Paeds neurology Flashcards
(178 cards)
1
Q
febrile convulsions occur only in children between the ages of
A
between the ages of 6 months and 5 years
2
Q
Febrile convulsions definition
A
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.
3
Q
Simple febrile convulsions are
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Simple febrile convulsions are generalised, tonic clonic seizures. They last less than 15 minutes and only occur once during a single febrile illness.
4
Q
Febrile convulsions can be described as complex when
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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.
5
Q
The differential diagnoses of a febrile convulsion are
A
6
Q
typical presentation of febrile convulsions
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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.
7
Q
management of febrile convulsions
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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.
8
Q
febrile convulsions parents advice on managing a seizure if a further episode occurs:
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9
Q
prognosis of febrile convulsions
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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
10
Q
Epilepsy defintion
A
11
Q
- The aim of treatment in epilepsy is to
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The aim of treatment is to be seizure free on the minimum anti-epileptic medications. Ideally monotherapy with a single anti-epileptic drug.
12
Q
- Different types of seizures
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- Generalised Tonic-Clonic Seizures
- Focal Seizures
- Absence Seizures
- Atonic Seizures
- Myoclonic Seizures
- Infantile Spasms
- Febrile convulsions
13
Q
- Management of tonic-clonic seizures is with
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- 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
14
Q
signs and symptoms of generalised tonic clonic seizures
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15
Q
Focal seizures start in which part of the brain? They affect which abilities?
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Focal seizures start in the temporal lobes. They affect hearing, speech, memory and emotions
16
Q
Focal seizures presentation
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Hallucinations
Memory flashbacks
Déjà vu
Doing strange things on autopilot
17
Q
Focal seizures treatment
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first line: lamotrigine or levetiracetam
second line: carbamazepine, oxcarbazepine or zonisamide
18
Q
Absence seizures presentation
A
19
Q
Absence seizures typically happen in which group?
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Children
20
Q
Absence seizures typically last how long
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10-20 seconds
21
Q
Atonic seizures definition/presentation
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22
Q
Atonic seizures may be indicative of which syndrome
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Lennox-Gastaut syndrome
23
Q
- Atonic seizures management
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First line: sodium valproate
Second line: lamotrigine
24
Q
Absence seizures management
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first line: ethosuximide
second line:
male: sodium valproate
female: lamotrigine or levetiracetam
carbamazepine may exacerbate absence seizures
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Myoclonic seizures presentation
Myoclonic seizures present as sudden brief muscle contractions, like a sudden “jump”. The patient usually remains awake during the episode
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Myoclonic seizures typically happen in… as part of which condition?
typically happen in children as part of juvenile myoclonic epilepsy
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Myoclonic seizures management
First line: sodium valproate
Other options: levetiracetam
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Infantile spasms presentation/definition
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Infantile spasms management
Prednisolone
Vigabatrin
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investigations and diagnosis of seizures and epilepsy in children
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When is an MRI brain used to investigate seizures in children (criteria)?
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* 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.
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Additional investigations can be considered to exclude other pathology that may cause seizures:
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General advice to give parents about epilepsy in children
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sodium valproateMOA and side effects
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Side effects of carbamazepine
Agranulocytosis
Aplastic anaemia
Induces the P450 system so there are many drug interactions
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Phenytoin SE
Folate and vitamin D deficiency
Megaloblastic anaemia (folate deficiency)
Osteomalacia (vitamin D deficiency)
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Ethosuximide SE
Night terrors
Rashes
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Lamotrigine SE
Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes.
Leukopenia
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Management of seizures
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Definition of status epilepticus
seizure lasting more than 5 minutes or 2 or more seizures without regaining consciousness in the interim.
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Management of status epileptics in the hospital
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Medical options in the community for management of status epilepticus
Buccal midazolam
Rectal diazepam
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infantile spasms typically present in
first 4 to 8 months of life and is more common in male infants
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* infantile spasms are often associated with
often associated with a serious underlying condition and carry a poor prognosis
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* 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
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* How long do infantile spasms last
only 1-2 seconds but may be repeated up to 50 times
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* 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)
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Management of infantile spasms
* poor prognosis
* vigabatrin is now considered first-line therapy
* ACTH is also used
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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
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Causes of cerebral palsy
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Types of cerebral palsy
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Patterns of spastic cerebral palsy
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Children at risk of developing cerebral palsy, such as those with
hypoxic-ischaemic encephalopathy
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CP presentation
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Complications and associated conditions in CP
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Management of CP
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Paediatricians will regularly see the child with CP to optimise their medications. This may involve:
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Which drug is used for excessive drooling in CP
Glycopyrronium bromide
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The differential diagnosis of an upper motor neurone lesion other than CP is
acquired brain injury or a tumour.
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Test for... to look for cerebellar involvement in CP
Test for coordination to look for cerebellar involvement
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Look for... that indicate extrapyramidal (basal ganglia) involvement in CP
Look for athetoid movements that indicate extrapyramidal (basal ganglia) involvement
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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.
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Patients with cerebral palsy may have a …or…gait
Patients with cerebral palsy may have a hemiplegic or diplegic gait
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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 flexion**of 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.
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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
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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.
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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.
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Congenital causes of hydrocephalus:
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Presentation of hydrocephalus
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Hydrocephalus management
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VP shunt complications
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Causes of headaches in children
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Tension headache presentation
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There are certain triggers for tension headaches in children
Stress, fear or discomfort
Skipping meals
Dehydration
Infection
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Tension headaches in children management
reassurance, analgesia, regular meals, avoiding dehydration and reducing stress.
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Types of migraines
Migraine without aura
Migraine with aura
Silent migraine (migraine with aura but without a headache)
Hemiplegic migraine
Abdominal migraine
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Migraine presentation including associated symptoms
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Management of migraines in children:
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Abdominal migraines presentation including associations
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In a child with a new headache, always check for symptoms and signs of which other conditions?
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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.
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Meningococcal septicaemia refers to
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Meningococcal meningitis is when
the bacteria meningococcus is infecting the meninges and the cerebrospinal fluid around the brain and spinal cord.
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The most common causes of bacterial meningitis in children and adults are
Neisseria meningitidis (meningococcus) and Streptococcus pneumoniae (pneumococcus).
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In neonates the most common cause of bacterial meningitis is
group B strep (GBS)
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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
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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**.
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* 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
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There are two special tests you can perform to look for meningeal irritation:
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Contraindications to lumbar puncture
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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.
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Post exposure prophylaxis for meningococcal infections
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The most common causes of viral meningitis are
herpes simplex virus (HSV)
enterovirus
varicella zoster virus (VZV)
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Investigations for viral meningitis
A sample of the CSF from the lumbar puncture should be sent for viral PCR testing.
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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**.
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Where in the spine does the needle go for a lumbar puncture? Lumbar puncture samples are sent for which tests at the lab?
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lumbar puncture results in bacterial vs viral meningitis
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Complications of meningitis
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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.
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Most common cause of encephalitis in children
herpes simple type 1 (HSV-1) from cold sores
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Most common cause of encephalitis in neonates
herpes simplex type 2 (HSV-2) from genital herpes, contracted during birth
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causes of encephalitis
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Presentation of encephalitis
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Children with features of encephalitis need some key investigations to establish the diagnosis:
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Contraindications to a lumbar puncture for encephalitis
GCS below 9 haemodynamically unstable
active seizures or post-ictal.
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Management of encephalitis
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Complications of encephalitis
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Tourette’s definition
the development of tics that are persistent for over a year
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Tics definition
Tics are involuntary movements or sounds that the child performs repetitively throughout the day
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Tics triggers
tics become more prominent when the person is under pressure or excited
- stress
- tired
- bored
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Tics usually decrease with:
* Concentration
* Exercise
* Distraction
* Sleep
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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.
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tics presenting age and associated conditions
Tics often present around or after 5 years of age. They can be associated with OCD and ADHD.
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examples of simple tics
* Clearing throat
* Blinking
* Head jerking
* Sniffing
* Grunting
* Eye rolling
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examples of complex tics
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management of tics
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conjunctivitis definition and presentation, and types
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bacterial vs viral conjunctivitis presentation
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causes of acute painful red eye
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causes of acute painless red eye
Conjunctivitis
Episcleritis
Subconjunctival haemorrhage
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Conditions that require emergency same-day referral to ophthalmology tend to cause
pain or reduced visual acuity
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Management of redeye
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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).
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Allergic conjunctivitis cause, presentation and management
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Periorbital cellulitis definition
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orbital cellulitis definition
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orbital and periorbital cellulitis risk factors
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orbital and periorbital cellulitis presentation
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Differentiating orbital from preseptal cellulitis
reduced visual acuity, proptosis, ophthalmoplegia/pain with eye movements are NOT consistent with preseptal cellulitis
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orbital and periorbital cellulitis investigations
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orbital and periorbital cellulitis management
admission to hospital for IV antibiotics due to the risk of cavernous sinus thrombosis and intracranial spread
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muscular dystrophies definition
Duchenne muscular dystrophy is an X-linked recessive inherited disorder in the dystrophin genes required for normal muscular function.
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muscular dystrophies aetiology
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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
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muscular dystrophies investigations
* raised creatinine kinase
* genetic testing has now replaced muscle biopsy as the way to obtain a definitive diagnosis
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muscular dystrophies management
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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
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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
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History and examination for a seizure event
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Childhood epileptic seizures causes
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focal seizure definition
* seizure origin within the network limited to one cerebral hemisphere
* most commonly arising from the temporal and frontal lobes
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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.
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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
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Syncope signs and symptoms
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Primary causes and secondary causes of syncope
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What to ask in a history of syncope
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Syncope vs seizure differences
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Syncope examination
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Syncope investigations
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Syncope management
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Reflex anoxic attacks definition and presentation
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Absence seizures definition and presentation
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Juvenile myoclonic epilepsy presentation, triggers and management
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Benign Rolandic epilepsy description, presentation and management
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Infantile spasms is a triad of:, management and consequences
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breath holding spells types
cyanotic breath holding spells and pallid breath holding spells (also known as reflex anoxic seizures).
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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.
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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.
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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.
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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.
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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.
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Raised intracranial pressure definition and pathophysiology
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Raised intracranial pressure causes
idiopathic intracranial hypertension
traumatic head injuries
infection: meningitis
tumours
hydrocephalus
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Raised intracranial pressure features
headache
vomiting
reduced levels of consciousness
papilloedema
Cushing's triad:
- widening pulse pressure
- bradycardia
- irregular breathing
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Raised intracranial pressure investigations and monitoring
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Raised intracranial pressure management
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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.
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Subarachnoid haemorrhage risk factors: general risk factors, associated particularly with, more common in
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Subarachnoid haemorrhage presentation
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Subarachnoid haemorrhage investigations
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Subarachnoid haemorrhage management
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Subarachnoid haemorrhage complications and their management
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triad of the shaken baby syndrome
Retinal haemorrhages, subdural haematoma and encephalopathy
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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."
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spastic cerebral palsy manifests as:
spastic (70%)
subtypes include hemiplegia, diplegia or quadriplegia
increased tone resulting from damage to upper motor neurons
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dyskinetic CP caused by:
dyskinetic
caused by damage to the basal ganglia and the substantia nigra
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ataxic CP is caused by:
ataxic
caused by damage to the cerebellum with typical cerebellar signs
