Neurology in Paediatrics Flashcards
Define cerebral palsy.
A movement disorder caused by a non progressive lesion in the motor pathways of the brain.
When does cerebral palsy develop?
It appears in development, although the lesion may have been present form birth.
Is cerebral palsy associated with other morbidities?
Yes - learning difficulties and other developmental delays can be associated with CP, but not always.
How does cerebral palsy present?
That depends on where the lesion is within the brain.
What % of children with cerebral palsy develop learning difficulties?
60%
What % of children with cerebral palsy develop hearing or visual problems of any kind? (% is the same for both, rather than % is both)
20%
What % of children with cerebral palsy develop epilepsy?
40%
How common is cerebral palsy?
1 in 2000 live births - most common motor impairment in children
Other than antenatal causes, what can cause cerebral palsy?
- Hypoxic ischaemic birth injury
- Post natal - head injury/secondary to any head pathology, or periventricular leucomalacia.
A mother takes her infant to the GP for a normal developmental follow-up.
The child is 6 months old, and shows a preference for his left hand over his right.
Form a list of differentials.
- Cerebral Palsy
- Stroke - clotting disorder, cardiac defect
- Brain malformation
- Brachial plexus lesion
- Infection -> secondary brain injury
A mother takes her infant to the GP for a normal developmental follow-up. The child is 6 months old, and shows a preference for his left hand over his right.
When is it normal for a child to show preference for one hand over the other?
Between ages 2 and 4 - basically 6 months is way too early so there must be a pathology behind the preference at this age.
What are some of the post natal causes of cerebral palsy, and what % of CP is caused by these?
10%
- Meningitis/encephalitis/encephalopathy
- Head trauma (inc. NAI)
- Symptomatic hypoglycaemia
- Hydrocephalus
- Hyperbilirubinaemia
What is the most common subtype of CP?
Define it.
Spastic CP.
Unilateral/Bilateral/unspecified.
Characterised by muscle spasms and increases muscle tone.
What are the 5 levels of gross motor function in CP?
1 - walks without limitations 2 - walks with limitations 3 - walk with a handheld mobility device 4 - self-mobility limited, powered mobility may be used. 5 - transported in a manual wheelchair
In spastic cerebral palsy, at what level is the damage?
Upper motor neurone level in the corticospinal or pyramidal tract.
What is the clasp knife phenomenon in CP?
Increased tone in flexion or extension, with a sudden relaxation as the muscle continues to stretch.
How should CP be managed in general terms?
MDT approach with support and education provided to the parents to help cope with the wide range of problems that a child with CP may have.
What specific treatments are available to treat hypertonia in CP?
- Botulinum toxin injections
- Selective dorsal rhizotomy
- Intrathecal baclofen
- Deep brain stimulation of basal ganglia
Other than spastic, what kinds of CP are there?
Describe them.
- Dyskinetic - movements are involuntary (chorea/athetosis/dystonia)
- Ataxic (hypotonic)
- Mixed
What is a seizure?
A paroxysmal abnormality of motor, sensory, autonomic, &/or cognitive function, due to transient brain dysfunction.
What makes a seizure epileptic?
They are unprovoked, and due to excessive and hypersynchronous electrical activity in the brain.
What are the 2 broad classifications of epileptic seizures?
Generalised and focal
What are the types of generalised seizures?
Absence Myoclonic Tonic Tonic-clonic Atonic Combination
What are infantile spasms?
Epileptic seizures in a child under 12 months
How is epilepsy diagnosed?
Clinically, from detailed hx and video evidence of a seizure.
Although epilepsy is usually idiopathic, what should be checked for on hx and examination in a child with seizures?
Evidence of neurocutaneous syndromes or neurological abnormalities.
How should we investigate suspected epilepsy?
ECG - check for underlying cardiac problem.
EEG - may be normal, can do an ambulatory EEG.
Brain imaging - MRI or CT if suspect structural abnormality.
What community based help can we give to families with an epileptic child?
Specialist epilepsy nurse contact.
What are the management steps for epilepsy?
- Education
- Lifestyle changes
- Antiepileptic drug therapy
Do all children with epilepsy need medication?
NO - should decide based on type and frequency of seizures/epilepsy.
What is the desired goal for AED therapy?
Monotherapy at minimum dosage
What AEDs are often first line for mot seizure types?
Valproate
Carbamazepine
For older children/teenagers with epilepsy, what additional considerations do we need to discuss with them?
- Driving
- Contraception
- Pregnancy
- Alcohol
- Poor sleep routines
What is status epilepticus?
Epileptic seizure lasting 30 minutes, or repeated seizures for 30 minutes without recovery of consciousness.
What is a febrile seizure?
Seizure associated with a fever occuring in an infant between 6 months and 6 years, with no other pathology causing seizures.
Are febrile seizures sinister?
No, they are benign.
What are the risk factors for developing a febrile convulsion?
High fever
Viral infection
Developmental delay
FHx
Do febrile convulsions need investigating?
No, they normally just resolve on their own if simple
If a child has febrile convulsions, does that increase the risk of epilepsy later in life?
Not in the majority of cases.
May increase risk if complex febrile convulsion.
A 10 year old comes to the GP with headaches.
What do you want to know?
SQITARS
Any aura?
Any sensory or motor symptoms?
Any visual changes?
A 10 year old comes to the GP with headaches.
He describes them as gradual in onset, with a band of tightness across his head. What is your main differential?
Primary, tension-type headache.
A 10 year old comes to the GP with headaches.
They last a whole day at a time, and is present on both sides. She also says she gets N&V during these episodes. Light makes them worse.
Whats your main differential?
Migraine without aura
Do all children with migraines get auras?
No - 90% of them don’t.
What is the most common aura that someone can experience?
Visual disturbance:
- negative e.g. hemianopia
- positive e.g. seeing lights/zigzag lines
What are the common triggers for migraines?
Disturbance in biorhythms (late night/early rise)
- Stress/wind down from stress
- Certain foods (unreliable)
- Menstruation/OCP
A 10 year old comes to the GP with headaches.
You suspect a benign primary cause, but what do you need to rule out?
Rule out headaches secondary to RICP or a space occupying lesion.
A 10 year old comes to the GP with headaches.
You suspect primary, innocent headaches, but mum wants reassurance, and you’re good doctor who knows what to do.
What examinations do you do?
- Cranial nerve examination
- Examine gait and full neuro exam
- Growth pattern
- Look for papilloedema
A 10 year old comes to the GP with headaches.
They tried using NSAIDs, but after while the headaches got worse. What’s going on?
Medication overuse -> rebound chronic daily headache.
Will resolve in 2 weeks after withdrawing medications.
What rescue treatments can we use for headaches/migraines?
- Analgesia
- Antiemetics
- Triptans (spray, not oral, not licensed in under 18s)
What prophylactic treatments can be used for migraines?
- Sodium channel blockers (valproate/topiramate)
- Beta blockers (propanolol)
- Tricyclics
Psychological support and relaxational technques!
What is Duchenne’s muscular dystrophy?
A most common, severe form of muscular dystrophy. X linked recessive so only boys get it.
How common is DMD?
Affects 1 in 3500 boys
Why does DMD occur?
Mutation in DMD gene causing lack of dystrophin -> connects muscle fibres to basal lamina -> excessive calcium entry into cells -> excess oxidative stress on cells -> cell death.
How does DMD present?
Progressive proximal muscle weakness and wasting, typically diagnosed at 5-5.5 years old
What are the notable features of DMD?
Abnormal gait with noticeable difficulty standing up.
Clumsiness
Waddling gait
General difficulty with motor skills
Thigh wasting most obvious
How should suspected DMD be investigated?
Positive Gower’s test Increased CK in blood EMG shows destruction of muscle tissue Genetic testing Muscle biopsy (rare since genetic testing)
What is the Gower’s test?
Pt asked to stand up from squatting. Positive if pt needs to use their hands and arms to “walk” up their body to standing.
How is DMD managed?
Aggressive use of corticosteroids - very effective
Physiotherapy
Mechanical ventilation in late stages
What is the progress associated with DMD?
Boys are usually in a wheelchair by 10. Death by age 20 usually.
Delay can sometimes be achieved with aggressive steroids.
How common are febrile convulsions?
3% of children will have a febrile convulsion between ages 6 months and 5 years.
How does the risk of further seizures change with every febrile convulsion a child has?
10% after 1
25% after 2
33% after 3 febrile convulsions
How should we manage febrile convulsions?
Identify and treat underlying infection.
Parental education - reassure, give written info, and advice for future seizures.
What advice do we give to parents to safety-net for future febrile convulsions?
Controlling temperature with paracetamol and damp sponge can help prevent convulsion
During - keep child safe, stay calm
Call 999 if it lasts more than a few minutes
After - take child to GP/A&E to be checked - needs investigation.
What can we give parents to give their child if they have a hx of prolonged febrile seizures?
Rectal diazepam or buccal diazepam for symptomatic relief
How common are headaches in children?
Up to 50% of 7 year olds, an 80% of 15 year olds have experinced at least one headache.
Equal M:F until puberty, then 1:3 M:F after puberty.
What are the IHS criteria for paediatric migraines?
A - 5+ attacks with all the following criteria
B - Headache attack lasting 4-72 hours
C - Headache with 2+ features (bilateral/unilateral fronto-temporal, pulsatile, moderate/severe, aggravated by activity)
D - Headache with nausea and/or vomiting or photo/phono-phobia
What blood tests are important to do in a child with a new onset of seizures?
Glucose
Calcium
A mother brings her 3 week old baby into see the GP because she is worried he is having seizures in his sleep. They only occur in his sleep.
She describes them as “the limb jerking kind”. What is this likely to be? Tell me about it.
Benign sleep myoclonus - present in the first month(s) of life. There is no chnage in HR or O2 saturations.
A mother brings her child in after they have a funny turn. The child says they felt funny before the episode, hot and clammy. Mum says they lost consciousness briefly. What is this likely to be?
Vasovagal syncope
If a child has syncope, what do we need to rule out before we can be happy saying it was just vasovagal?
Any cardiac or neurological cause - can usually tell from the hx.
If a syncope hx in a child doesn’t sound exactly like vasovagal syncope, what Ix can we do it check?
ECG Lying and standing BP Blood glucose Pregnancy test in older girls Full cardiac and neurological examination
What red flags would you look for in the hx that would suggest cardiac origin of syncope in a child?
- lack of prodrome
- palpitations or chest pain
- exercise-induced syncope
- past cardiac history
- family history of early cardiac death, arrhythmia or sudden death.
A mother is concerned her son is having some partial seizures. When you question her further, she says she notices him moving in a strange repetative way sometimes. What might this be, and if it is, when would it be observed generally?
Simple motor tic - often at its worst when they are watching television or they are excited.
How does Tourettes syndrome usually present?
Tics, sometimes with other symptoms such as echolalia (repeating other peoples words), Palilalia (repeating their own words), Copropraxia (obscene gestures), Coprolalia (obscene words), or echopraxia (copying other poeple’s movements).
Often impairs concentration and general function.
What is a tic?
sudden, purposeless, repetitive, non-rhythmic, stereotyped movements or vocalisations that are not sue to drugs or other secondary causes.
How common are neural tube defects?
2nd most common disabling congenital defects.
What are the factors that influence development of neural tube defects?
Multiple genes, nutrition, and environmental factors.
What is the neural tube?
Embryonic precursor to brain and spinal cord.
Why do neural tube defects occur?
Due to abnormal closure of the neural plate which occurs between days 21 and 28 of gestation.
How can neural tube defects be classified?
Into open and closed or by site of involvement (spinal and cranial).
What are the cranial neural tube defects?
Anencephaly, Encephalocele, and congenital dermal sinus.
What is anencephaly?
A severe congenital condition in which a large part of the skull is absent along with the cerebral hemispheres of the brain.
What is encephalocele?
Sac-like protrusions of the brain and the membranes that cover it through openings in the skull
What is congenital dermal sinus?
A dermal indentation found along the midline of the neuraxis.
What is the most common spinal NTD?
Spina bifida
What is spina bifida?
Vertebral arch of spinal column is incompletely formed or absent, at any point from base of skull to sacrum, most commonly affecting the lumbar spine.
Other than spina bifida, what other spinal NTDs are there?
Spina bifida occulta, myelomeningocele, meningocele, congenital dermal sinus, and caudal agenesis.
Which are the 2 most common NTDs?
Spina bifida and anencephaly (95%, half each)
What are the risk factors for a NTD in a foetus?
FHx of any type of defect, concurrent genetic/chromosomal syndromes, inadequate folate during pregnancy, and therapy with anti-epileptic drugs during pregnancy.
What is the prognosis associated with anencephaly?
Most cases are terminated following prenatal diagnosis, but those that are born are either stillborn or die shortly after birth.
How are NTDs diagnosed?
Usually on prenatal scans, but sometimes picked up after birth.
How are NTDs investigated in newborns?
MRI for imaging neural tissue, or CT for bony defects and anatomy.
Other than prenatal USS, how can a prenatal diagnosis of a NTD be made?
Screening by measuring maternal serum alpha-fetoprotein - best detected at 16-18 weeks but is less sensitive for closed defects or if woman is taking sodium valproate.
At what point in the pregnancy is USS best for diagnosing NTDs?
In the second trimester.
How should a neonate with a NTD be managed?
Depending on the severity, and by an MDT team to address associated difficulties that occur alongside the NTD.
How should a neonate with an open NTD be managed initially?
Keep them warm, cover the defect with a sterile saline dressing, and position them prone to avoid pressure on the defect.
How should an open NTD be managed definitively?
Close them promptly.
How should a neonate with hydrocephalus secondary to a NTD be managed?
Put a shunt in place
What complications are associated with NTDs?
Infections, motor and sensory problems, learning disability, developmental delay, hearing impairment, and bladder and bowel dysfunction.
What does the prognosis of a NTD depend on?
The nature of the defect and the associated malformations.
How can NTDs be prevented?
Periconceptional folate supplement at 400 micrograms for low risk mothers, and 5mg for high risk mothers.
