type of antiepileptic actually exacerbates absence seizures?
Carbamazapine
What is the 1st line for rescue therapy for prolonged epileptic seizures?
Buccal midazolam
Which antiepleptic drug requires monitoring?
Carbamazapine
After how long seizure-free can antiepileptic therapy be stopped?
2 years
Recall 2 Side effects of valporate
Weight gain
Hair loss
How is status epilepticus defined?
1 seizure lasting >5 mins OR
>2 seizures within a 5 min period without the person returning to normal between them
What is the age range of peak incidence for infantile spasm?
3-8 months
What is West syndrome?
Specific type of epilepsy characterised by infantile spasms, age of onset + EEG pattern
What is an infantile spasm?
Sudden, rapid, tonic contraction of trunk + limb muscles with gradual relaxation over 0.5-2s
Give 3 characteristics of West syndrome
‘salaam’ attacks: flexion of the head, trunk + arms followed by extension of the arms
Lasts only 1-2s but may be repeated up to 50x
Progressive mental handicap
What is a ‘Salaam’ attack?
Head goes down + arms go up in the air
What is seen on EEG in West Syndrome?
Hypsarrhythmia
What is the prognosis for West syndrome?
Poor
Most have learning difficulties
Most go onto to develop Lennox-gastaut syndrome
How should West syndrome be managed?
Guidance from paediatric neurologist within 24h
Urgent assessment by paediatric neurologist
Vigabatrin + Prednisolone
What investigations should be done for vasovagal syncope?
LSBP with ECG if indicated
FBC (to r/o anaemia/ bleeding)
What are the indications for CT head in children?
Head injury + at least 1 of:
- suspected NAI
- post-traumatic seizure
- GCS <14
- Suspected skull fracture
- Focal neurology
OR
2 other risk factors
(LOC >5 mins, abnormal drowsiness, >3 episodes of vomiting, high-impact injury, amnesia > 5 mins)
Describe the pathophysiology of extradural haemorrhage
Low impact trauma to pterion overlying the middle meningeal artery
How does extradural haemorrhage manifest in a patient?
- Initial LOC
- Briefly regains consciousness= ‘Lucid interval’
- LOC due to expanding haematoma + brain herniation
Give 2 signs of tear of the MMA
Battle sign
Racoon eyes
What are the signs of extradural haemorrhage on MRI?
Biconvex (or lentiform), hyperdense collection around surface of the brain
Limited by suture lines of skull.
“Lemon”
How should extradural haemorrhage be managed?
Fluid resuscitation to correct hypovolaemia
Evacuation of haematoma + arrest bleeding
What is the cause of subdural haemorrhage?
Shearing in vein as it crosses subdural space
Bleed into space between dura + arachnoid membrane
What are 8 symptoms of subdural in a child?
Bruises, esp to head + neck
Apnoea or Irregular breathing
Seizures
Altered GCS
Lethargy
Poor feeding
Cardiorespiratory collapse or sudden death.
Vomiting
What is often the cause of subdural in infants?
NAI
Which infants are most at risk of intraventricular haemorrhage?
Premature babies due to VLBW
What are 2 causes of intraventricular haemorrhage in infants?
ECMO in preterm babies with ARDS
Congenital CMV infection
ECMO = extracorporeal membrane oxygenation
Recall 5 signs and symptoms of intraventricular haemorrhage
Sleepiness + lethargy
Apnoea
Reduced Moro reflex
Hypotonia
Tense fontanelle
Which investigation is used to diagnose intraventricular haemorrhage in a baby?
Trans-fontanelle USS
How should intraventricular haemorrhage be treated in a baby?
Fluids
Anticonvulsant
Acetazolamide (to reduce CSF) and LP
Ventriculo-peritoneal shunt if hydrocephalus
What is the difference between communicating and non-communicating hydrocephalus?
Communicatig: CSF flow obstructed after it enters the ventricles
Non-communicating: flow of CSF is obstructed WITHIN the ventricles
What are the 2 causes of communicating hydrocephalus?
Meningitis
SAH
What are the possible causes of non-communicating hydrocephalus?
Aqueduct stenosis:
Congenital causes: Dandy-Walker malformation or Chiari malformation
Acquired: IVH/ tumour
What is the sunset sign?
Eyes appear to be driven down bilaterally - sign of raised ICP
What are the 2 key investigations for hydrocephalus?
Cranial USS
Measurement of head circumference
What is the first line management for hydrocephalus?
Ventriculoperitoneal shunt
What is the second line management for hydrocephalus?
Furosemide - to inhibit CSF production
What % of migraines have aura?
10%
Is migraine likely to be UL or BL?
Could be either
What are the symptoms of cluster headaches?
UL (eyes/ side of face) sharp throbbing pain, causing swelling/ watering inclusters
What are some signs that migraine is secondary to something more sinister?
Visual field defects
Gait or CN abnormalities
Growth failure
Papilloedema
Early morning headache
If simple analgesia doesn’t work, what is the 2nd line pain med in migraines?
Nasal sumatriptan
Recall 2 prophylactic medications for migraine
Topiramate
Propranolol
What is Tourette’s?
Chronic + multiple tics: starting before 18y + persisting >1y
What are the 3 types of tic?
Motor, vocal or phonic
Recall 2 therapies that are useful in tics
- Habit reversal therapy (learn movements to ‘compete’ with tics)
- Exposure with response prevention - help the child get used to the unpleasant feeling before a tic so that they can stop the tic occuring
What is the first line medication for tic disorder?
Antipsychotics - eg risperidone
What is the inheritance pattern of Duchenne muscular dystrophy?
x-linked recessive
At what age does duchenne muscular dystrophy present, and at what age can it be diagnosed?
1-3y presentation
Can be diagnosed at 5y
Which gene is deleted in Duchenne muscular dystrophy?
Dystrophin
Describe the pathophysiology of duchenne muscular dystrophy?
Dystophin gene connects cytoskeleton to muscle fibres to ECM through membrane
Where deficient –> influx of Ca –> calmodulin breakdown –> excess free radicals –> myofibre necrosis
What type of gait is seen in Duchenne muscular dystrophy?
Waddling
Other than gait, what else is affected in early DMD?
Language delay
What is Gower’s sign?
The need to turn prone to rise - seen in DMD
How is the heart affected by Duchenne muscular dystrophy?
Primary dilated cardiomyopathy
What is elevated in the plasma in Duchenne muscular dystrophy?
CK (creatine kinase) due to myofibre necrosis
How should Duchenne muscular dystrophy be managed?
No cure: often Mx is to alleviate the Sx
Physiotherapy to clear lungs + exercise to help prevent contractures
Medical:
- CPAP (due to weakness of intercostals)
- Glucocorticoids (to delay need for wheelchair)
- Cardioprotective drugs
What is the difference between Beck’s MD and DMD?
Same signs + Sx but often less severe + progresses at a slower rate
What is the genetic cause of myotonic muscular dystrophy?
Autosomal dominant trinucleotide repeat disorder
When does myotonic muscular dystrophy present?
20s to 30s
In what way is myotonic muscular dystrophy the reverse of duchenne muscular dystrophy?
Affects small muscles more than large muscles
Which form of MEN is NF1 associated with?
MEN2
Recall 4 signs and symptoms of NF1
Café au lait spots
Neurofibroma
Axillary freckles
Lisch nodule
What is tuberous sclerosis?
Rare genetic condition that causes mainly benign tumours to develop in different parts of the body
Recall 3 cutaneous features of tuberous sclerosis
“Ash leaf” patch
Shagreen patches (rough skin on lumbar spine)
Angiofibromata (butterfly facial distribution)
Recall some neurological features of tuberous sclerosis
Infantile spasms
Focal epilepsy
Intellectual disability (often with ASD)
Why is there a risk of hydrocephalus in tuberous sclerosis?
Development of subependymal giant cell astrocytoma
Which investigations are useful for diagnosing tuberous sclerosis?
CT/ MRI
What is the layman’s term for the haemangioma in Sturge Weber syndrome?
Port wine stain
Where does the haemangiomatous facial lesion present in Sturge Weber Syndrome?
Trigeminal nerve distribution
Recall 5 signs and symptoms of Sturge Weber Syndrome
Epilepsy
Contralateral hemiplegia
Phaeochromocytoma
Intellectual disability
Glaucoma
How should Sturge Weber Syndrome diagnosis be confirmed?
MRI
What are the 3 symptoms that may present via somatisation?
Abdo pain
Reucrrent headaches
Limb pain
What is Apley’s rule?
The further the pain is from the umbilicus, the more likely the pain is of an organic nature
What is the 1st line management for somatisation?
Promote communication between family + children
Pain-coping skills ie relaxation techniques for headaches
What should be done if 1st line treatments for somatisation fail?
Referral to CAMHS
Which type of developmental delay has the best prognosis?
Isolated delay (global has a/w syndromes that have poorer prognosis)
How does the aetiology of conjunctivitis differ between children and adults?
In adults is usually viral or allergic, in children is more likely to be bacterial
What is the most likely cause of sticky eyes in a <48 hours neonate?
Gonorrhoea
What is the most likely cause of sticky eyes in a neonate in first 1-2w?
Chlamydia: often co-presents with pneumonia
How should neonatal gonorrhoeal infection be investigated and treated?
Gram stain + culture
3rd gen cephalosporin (eg ceftriaxone)
How should neonatal chlamydia infection be investigated and treated?
Immunofluorescent staining
Oral erythromycin
Which is more common in children out of hypermetropia and myopia?
Hypermetropia
What is the cause of Retinopathy of Prematurity?
Vascular proliferation leads to retinal detachment –> fibrosis and blindness
What is the main RF for retinopathy of prematurity?
Uncontrolled use of high concentrations of oxygen
What are the 2 key signs of retinopathy of prematurity?
Unusual eye movements
White pupils
What is the first line treatment of retinopathy of prematurity?
Laser photocoagulation
What is strabismus?
Abnormal alignment of eyes
At what age is strabismus diagnosed?
1-4 years
What are the 2 types of strabismus?
Non-paralytic (refractive error in one or more eyes)
Paralytic (squinting eye could be caused by motor nerve paralysis or SOL - 3rd nerve palsy)
How should strabismus be managed?
1st line = glasses
2nd line = eye patching
3rd line = eye drops
4th line = eye muscle surgery
Which burn pattern is typical of NAI?
Glove and stocking
Which type of long bone fracture is indicative of NAI?
Spiral fractures
What is the classic triad of features in shaken-baby syndrome?
- Retinal haemorrhages
- CT showing brain swelling/ encephalopathy
- CT showing subdural haematoma
What needs to be ruled out in suspected NAI?
Leukaemia, ITP + haemophilia
What is the definition of SIDS?
Deaths which remain unexplained after a post-mortem
What is the peak age for SIDS?
2-4 months
Recall 6 important risk factors for SIDS
Front-sleeping baby
Prematurity
LBW
Male
Maternal smoking
Microenvironment (pillow, heat)
For how long should parents share a room with baby?
6 months
Which organisation provides support for SIDS?
Lullaby Trust
For how long should a child be excluded from school if they have scarlet fever?
24 hours after antibiotics
For how long should a child be excluded from school if they have whooping cough?
48 hours after Abx
For how long should a child be excluded from school if they have measles?
4 days from onset of rash
For how long should a child be excluded from school if they have rubella?
4 days from onset of rash
For how long should a child be excluded from school if they have chickenpox?
Until all lesions crusted over
For how long should a child be excluded from school if they have impetigo?
Until all lesions crusted over
For how long should a child be excluded from school if they have mumps?
5 days from onset of swollen glands
For how long should a child be excluded from school if they have influenza?
Until recovered
When do fontanelle close by?
1 year usually, can be as late as 2
What should be given before buccal midazolam in a fitting child?
Oxygen
What infections are children with DiGeorge syndrome particularly at risk of and why?
Candidiasis
No thymus –> no T cells
Give 2 causes of evanescent salmon pink rash
Listeriosis (neonate)
Juvenile idiopathic arthritis
Why shouldn’t metoclopramide be given to children?
Can give oculogyric crises
Recall 3 conditions that cause rigors in children?
Pyelonephritis
Influenza
Malaria
How should burns be managed medically?
Cover for potential toxic shock syndrome with ceftriaxone and clindamycin
How can the % of Total Burn Surface Area be measured OE?
Hand is 1% as a rough guide
Recall the 5 points of the Fraser guidelines
- YP understands professional’s advice
- YP cannot be persuaded to inform parents
- YP is likely to begin/ continue having sex regardless of contraception
- Physical/ mental wellbeing likely to suffer from lack of contraception
- YP’s BI require them
What % of children who have a febrile convulsion will have one again?
33-50%
