Nervous system 2 Flashcards
List causes of fits, faints and funny turns:
1) In infancy
2) Beyond infancy
3) School-age
1) In infancy:
- Apnoea and acute life-threatening episodes
- Febrile convulsions
- Breath-holding
- Infantile spasms
- Epilepsy
- Hypoglycaemia and metabolic conditions
2) Beyond infancy
- Febrile convulsions
- Breath-holding
- Night terrors
- Epilepsy
- Benign paroxsymal vertigo
3) School-age
- Epilepsy
- Syncope
- Hyperventilation
- Hysteria
- Tics
What is important to include in a hx of a child presenting with a fit, faint or funny turn?
Episode:
- What child was doing at onset / any precipitating factors
- Length of episode
- Loss / alteration of consciousness
- Any involuntary movements
- Change in colour: pallor / cyanosis
- Reaction of the child to the event
- Postictal phase?
BIND
FH
What physical examinations should be performed of a child presenting with a fit, faint or funny turn?
Cardiac and neurological examination
Check for dysmorphic features, micro- or macrocephaly and hepatosplenomegaly = suggest metabolic disorders
What investigations should be done child presenting with a fit, faint or funny turn?
EEC
ECG
Blood glucose
What is BIND?
Birth
Immunisations
Neonatal
Development
What are characteristic features of apnoea and acute life threatening events (ALTE)?
Usually found limp or twitching
Age <6 months
Usually no precipitating event
Concern over whether a cardiac arrhythmia, convulsion or choking episode has precipitated the event
What is the usual course of management of apnoea and acute life threatening events (ALTE)?
Usually admitted for observation and discharged after an uneventful night
What can precipitate syncope?
= Vasovagal
Pain
Emotion
Prolonged standing
How does syncope present?
Blurred vision
Light-headedness
Sweating
Nausea
Resolved on lying down
List some causes of headaches (11)
1) Tension headache
2) Migraine
3) Raised ICP
4) HTN
5) Dental caries
6) Infection
7) Meningitis (acute)
8) Haemorrhage (rare)
9) Tumour (rare)
10) Eye strain
11) Sinusitis
List some red flags of a child presenting with a headache (10)
1) Acute onset of severe pain
2) Fever
3) Headache intensified by lying down
4) Associated vomiting
5) Fall-off in school performance or regression of developmental skills
6) Consistently unilateral pain
7) Cranial bruit
8) HTN
9) Papilloedema
10) Fall-off in growth
Describe the character of a tension headache
Constricting and band-like
When is a tension headache worst?
Towards the end of the end of the day
Are there any associated symptoms or findings on examination of a tension headache?
No
Describe the character of a migraine
Throbbing, unilateral
What features are associated with a migraine?
N&V
Aura
Photophobia
FH
Are there any findings on physical examination of a migraine?
No
Describe the character of a headache due to raised ICP
Worse on lying down, may be localised to the site of the lesion
Describe the timing of a headache due to raised ICP
Worse early morning
Wakes child at night
List some associated features of raised ICP
Vomiting without nausea
Other features depend on site of lesion
What may be found on physical examination of a child presenting with a headache due to raised ICP
Slow pulse High BP Papilloedema Enlarged head circumference Focal signs
What should be included on physical examination of a child presenting with a headache?
Is the child ill?
- Fever, meningeal signs and reduced level of consciousness indicate meningitis / meningoencephalitis
HTN
Signs of raised ICP
- Slow pulse, high BP, papilloedema and enlarging head circumference (preschool child)
Focal neurological signs
- Cranial nerve palsies and cerebellar signs suggest infratentorial tumour
- Focal spasicity indicate cerebral lesion
- Delayed growth and puberty indicate pituitary tumour
What investigations are done for a child presenting with a headache?
Rarely done unless evidence of raised ICP or neurological signs
CT / MRI
What is the management of a tension headache?
Reassurance
Avoid medications as they are unlikely to help
What is thought to be the pathophysiology of a migraine?
Constriction followed by vasodilation of the intracranial arteries
What is the typical age of onset of a migraine in a child?
Late childhood or early adolescence
How does a migraine present?
Preceded by aura (vasoconstriction)
Within a few minutes a throbbing, unilateral headache occurs accompanied by n&v
Last 1-72hrs
Sleep usually ends the attack
Rarely, complicated migraine occurs when focal neurological symptoms and sign are present
How does a migraine present in younger children?
Attack often bilateral with no aura, nausea or vomiting
What is the management of migraine?
1st line: rest with simple analgesia
Avoid triggers eg chocolate, cheese, citrus fruits, nuts, caffeine
If frequent, prophylaxis with propanolol or pizotifen
Sumatriptan (5-hydroxy-tryptamine agonist) can help abort acute migraine in adolescents but is not recommended in childhood
List some causes of hydrocephalus
Obstruction of CSF flow:
- Congenital abnormality of the brain eg aqueductal stenosis
- Acquired as a result of intracranial haemorrhage, infection or tumour
Failure to resorb CSF
What group of children are particularly at risk of hydrocephalus?
Premature babies with severe intracranial haemorrhage
What is hydrocephalus associated with?
NTD
Occurs in 80% of children with spina bifida
How does hydrocephalus present?
Accelerated head growth:
- Anterior fontanelle is wide open and bulging
- Sutures separated
- Scalp veins dilated
- Forehead is broad and eyes deviated down = ‘setting sun’ sign
Irritability, lethargy, poor appetite and vomiting common
Spasticity, clonus and brisk deep tendon reflexes
How does hydrocephalus present in older children?
More subtle
Headache and deterioration in school performance
How is hydrocephalus managed?
Urgent
Cranial USS or MRI used to determine most appropriate neurological procedure
Most commonly an extracranial shunt put in to drain CSF
- Most shunts are ventriculoperitoneal = CSF drains from lateral ventricle into peritoneal cavity (ventriculoatrial rarely used)
What are some complications of shunt placement in hydrocephalus?
Blockage and infection
Parents must be educated to recognise the features of raised ICP - which indicate these problems
What is the prognosis of children with hydrocephalus?
At risk of developmental disabilities and LD, esp relating to performance tasks and memory
Visual problems common
Important to receive long term follow up
What is hydrocephalus?
Obstruction of CSF flow leads to dilatation of ventricles
When skulls are not fused this causes grossly enlarged head and raised ICP
What is a non-communicating vs a communicating cause of hydrocephalus?
Non-communicating (obstructive) - CSF blocked along one/more of the narrow passages connecting the ventricles:
- Aqueduct stenosis
- Posterior fossa
- Tumours
Communicating - CSF blocked after it exits the ventricles, but CSF can still flow between ventricles:
- Meningitis
- SAH
- IVH
What is a tic?
A rapid, repetitive, brief, involuntary movement such as blinking, jerking or facial grimacing
What can precipitate a tic? Who gets them?
Anxiety, fatigue or excitement
Common in school children
Peak age 8-11yrs
M>F
What can tics resemble and how can they be differentiated?
Simple or complex partial (focal) seizures
Differentiated by the fact that tics can be controlled voluntary and are not associated with alteration in consciousness
What features indicate an immediate CT with a child presenting following a head injury? (10)
1) Witnessed LOC >5 mins
2) Amnesia >5mins
3) >2 discrete episodes of vomiting
4) Clinically suspected NAI
5) Post traumatic epilepsy
6) GCS <14 or <15 for an infant
7) Suspected open or depressed skill injury or tense fontanelle
8) Focal neurological deficit
9) Swelling, bruising or laceration >5cm
10) Dangerous mechanism of injury
What is plagiocephaly?
Common asymmetry of the skull
Normal head circumference
Usually improves over the first few months
How does plagiocephaly present?
Flat spot on the back or one side of the head
Cuased by remaining in supine position for too long (sleeping position)
No associated symptoms
Does not affect the brain
How is plagiocephaly managed?
Early recognition
‘Tummy time’
Altering positioning of toys
Alleviate pressure from head whilst in car seat
Physiotherapy if difficulty turning to one side?
?Helmets and bands - controversial
What syndrome has motor and vocal tics (rarely tic free)?
Tourett’s syndrome
How is Tourett’s syndrome managed?
Clonidine / risperidone
What is the commonest neuromuscular disorder of childhood?
Duchenne muscular dystrophy (DMD)
What are the genetics involved in DMD?
X linked recessive condition caused by a mutation in dystrophin gene on Chr X(q28) - a protein which keeps muscle cells in tact
How does DMD present?
Typically presents by 4yr with motor and speech development delay
- Waddling gait
- Gower’s sign
- Hyporeflexia
- Calf pseudohypertrophy (muscle replaced with fat)
- Sparing of facial, extraocular and bulbar muscles
What investigations are done for DND?
Bloods show raised serum creatinine kinase
Measure all boys not walking by age 18m
What is the management of DMD?
Prophylactic - genetic counselling
Supportive - physiotherapy, mobility aids
What is the prognosis of DMD?
Affected children develop progressive cardiomyopathies
Ability to walk lost by age 10
Death by cardiorespiratory failure by early 20s
What is Gower’s sign?
Using hands and arms to “walk” up their own body from a squatting position
Due to lack of hip and thigh muscle strength
What is spina bifida?
Failure of closure of the posterior neuropore
How is spina bifida prevented?
Periconceptual folic acid supplementation (reduced incidence by 75%)
5mg
What monitoring is done throughout pregnancy to check for spina bifida?
USS or alpha-fetoprotein (raised in spina bifida)
List 4 types of spina bifida
1) Anencephaly
2) Myelomenigocoele
3) Meningocoele
4) Spina bifida occulta
What is anencephaly?
Most severe form
Complete failure of the development of the cranial part of the neural tube and the brain does not develop
What is myelomeningocoele?
Open lesion with the malformed and exposed spinal cord (myelocoele) being covered in a thin membrane of meninges (meningoecele)
What is myelomeningocoele associated with?
Severe neurological abnormality of the LL, bladder and anal innervation together with hydrocephalus in 90%
Surviving children have major disabilities requiring lifelong supervision
What is meningocoele?
Spinal cord is intact and functions normally but defect involves an exposed bag of meningeal membranes which rupture easily
What are major risks in meningocoele?
Meningitis and hydrocephalis
Rapid surgical closure necessary to avoid infection
What is spina bifida occulta (SBO)?
‘hidden’ abnormality of the developing neural tube which, if unrecognised and untreated, may later cause serious neurological disability
SBO may be the only visible feature of tethering of the spinal cord within the spinal canal, with eventual stretching of the cord
What features are associated with SBO?
Bladder dysfunction and pyramidal tract sings in lower limbs as child growths
Subtle abnormality of midline over spine:
- Deep pit over lower back
- Tuft of hair
- A naevus
- A lipoma at or near the midline
How SBO investigated?
US
How does spina bifida present?
Can occur at any spinal level Swelling over spine Spinal cord exposed Causes paralysis below lesion Hydrocephalus Neuropathic bladder - incontinence Faecal incontinence
