Paediatrics- Neuro, Development Flashcards
WHat is cerebral Palsy
Cerebral palsy (CP) is the name given to the permanent neurological problems resulting from damage to the brain around the time of birth.
It is not a progressive condition, however the nature of the symptoms and problems may change over time during growth and development.
There is huge variation in the severity and type of symptoms, ranging from completely wheelchair bound and dependent on others for all activities of daily living, to para-olympic athletes with only subtle problems with coordination or mobility.
Causes of Cerebral Palsy can be split into antenatal, perinatal and postnatal.
Name some causes for each?
Antenatal:
- Maternal infections
- Trauma during pregnancy
Perinatal:
- Birth asphyxia
- Pre-term birth
Postnatal:
- Meningitis
- Severe neonatal jaundice
- Head injury
Type of Cerebral Palsy
Spastic
Dyskinetic
Ataxic
Mixed
Spastic CP is also known as ______ CP.
Dyskinetic CP is also known as ______ CP and _________ CP.
Spastic CP is also known as pyramidal CP. Dyskinetic CP is also known as athetoid CP and extrapyramidal CP.
What is Spastic CP
Spastic: hypertonia (increased tone) and reduced function resulting from damage to upper motor neurones
WHat is Dyskinetic CP
Dyskinetic: problems controlling muscle tone, with hypertonia and hypotonia, causing athetoid movements and oro-motor problems. This is the result of damage to the basal ganglia.
What is ataxic CP
Ataxic: problems with coordinated movement resulting from damage to the cerebellum
What is mixed CP
Mixed: a mix of spastic, dyskinetic and/or ataxic features
Name the patterns of spastic CP
- Monoplegia: one limb affected
- Hemiplegia: one side of the body affected
- Diplegia: four limbs are affects, but mostly the legs
- Quadriplegia: four limbs are affected more severely, often with seizures, speech disturbance and other impairments
Children at risk of developing cerebral palsy, such as those with ______ ________ _____________, need to be followed up to identify any signs and symptoms that develop.
Children at risk of developing cerebral palsy, such as those with hypoxic-ischaemic encephalopathy, need to be followed up to identify any signs and symptoms that develop.
Signs and symptoms of cerebral palsy will become more evident during development:
Failure to meet milestones
Increased or decreased tone, generally or in specific limbs
Hand preference below 18 months is a key sign to remember for exams
Problems with coordination, speech or walking
Feeding or swallowing problems
Learning difficulties
You can gain a lot of information about a child from their gait:
What do these words mean?
Hemiplegic / diplegic gait:
Broad based gait / ataxic gait:
High stepping gait:
Waddling gait:
Antalgic gait (limp):
Hemiplegic / diplegic gait: indicates an upper motor neurone lesion
Broad based gait / ataxic gait: indicates a cerebellar lesion
High stepping gait: indicates foot drop or a lower motor neurone lesion
Waddling gait: indicates pelvic muscle weakness due to myopathy
Antalgic gait (limp): indicates localised pain
Explain the UMN and LMN findings of
Inspection
TOne
Power
Reflexes
Patients with cerebral palsy may have a _______ or ______ gait. This gait is caused by increased muscle tone and spasticity in the legs. The leg will be _______ with ______ _________ 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.
Patients with cerebral palsy may have a hemiplegic or diplegic gait. 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.
CP
They will have signs of an ______ ______ ____ lesion with good muscle bulk, increased tone, brisk reflexes and slightly reduced power.
Power may be _______. Look for ________ movements that indicate extrapyramidal (basal ganglia) involvement. Test for coordination to look for cerebellar involvement.
They will have signs of an upper motor neurone lesion, with good muscle bulk, increased tone, brisk reflexes and slightly reduced power.
Power may be normal. Look for athetoid movements that indicate extrapyramidal (basal ganglia) involvement. Test for coordination to look for cerebellar involvement.
CP
The differential diagnosis of an upper motor neurone lesion is ________ _____ ______ or ______
The differential diagnosis of an upper motor neurone lesion is acquired brain injury or tumour.
Complications and Associated Conditions with CP
Learning disability
Epilepsy
Kyphoscoliosis
Muscle contractures
Hearing and visual impairment
Gastro-oesophageal reflux
Management of CP
Physiotherapy is used to stretch and strengthen muscles, maximise function and prevent muscle contractures.
Occupational therapy is used to help patients manage their everyday activities, such as getting dressed and using the bathroom. That can involve techniques to perform tasks despite disability. They can also make adaptations and supply equipment, such as rails for assistance or fitting a hoist for a patient who is entirely wheelchair bound.
Speech and language therapy can help with speech and swallowing. When swallowing difficultly prevents them meeting their nutritional requirements they may require an NG tube or PEG tube to be fitted.
Dieticians can help ensure they meet nutritional requirements. Some children may require PEG feeding through a port on their abdomen that gives direct access to the stomach.
Orthopaedic surgeons can perform procedures to release contractures or lengthen tendons (tenotomy).
Paediatricians will regularly see the child to optimise their medications. This may involve:
- Muscle relaxants (e.g. baclofen) for muscle spasticity and contractures
- Anti-epileptic drugs for seizures
- Glycopyrronium bromide for excessive drooling
Social workers to help with benefits and support.
Charities and support groups provide opportunities to connect with others affected by cerebral palsy and learn and share information on the condition.
What are febrile convulsions
Febrile convulsions are 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. By definition, febrile convulsions occur only in children between the ages of 6 months and 5 years.
What are Simple Febrile Convulsions
Simple febrile convulsions are generalised, tonic clonic seizures. They last less than 15 minutes and only occur once during a single febrile illness.
What are Complex Febrile Convulsions
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.
Diagnosis of Febrile convulsions
In order the make a diagnosis of a febrile convulsion, other neurological pathology must be excluded. The differential diagnoses of a febrile convulsion are:
- Epilepsy
- Meningitis, encephalitis or another neurological infection such as cerebral malaria
- Intracranial space occupying lesions, for example brain tumours or intracranial haemorrhage
- Syncopal episode
- Electrolyte abnormalities
- Trauma (always think about non accidental injury)
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.
Management of febrile convulsions
I
n 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.
Give parents advice on managing a seizure if a further episode occurs:
- Stay with the child
- Put the child in a safe place, for example on a carpeted floor with a pillow under their head
- Place them in the recovery position and away from potential sources of injury
- Don’t put anything in their mouth
- Call an ambulance if the seizure lasts more than 5 minutes
The first seizure should always result in a trip to hospital for assessment, however if parents are confident in subsequent events and can safely manage the child at home then they can visit their GP at the next available opportunity.
Prognosis
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
WHat is Epilepsy
Epilepsy is an umbrella term for a condition where there is a tendency to have seizures. Seizures are transient episodes of abnormal electrical activity in the brain. There are many different types of seizure.
A diagnosis of epilepsy is made by a specialist based on the characteristics of the seizure episodes.
What are the different types of Seizures
Generalised Tonic-Clonic Seizures
Focal Seizures
Absence Seizures
Atonic Seizures
Myoclonic Seizures
Infantile Spasms
Febrile Convulsions
What are Generalised Tonic-Clonic Seizures
Explain its management
These are what most people think of with an epileptic seizure. There is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking) movements. Typically the tonic phase comes before the clonic phase. There may be associated tongue biting, incontinence, groaning and irregular breathing.
After the seizure there is a prolonged post-ictal period where the person is confused, drowsy and feels irritable or low.
Management of tonic-clonic seizures is with:
- First line: sodium valproate
- Second line: lamotrigine or carbamazepine
What are Focal Seizures
Explain its management
Focal seizures start in the temporal lobes. They affect hearing, speech, memory and emotions. There are various ways that focal seizures can present:
- Hallucinations
- Memory flashbacks
- Déjà vu
- Doing strange things on autopilot
One way to remember the treatment is that the choice of medication is the reverse of tonic-clonic seizures:
- First line: carbamazepine or lamotrigine
- Second line: sodium valproate or levetiracetam
What is Absence Seizures
What is the management
Absence seizures typically happen in children. The patient becomes blank, stares into space and then abruptly returns to normal. During the episode they are unaware of their surroundings and won’t respond. These typically only lasts 10 to 20 seconds. Most patients (more than 90%) stop having absence seizures as they get older. Management is:
- First line: sodium valproate or ethosuximide
What is Atonic Seizures
What is the management
Atonic seizures are also known as drop attacks. They are characterised by brief lapses in muscle tone. These don’t usually last more than 3 minutes. They typically begin in childhood. They may be indicative of Lennox-Gastaut syndrome. Management is:
- First line: sodium valproate
- Second line: lamotrigine
What are Myoclonic Seizures
Explain its management
Myoclonic seizures present as sudden brief muscle contractions, like a sudden “jump”. The patient usually remains awake during the episode. They occur in various forms of epilepsy but typically happen in children as part of juvenile myoclonic epilepsy. Management is:
- First line: sodium valproate
- Other options: lamotrigine, levetiracetam or topiramate
What are Infantile Spasms
Explain its manageement
This is also known as West syndrome. It is a rare (1 in 4000) disorder starting in infancy at around 6 months of age. It is characterised by clusters of full body spasms. There is a poor prognosis: 1/3 die by age 25, however 1/3 are seizure free. It can be difficult to treat but first line treatments are:
- Prednisolone
- Vigabatrin
Investigations and Diagnosis for Epilepsy
A good history is the key to a diagnosis of epilepsy. It is important to establish that any episodes were seizures, as opposed to vasovagal episodes or febrile convulsions. Try to identify the type of seizure. Patients with a clear history of a febrile convulsion or vasovagal episode do not require further investigations.
An electroencephalogram (EEG) can show typical patterns in different forms of epilepsy and support the diagnosis. Perform an EEG after the second simple tonic-clonic seizure. Children are allowed one simple seizure before being investigated for epilepsy.
An MRI brain can be used to visualise the structure of the brain. It is used to diagnose structural problems that may be associated with seizures and other pathology such as tumours. It should be considered when:
- The first seizure is in children under 2 years
- Focal seizures
- There is no response to first line anti-epileptic medications
Additional investigations can be considered to exclude other pathology that may cause seizures:
- ECG to exclude problems in the heart.
- Blood electrolytes including sodium, potassium, calcium and magnesium
- Blood glucose for hypoglycaemia and diabetes
- Blood cultures, urine cultures and lumbar puncture where sepsis, encephalitis or meningitis is suspected
WHat is the General Advice for children with epilepsy
Patients and families presenting with seizures need to be given advice about safety precautions, recognising, managing and reporting further seizures. It is important to avoid situations where a seizure may put the child in danger, with advise to:
- Take showers rather than baths
- Be very cautious with swimming unless seizures are well controlled and they are closely supervised
- Be cautious with heights
- Be cautious with traffic
- Be cautious with any heavy, hot or electrical equipment
Older teenagers with epilepsy will need to avoid driving unless they meet specific criteria regarding control of their epilepsy. These rules change frequently so it is always worth looking them up if advising patients.
SE of Sodium Valproate
This is a first line option for most forms of epilepsy (except focal seizures). It works by increasing the activity of GABA, which has a relaxing effect on the brain. Notable side effects of sodium valproate include:
- Teratogenic, so patients need careful advice about contraception
- Liver damage and hepatitis
- Hair loss
- Tremor
There are a lot of warning about the teratogenic effects of sodium valproate and NICE updated their guidelines in 2018 to reflect this. It must be avoided in girls unless there are no suitable alternatives and strict criteria are met to ensure they do not get pregnant.
SE of Carbamazepine
This is first line for focal seizures. Notable side effects are:
- Agranulocytosis
- Aplastic anaemia
- Induces the P450 system so there are many drug interactions
*
SE of Phenytoin
Notable side effects:
- Folate and vitamin D deficiency
- Megaloblastic anaemia (folate deficiency)
- Osteomalacia (vitamin D deficiency)
SE of Ethosuximide
Notable side effects:
Night terrors
Rashes
SE of Lamotrigine
Notable side effects:
- Stevens-Johnson syndrome or DRESS syndrome. These are life threatening skin rashes.
- Leukopenia
General Management of Seizures
- Put the patient in a safe position (e.g. on a carpeted floor)
- Place in the recovery position if possible
- Put something soft under their head to protect against head injury
- Remove obstacles that could lead to injury
- Make a note of the time at the start and end of the seizure
- Call an ambulance if lasting more than 5 minutes or this is their first seizure
What is Status Epilepticus
Status epilepticus is an important condition you need to be aware of and how to treat. It is a medical emergency.
It is defined as a seizure lasting more than 5 minutes or 2 or more seizures without regaining consciousness in the interim.
Management of status epileptics in the hospital (take an ABCDE approach):
- Secure the airway
- Give high-concentration oxygen
- Assess cardiac and respiratory function
- Check blood glucose levels
- Gain intravenous access (insert a cannula)
- IV lorazepam, repeated after 10 minutes if the seizure continues
If the seizures persist the final step is an infusion of IV phenobarbital or phenytoin. At this point intubation and ventilation to secure the airway needs to be considered, along with transfer to the intensive care unit if appropriate
Medical options in the community: for seizures
Buccal midazolam
Rectal diazepam
What are Breath holding spells
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. They typically occur 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.
Breath holding spells are often divided into two types
cyanotic breath holding spells and pallid breath holding spells (also known as reflex anoxic seizures).
When do Cyanotic Breath Holding Spells occur
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.
When do Reflex Anoxic Seizures occur
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.
Management of Breath Holding Spells
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.
Causes of Headaches in Children
- Tension headaches
- Migraines
- Ear, nose and throat infection
- Analgesic headache
- Problems with vision
- Raised intracranial pressure
- Brain tumours
- Meningitis
- Encephalitis
- Carbon monoxide poisoning
What are Tension Headaches
What are its symptoms and Triggers
Management?
Tension headaches are very common. Classically they produce a mild ache across the forehead and pain or pressure in a band-like pattern around the head. Tension headaches comes on and resolve gradually and don’t produce visual changes or pulsating sensations. They are typically symmetrical.
Symptoms may be very non-specific in younger children. They may become quiet, stop playing, turn pale or become tired. They tend to resolve more quickly in children compared with adults, often within 30 minutes.
There are certain triggers for tension headaches in children:
- Stress, fear or discomfort
- Skipping meals
- Dehydration
- Infection
Management is with reassurance, analgesia, regular meals, avoiding dehydration and reducing stress.
What are Migraines
What are the different types
Migraines are a complex neurological condition that cause headache and other associated symptoms. They occur in “attacks” that often follow a typical pattern.
There are several types of migraine:
- Migraine without aura
- Migraine with aura
- Silent migraine (migraine with aura but without a headache)
- Hemiplegic migraine
- Abdominal migraine
Migraines present differently to tension headaches. Symptoms tend to be:
Unilateral
More severe
Throbbing in nature
Take longer to resolve
Migraines are often associated with:
Visual aura
Photophobia and phonophobia
Nausea and vomiting
Abdominal pain
Management of migraines in children:
- Rest, fluids and low stimulus environment
- Paracetamol
- Ibuprofen
- Sumatriptan
- Antiemetics, such as domperidone (unless contraindicated)
Where the migraines are having a significant impact on life, for example frequent attacks or missing school, prophylactic treatment can be tried to reduce the frequency and severity of the migraines. This is usually guided by a specialist. Options for migraine prophylaxis are:
- Propranolol (avoid in asthma)
- Pizotifen (often causes drowsiness)
- Topiramate (girls with child bearing potential need highly effective contraception as it is very teratogenic).
Children are more likely than adults to suffer with a condition called abdominal migraine. This may occur in young children before they develop traditional migraines as they get older. They present with episodes of central abdominal pain lasting more than 1 hour. Examination will be normal. There may be associated:
Nausea and vomiting
Anorexia
Headache
Pallor
When a patient presents with possible migraines what do you ask about
recurrent central abdominal pain as a child.
They may have a history of abdominal migraine that started before the headaches
What is Hydrocephalus
Hydrocephalus describes 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.
WHat are Normal CSF Physiology
There are four ventricles in the brain: two lateral ventricles, the third and the fourth ventricles. The ventricles containing CSF. The CSF provides a cushion for the brain tissue. CSF is created in the four choroid plexuses (one in each ventricle) and by the walls of the ventricles. CSF is absorbed into the venous system by the arachnoid granulations.
Congenital Causes hydrocephalu
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.
Other causes:
- Arachnoid cysts can block the outflow of CSF if they are large enough
- Arnold-Chiari malformation is where the cerebellum herniates downwards through the foramen magnum, blocking the outflow of CSF
- Chromosomal abnormalities and congenital malformations can cause obstruction to CSF drainage.
Presentation of hydrocephalus
The cranial bones in babies are not fused at the sutures until around 2 years of age. Therefore, the skull is able to expand to fit the cranial contents. When a baby has hydrocephalus it causes outward pressure on the cranial bones. Therefore, babies with hydrocephalus will have an enlarged and rapidly increasing head circumference (occipito-frontal circumference).
Other signs:
- Bulging anterior fontanelle
- Poor feeding and vomiting
- Poor tone
- Sleepiness
What is Ventriculoperitoneal Shunt
Placing a VP shunt that drains CSF from the ventricles into another body cavity is the mainstay of treatment for hydrocephalus. Usually the peritoneal cavity is used to drain CSF, as there is plenty of space and it is easily reabsorbed. The surgeon places a small tube (catheter) through a small hole in the skull at the back of the head and into one of the ventricles. A valve on the end of this tube is placed subcutaneously, and a catheter on the other side of the valve runs under the skin into the peritoneal cavity. The valve helps to regulate the amount of CSF that drains from the ventricles.
VP Shunt Complications
- Infection
- Blockage
- Excessive drainage
- Intraventricular haemorrhage during shunt related surgery
- Outgrowing them (they typically need replacing around every 2 years as the child grows)
What is Syncope
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.
What is the difference between syncope and seizure
