Paeds neurology Flashcards
febrile convulsions occur only in children between the ages of
between the ages of 6 months and 5 years
Febrile convulsions definition
type of seizure that occurs in children with a high fever. They are not caused by epilepsy or other underlying neurological pathology, such as meningitis or tumours.
Simple febrile convulsions are
Simple febrile convulsions are generalised, tonic clonic seizures. They last less than 15 minutes and only occur once during a single febrile illness.
Febrile convulsions can be described as complex when
Febrile convulsions can be described as complex when they consist of **partial **or focal seizures, last more than 15 minutes or occur multiple times during the same febrile illness.
The differential diagnoses of a febrile convulsion are
typical presentation of febrile convulsions
A typical presentation is a child around 18 months of age presenting with a 2 – 5 minute tonic clonic seizure during a high fever. The fever is usually caused by an underlying viral illness or bacterial infection such as tonsillitis.
Once a diagnosis of a febrile convulsion has been made, look for the underlying source of infection.
to make a diagnosis of a febrile convulsion, other neurological pathology must be excluded.
management of febrile convulsions
In the febrile child the first stage is to identify and manage the underlying source of infection and control the fever with simple analgesia such as paracetamol and ibuprofen. Simple febrile convulsions do not require further investigations and parents can be reassured and educated about the condition.
Complex febrile convulsions may need further investigation.
febrile convulsions parents advice on managing a seizure if a further episode occurs:
prognosis of febrile convulsions
Febrile convulsions do not typically cause any lasting damage. One in three will have another febrile convulsion.
The risk of developing epilepsy is:
- 1.8% for the general population
- 2-7.5% after a simple febrile convulsion
- 10-20% after a complex febrile convulsion
Epilepsy defintion
- The aim of treatment in epilepsy is to
The aim of treatment is to be seizure free on the minimum anti-epileptic medications. Ideally monotherapy with a single anti-epileptic drug.
- Different types of seizures
- Generalised Tonic-Clonic Seizures
- Focal Seizures
- Absence Seizures
- Atonic Seizures
- Myoclonic Seizures
- Infantile Spasms
- Febrile convulsions
- Management of tonic-clonic seizures is with
- males: sodium valproate
- females: lamotrigine or levetiracetam
- girls aged under 10 years and who are unlikely to need treatment when they are old enough to have children or women who are unable to have children may be offered sodium valproate first-line
signs and symptoms of generalised tonic clonic seizures
Focal seizures start in which part of the brain? They affect which abilities?
Focal seizures start in the temporal lobes. They affect hearing, speech, memory and emotions
Focal seizures presentation
Hallucinations
Memory flashbacks
Déjà vu
Doing strange things on autopilot
Focal seizures treatment
first line: lamotrigine or levetiracetam
second line: carbamazepine, oxcarbazepine or zonisamide
Absence seizures presentation
Absence seizures typically happen in which group?
Children
Absence seizures typically last how long
10-20 seconds
Atonic seizures definition/presentation
Atonic seizures may be indicative of which syndrome
Lennox-Gastaut syndrome
- Atonic seizures management
First line: sodium valproate
Second line: lamotrigine
Absence seizures management
first line: ethosuximide
second line:
male: sodium valproate
female: lamotrigine or levetiracetam
carbamazepine may exacerbate absence seizures
Myoclonic seizures presentation
Myoclonic seizures present as sudden brief muscle contractions, like a sudden “jump”. The patient usually remains awake during the episode
Myoclonic seizures typically happen in… as part of which condition?
typically happen in children as part of juvenile myoclonic epilepsy
Myoclonic seizures management
First line: sodium valproate
Other options: levetiracetam
Infantile spasms presentation/definition
Infantile spasms management
Prednisolone
Vigabatrin
investigations and diagnosis of seizures and epilepsy in children
When is an MRI brain used to investigate seizures in children (criteria)?
- When is an EEG used to investigate seizures in children?
Perform an EEG after the second simple tonic-clonic seizure.
Children are allowed one simple seizure before being investigated for epilepsy.
Additional investigations can be considered to exclude other pathology that may cause seizures:
General advice to give parents about epilepsy in children
sodium valproateMOA and side effects
Side effects of carbamazepine
Agranulocytosis
Aplastic anaemia
Induces the P450 system so there are many drug interactions
Phenytoin SE
Folate and vitamin D deficiency
Megaloblastic anaemia (folate deficiency)
Osteomalacia (vitamin D deficiency)
Ethosuximide SE
Night terrors
Rashes
Lamotrigine SE
Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes.
Leukopenia
Management of seizures
Definition of status epilepticus
seizure lasting more than 5 minutes or 2 or more seizures without regaining consciousness in the interim.
Management of status epileptics in the hospital
Medical options in the community for management of status epilepticus
Buccal midazolam
Rectal diazepam
infantile spasms typically present in
first 4 to 8 months of life and is more common in male infants
- infantile spasms are often associated with
often associated with a serious underlying condition and carry a poor prognosis
- infantile spasms Features
- characteristic ‘salaam’ attacks: flexion of the head, trunk and arms followed by extension of the arms–> ‘jack knife’ spasms, which usually occur in clusters.
- this lasts only 1-2 seconds but may be repeated up to 50 times
- progressive mental handicap
- How long do infantile spasms last
only 1-2 seconds but may be repeated up to 50 times
- Investigation and findings in infantile spasms
- the EEG shows hypsarrhythmia in two-thirds of infants
- CT demonstrates diffuse or localised brain disease in 70% (e.g. tuberous sclerosis)
Management of infantile spasms
- poor prognosis
- vigabatrin is now considered first-line therapy
- ACTH is also used
Cerebral palsy definition
Cerebral palsy (CP) is the name given to the permanent neurological problems resulting from damage to the brain around the time of birth
Causes of cerebral palsy
Types of cerebral palsy
Patterns of spastic cerebral palsy
Children at risk of developing cerebral palsy, such as those with
hypoxic-ischaemic encephalopathy
CP presentation
Complications and associated conditions in CP
Management of CP
Paediatricians will regularly see the child with CP to optimise their medications. This may involve:
Which drug is used for excessive drooling in CP
Glycopyrronium bromide
The differential diagnosis of an upper motor neurone lesion other than CP is
acquired brain injury or a tumour.
Test for… to look for cerebellar involvement in CP
Test for coordination to look for cerebellar involvement
Look for… that indicate extrapyramidal (basal ganglia) involvement in CP
Look for athetoid movements that indicate extrapyramidal (basal ganglia) involvement
People with CP will have signs of an… neurone lesion, with these signs:
signs of an upper motor neurone lesion, with good muscle bulk, increased tone, brisk reflexes and slightly reduced power. Power may be normal.
Patients with cerebral palsy may have a …or…gait
Patients with cerebral palsy may have a hemiplegic or diplegic gait
In CP a hemiplegic or diplegic gait is caused by
This gait is caused by increased muscle tone and spasticity in the legs. The leg will be extended with plantar flexionof the feet and toes. This means they have to swing the leg around in a large semicircle when moving their leg from behind them to in front. There is not enough space to swing the extended leg in a straight line below them.
You can gain a lot of information about a child from their gait:
Hemiplegic / diplegic gait: indicates
Broad based gait / ataxic gait: indicates
High stepping gait: indicates f
Waddling gait: indicates
Antalgic gait (limp): indicates
Neuro exam findings in upper vs lower motor neuron lesions
Hydrocephalus definition. It is a result of two main categories of causes:
cerebrospinal fluid (CSF) building up abnormally within the brain and spinal cord.
This is a result of either over-production of CSF or a problem with draining or absorbing CSF.
The most common cause of hydrocephalus is
aqueductal stenosis, leading to insufficiency drainage of CSF.
The cerebral aqueduct that connects the third and fourth ventricle is stenosed (narrowed).
This blocks the normal flow of CSF out of the third ventricle, causing CSF to build up in the lateral and third ventricles.
Congenital causes of hydrocephalus:
Presentation of hydrocephalus
Hydrocephalus management
VP shunt complications
Causes of headaches in children
Tension headache presentation
There are certain triggers for tension headaches in children
Stress, fear or discomfort
Skipping meals
Dehydration
Infection
Tension headaches in children management
reassurance, analgesia, regular meals, avoiding dehydration and reducing stress.
Types of migraines
Migraine without aura
Migraine with aura
Silent migraine (migraine with aura but without a headache)
Hemiplegic migraine
Abdominal migraine
Migraine presentation including associated symptoms
Management of migraines in children:
Abdominal migraines presentation including associations
In a child with a new headache, always check for symptoms and signs of which other conditions?
Meningitis is defined as
inflammation of the meninges. The meninges are the lining of the brain and spinal cord. This inflammation is usually due to a bacterial or viral infection.
Meningococcal septicaemia refers to
Meningococcal meningitis is when
the bacteria meningococcus is infecting the meninges and the cerebrospinal fluid around the brain and spinal cord.
The most common causes of bacterial meningitis in children and adults are
Neisseria meningitidis (meningococcus) and Streptococcus pneumoniae (pneumococcus).
In neonates the most common cause of bacterial meningitis is
group B strep (GBS)
Typical symptoms of meningitis are… Where there is meningococcal septicaemia children can present with
fever
neck stiffness
vomiting
headache
photophobia
altered consciousness
seizures
Where there is meningococcal septicaemia children can present with a non-blanching rash
Neonates and babies with meningitis or meningococcal septicaemia can present with
very non-specific signs and symptoms, such as hypotonia, poor feeding, lethargy, hypothermia and a bulging fontanelle.
- NICE recommend a… as part of the investigations for all children with meningitis:
NICE recommend a lumbar puncture as part of the investigations for all children:
- Under 1 month presenting with fever
- 1 to 3 months with fever and are unwell
- Under 1 year with unexplained fever and other features of serious illness
There are two special tests you can perform to look for meningeal irritation:
Contraindications to lumbar puncture
Management of bacterial meningitis
** blood culture and a lumbar puncture** for cerebrospinal fluid (CSF) should be performed prior to starting antibiotics, however if the patient is acutely unwell antibiotics should not be delayed.
Send blood tests for meningococcal PCR if meningococcal disease is suspected.
Vancomycin should be added to these antibiotics if there is a risk of penicillin resistant pneumococcal infection, for example recent foreign travel or prolonged antibiotic exposure.
Post exposure prophylaxis for meningococcal infections
The most common causes of viral meningitis are
herpes simplex virus (HSV)
enterovirus
varicella zoster virus (VZV)
Investigations for viral meningitis
A sample of the CSF from the lumbar puncture should be sent for viral PCR testing.
Management of viral meningitis
Viral meningitis tends to be milder than bacterial and often only requires supportive treatment.
Aciclovir can be used to treat suspected or confirmed HSV or VZV infection.
Where in the spine does the needle go for a lumbar puncture? Lumbar puncture samples are sent for which tests at the lab?
lumbar puncture results in bacterial vs viral meningitis
Complications of meningitis
Encephalitis definition
Encephalitis means inflammation of the brain. This can be the result of infective or non-infective causes. Non-infective causes are autoimmune, meaning antibodies are created that target brain tissue.
Most common cause of encephalitis in children
herpes simple type 1 (HSV-1) from cold sores
Most common cause of encephalitis in neonates
herpes simplex type 2 (HSV-2) from genital herpes, contracted during birth
causes of encephalitis
Presentation of encephalitis
Children with features of encephalitis need some key investigations to establish the diagnosis:
Contraindications to a lumbar puncture for encephalitis
GCS below 9 haemodynamically unstable
active seizures or post-ictal.
Management of encephalitis
Complications of encephalitis
Tourette’s definition
the development of tics that are persistent for over a year
Tics definition
Tics are involuntary movements or sounds that the child performs repetitively throughout the day
Tics triggers
tics become more prominent when the person is under pressure or excited
- stress
- tired
- bored
Tics usually decrease with:
- Concentration
- Exercise
- Distraction
- Sleep
premonitory sensations definition
The person may describe an overwhelming urge to perform the tic. This urge increases the more they suppress it. They feel they need to complete the tic, often several times, to get relief from that urge.
tics presenting age and associated conditions
Tics often present around or after 5 years of age. They can be associated with OCD and ADHD.
examples of simple tics
- Clearing throat
- Blinking
- Head jerking
- Sniffing
- Grunting
- Eye rolling
examples of complex tics
management of tics
conjunctivitis definition and presentation, and types
bacterial vs viral conjunctivitis presentation
causes of acute painful red eye
causes of acute painless red eye
Conjunctivitis
Episcleritis
Subconjunctival haemorrhage
Conditions that require emergency same-day referral to ophthalmology tend to cause
pain or reduced visual acuity
Management of redeye
Neonatal conjunctivitis may be caused by which organism? it can lead to…
Neonates under one month with conjunctivitis need urgent ophthalmology assessment. Neonatal conjunctivitis may be caused by gonococcal infection, which can cause serious complications (e.g., permanent vision loss).
Allergic conjunctivitis cause, presentation and management
Periorbital cellulitis definition
orbital cellulitis definition
orbital and periorbital cellulitis risk factors
orbital and periorbital cellulitis presentation
Differentiating orbital from preseptal cellulitis
reduced visual acuity, proptosis, ophthalmoplegia/pain with eye movements are NOT consistent with preseptal cellulitis
orbital and periorbital cellulitis investigations
orbital and periorbital cellulitis management
admission to hospital for IV antibiotics due to the risk of cavernous sinus thrombosis and intracranial spread
muscular dystrophies definition
Duchenne muscular dystrophy is an X-linked recessive inherited disorder in the dystrophin genes required for normal muscular function.
muscular dystrophies aetiology
muscular dystrophies presentation
Duchenne’s Muscular Dystrophy
* Symptoms appear ages 1-6yrs
● Progressive proximal muscle weakness
● Difficulty walking, clumsy
● Abnormal waddling gait, toe walking
● Difficulty climbing stairs
● Incontinence
● Delayed motor milestones
● In DMD most have lost ability to walk by 12 years
● Enlarged calves
o Fatty infiltration
o Pseudohypertrophy
● Absent knee jerks
● Gower’s sign
o Using their hands and arms to “walk” up their own body from a squatting position
o Due to lack of proximal muscle strength
● 30% have intellectual impairment in Duchenne’s
● Much less common in Becker
Becker’s Muscular Dystrophy
* Presents later in childhood with muscle wasting and weakness
* Patients commonly become wheelchair-bound in their teens and can survive into their thirties
muscular dystrophies investigations
- raised creatinine kinase
- genetic testing has now replaced muscle biopsy as the way to obtain a definitive diagnosis
muscular dystrophies management
muscular dystrophies prognosis
- Ability to walk lost by age 12
- Ventilator support required ~25
- Death occurs at early age due to pneumonia/MI
- associated with dilated cardiomyopathy
- 10-40% live to 40 du to respiratory support
Differential diagnosis of seizures
Epileptic seizures
Non epileptic events
*Syncopes and anoxic seizures
*Psychological disorders
*Sleep disorders
*Paroxysmal movement disorders
*Migraine and related disorders
*Fabricated illness
*Miscellaneous disorders
History and examination for a seizure event
Childhood epileptic seizures causes
focal seizure definition
- seizure origin within the network limited to one cerebral hemisphere
- most commonly arising from the temporal and frontal lobes
generalised seizure deifinition and types
- seizure onset within and rapidly engaging bilaterally distributed networks with loss of consciousness
- types of generalised seizure include tonic-clonic, tonic, atonic, myoclonic and absence.
syncope definition
Syncope is the term used to describe the event of temporarily losing consciousness due to a disruption of blood flow to the brain, often leading to a fall. Syncopal episodes are also known as vasovagal episodes, or simply fainting
Syncope signs and symptoms
Primary causes and secondary causes of syncope
What to ask in a history of syncope
Syncope vs seizure differences
Syncope examination
Syncope investigations
Syncope management
Reflex anoxic attacks definition and presentation
Absence seizures definition and presentation
Juvenile myoclonic epilepsy presentation, triggers and management
Benign Rolandic epilepsy description, presentation and management
Infantile spasms is a triad of:, management and consequences
breath holding spells types
cyanotic breath holding spells and pallid breath holding spells (also known as reflex anoxic seizures).
Breath holding spells definition
Breath holding spells are also known as breath holding attacks. They are involuntary episodes during which a child holds their breath, usually triggered by something upsetting or scaring them.
Breath holding spells age of presentation and long term effects
between 6 and 18 months of age.
The child has no control over the breath holding spells. They are not harmful in the long term, do not lead to epilepsy and most children outgrow them by 4 or 5 years.
Cyanotic breath holding spells presentation
Cyanotic breath holding spells occur when the child is really upset, worked up and crying. After letting out a long cry they stop breathing, become cyanotic and lose consciousness. Within a minute they regain consciousness and start breathing. They can be a bit tired and lethargic after an episode.
reflexi anoxic attacks presentation
Reflex anoxic seizures occur when the child is startled. The vagus nerve sends strong signals to the heart that causes it to stop beating.
The child will suddenly go pale, lose consciousness and may start to have some seizure-like muscle twitching. Within 30 seconds the heart restarts and the child becomes conscious again.
Breath holding spells management
After excluding other pathology and making a diagnosis, educating and reassuring parents about breath holding spells is the key to management.
Breath holding spells have been linked with iron deficiency anaemia. Treating the child if they are iron deficiency anaemic can help minimise further episodes.
Raised intracranial pressure definition and pathophysiology
Raised intracranial pressure causes
idiopathic intracranial hypertension
traumatic head injuries
infection: meningitis
tumours
hydrocephalus
Raised intracranial pressure features
headache
vomiting
reduced levels of consciousness
papilloedema
Cushing’s triad:
- widening pulse pressure
- bradycardia
- irregular breathing
Raised intracranial pressure investigations and monitoring
Raised intracranial pressure management
Subarachnoid haemorrhage definition and prognosis
Subarachnoid haemorrhage involves bleeding in the subarachnoid space, where the cerebrospinal fluid is located, between the pia mater and the arachnoid membrane. This is usually the result of a ruptured cerebral aneurysm.
very high mortality (around 30%) and morbidity, making it essential not to miss.
Subarachnoid haemorrhage risk factors: general risk factors, associated particularly with, more common in
Subarachnoid haemorrhage presentation
Subarachnoid haemorrhage investigations
Subarachnoid haemorrhage management
Subarachnoid haemorrhage complications and their management
triad of the shaken baby syndrome
Retinal haemorrhages, subdural haematoma and encephalopathy
Dyskinetic cerebral palsy typically manifests as:
Dyskinetic cerebral palsy typically manifests as athetoid movements and oro-motor problems
“On examination of the child, you notice that he is drooling and is making lots of slow, writhing movements of his hands and feet. The father tells you that this is common and that his son struggles to hold onto objects such as toys.”
spastic cerebral palsy manifests as:
spastic (70%)
subtypes include hemiplegia, diplegia or quadriplegia
increased tone resulting from damage to upper motor neurons
dyskinetic CP caused by:
dyskinetic
caused by damage to the basal ganglia and the substantia nigra
ataxic CP is caused by:
ataxic
caused by damage to the cerebellum with typical cerebellar signs
