Neurology Flashcards
What % of children end up having a febrile convulsion and over what age range is it most common?
4%
Happens in children aged 6m-6y but most common below 2y
What are the most common sources of infection that might lead to febrile convulsion?
UTI
Otitis media
URTI - or tonsillitis
How does a febrile seizure present?
The parent might not know the child is febrile beforehand
Seizures usually tonic-clonic in nature and last <15mins
There is usually post-octal drowsiness (no longer than hour)
Might be some apnoea and peripheral cyanosis
What % of febrile convulsions recur?
30-50% but children will grow out of them by the time they are 6y
What features make febrile conclusions atypical?
More than 1 in same febrile illness
Lasting longer than 15mins
Focalising features (unilateral or sensory)
If the child is found to have a cerebral infections (e.g. meningitis this is NOT a FebCon)
Is there an increased risk of epilepsy with febrile convulsion?
FebCon IS NOT THE SAME AS EPILEPSY (important to remember this)
If the child has a family hx of epilepsy there chance of developing it after having a Febrile seizure is 1% (Up from 0.5% background risk)
How should you advise a parent to manage a febrile seizure in the future?
Time it + film it if possible
Remove anything from around the child that they could harm themselves with
Place a pillow under their head
DO NOT place anything in their mouth
Do NOT restrain them
If it continues for longer than 15 minutes then call and ambulance
How could you consider managing febrile convulsion in secondary care?
If not stopping after 5 minutes consider intervention
BUCCAL MIDAZOLAM 0.5mg/kg
Wait 10 mins - if not resolves
IV LORAZEPAM 0.1mg/kg
If still seizing after 30 mins they are in STATUS and need to consider senior anaesthetic advice and phenytoin
Can you give anti-pyretics in febrile convulsions?
PARACETAMOL does NOT reduce the rate of febrile seizure (no relation between degree of fever and seizure)- however still encourage parents to give it as it will make the child more comfortable
What are some examples of seizure mimics in the paediatric patient?
Breath holding attacks
Syncope
Psychologically determine paroxysmal events
What is a breath-holding attack and when does it occur?
- Can be self-induced by tantrum or occur after excessive crying
- Blue and breath holding
- Children can fall to floor and can sometimes twitch on way down/just after which can appear like a seizure, although it is just due to vasovagal response (RELFEX ANOXIC SEIZURE)
What is syncope and when does it occur?
What may it be confused with?(and how do you know its not that)
- Just fainting, often vasovagal episodes just like in adults
- Usually occurs from 7m onwards
- Might happen more with certain RFx: head banging, fright, sudden standing, hair brushing
- Usually there is an AURA
- Might also be MYOCLONIC JERKY making it seem like seizure (usually lasts <20s making you think it’s not seizure)
What is characteristic of physiologically determined paroxysmal events?
Thrashing movements that wax or wane (e.g. pelvic thrusting)
Slumping to the floor in dramatic fashion
The child might have some sort of gain from situation (e.g. parent’s attention)
Rapid return to normal
Other than FebCon and mimics what are some other causes of seizure in children?
- Meningitis or encephalitis
- Head trauma
- Metabolic e.g. DKA
- Tumours
- Toxins e.g. cocaine
- Cardiac arrhythmias
- GORD EVENTS (pulling up of legs can seem like seizure)
What is cerebral palsy?
CP is a lifelong disorder of movement and coordination caused by damage to the brain before, during or shortly after birth
- There are many different causes CP just describes the disorder
What are some pre-natal causes of CP?
Congenital malformations (poor migration of brain cells)
Poor, pre-natal myelination of nerve cell fibres
Prenatal TORCH infections (toxoplasmosis, cytomegalovirus, rubella and herpes)
What are some peri-natal causes of CP?
Usually hypoxia is the cause - either asphyxia or major haemorrhage leading to hypo perfusion of brain tissue
What are some post-natal causes of CP?
Non-functional connections between brain cells (sustained hypoxia, infection or trauma)
Hyperbilirubinaemia or neonatal stroke
Is CP more common in pre-term babies?
YES
What is the most common movement type in CP and what are some sub-types of this?
SPASTIC CEREBRAL PALSY Described depending on which limbs are affected... MONOPLEGIC (just one limb - RARE) DIPLEGIC (one half of body usually LEGS) HEMIPLEGIC (either R or L) QUADRIPLEGIC (all 4 limbs)
What is spasticity?
This is a STRETCH RELATED RESPONSE
Velocity-dependent increased resistance to stretch
What are some signs and symptoms of spastic cerebral palsy? Think about each joint
Clasp-knife spasticity - initially lots of resistance then snaps down
Ankle plantar flexion and usually valgus or varus foot deformity
HIP - flexion, internal rotation, limited adduction
WRIST - flexed and pronated
ELBOW: flexed
SHOULDER: adducted
What is choreoathetosis ?
This is another movement disorder associated with CP
It is caused by problems at basal ganglia and causes jerky movements
Greatly increased tone while awake and slightly reduced in early stages of sleep
What is ataxia?
Another form of CP
Children have striking loss of balance in their early years
Hypoxia and ischaemia known causative factors for this
How do we rate the severity of CP?
Using the GMFCS
Gross Motor Function Classification System
How might CP present to you?
- Unusual fidgeting common
- Asymmetry or paucity of movement
- Unusually floppy or unusually rigid
- GROSS MOTOR DELAY (not sitting by 8 months, not walking by 18 months or asymmetry of hands early on)
- Feeding and nutrition - pseudo bulbar palsy (can’t control facial movements)
- Many Chesty LRTIs
- Bladder infections and incontinence
- Bowel constipation
How would you investigate/diagnose CP?
Might be able to of from hx alone - always go back and ask about hx of prematurity or birth complications
MRI - if spastic CP suspected get it of pyramidal tract and if it is another form of CP get it of basal ganglia
How should CP be managed?
MDT input: Drs, specialist nurse, physio, OT, SALT
Focussed around improving posture and movement - stretching exercises, orthoses, wheelchair, botulinum toxin
BACLOFEN - can be given to relax muscles
SELECTIVE DORSAL RHIZOTOMY (SDR)
Treat sequelae (seizures, constipation, nutrition and psychological)
What other neurological deficits are children with CP likely to develop?
Hearing and vision Speech Learning difficulties Epilepsy Nutritional impairment Psychiatric
What are some examples of primary headaches that chidden can get?
Migraines
Tension headaches
Cluster headaches
Cough/exertion headache
How does a migraine present?
Aura? (only 10%) can last from 1-72hrs Can be pustule over frontal/temporal area Can be associated with GI sx Photophobia Can be familial and cyclical
How does a tension-type headache present?
Feels like a tight band across forehead
Usually no other symptoms
What are some examples of secondary headaches?
These are headaches that are due to underlying pathology
Usually caused by increased ICP or SOL
What is a basilar type migraine?
This is a headache that is associated with vomiting and more cerebellar type symptoms such as DANISH
Describe a characteristic headache associated with a SOL?
Worse when lying down
Associated with vomiting
Might be change in mood, personality or educational performance
What other symptoms might you find with a child with a SOL?
Visual field defects Cranial nerve defects Diplopia Gait abnormalities Changes in character or personality Growth failure Papilloedema
What can we do to manage ACUTE migraines in children?
- Reassurance and basic analgesia (para and Ibo (better) 2.If that doesnt work give 5HT1- receptor agonist SUMATRIPTAN (orally from 6 years and nasally from 12 years
May also need an anti emetic (Domperidone for 12+)
Anti-emetics are recommended. Domperidone is licensed in children of all ages, and prochlorperazine is licensed in children older than 12 years of age. Domperidone can also be used, in the absence of nausea and vomiting, to promote gastric motility.
When can you consider prophylactic migraine treatment for children?
What can you use?
Prophylaxis used if:
- suffer at least two attacks a month
- suffer significant disability
- cant take other treatment
Propranolol (CI in asthma)
Pizotifen (SE: droopiness and weight gain)
Topiramate
What is hydrocephalus?
How is hydrocephalus treated?
This is when there is an abnormality meaning the CSF that is constantly being produced cannot be cleared from the brain and builds up.
In children because the suture lines have not formed this means the fluid can collect and stretch the cranium
***if this happens in utero this will mean a C-S is required
Treatment
A SHUNT is formed between the ventricular system and the peritoneum - excess fluid drains into the abdomen
What are some congenital causes of hydrocephalus?
- Congenital malformation of outflow foramina of the fourth ventricle - DANDY-WALKER SYNDROME
- Arnold-Chiari malformation of the cerebral aqueduct
- Aqueduct stenosis
What are some other causes of hydrocephalus?
Communicating
- SAH
- infection
Non communicating (obstruction)
- E.g. SOL, intra vent heamorrage, infection (web)
- do not LP
What is plagiocephaly?
Abnormal shaping or flattening on one side of baby’s head
What causes plagiocephaly?
- Usually it is caused by the baby lying on their back for long periods of time and this is not concerning
- Can happen in utero
- Can happen due to cranio-synostosis - premature fusion of suture lines
Who is plagiocephaly more common in?
Plagiocephaly more common in
- Prems
- Twins
- Breech babies
What are the two forms?
Synostotic - two or more sutures in baby’s head have fused
Non-Synostotic - no fusion
How should plagiocephaly be managed?
- Encourage allowing the baby to play on its stomach
- Physical therapy and orthotics can help where conservative management fails
What is a neuroblastoma and who does it occur in?
It is a tumour of neural crest tissue that occurs in the adrenal medulla and sympathetic nervous system
Most common in children aged under 5
How will children with neuroblastoma present?
- Usually with abdominal mass that is coming from adrenal medulla
- Technically tumour can occur anywhere down sympathetic chain
- Usually the symptoms that are presenting are of metastatic disease
- Bone pain
- Myelosuppression
- Weight loss
- Malaise
How would we make/confirm a diagnosis of neuroblastoma?
- RAISED URINE CATECHOLAMINES (VMA, HVA)
- Biopsy
- Bone marrow sampling
- Bone scan
- Meta-iodobenzyl guanidine scan
What is the prognosis for neuroblastoma?
- Depends on age of presentation
- BUT if they are older than 1 year then their prognosis is very poor - usually because metastasis rate is so high
What is spina bifida?
- Poor formation of neural tube in first 28 days post-conception
- FAILURE OF FUSION OF SPINAL ARCH
- This happens to differing degrees
What should be done to prevent spina bifida?
-Prophylactic folic acid supplementation (400mcg) or 5mg in women with epilepsy or diabetes
How does spina bifida present?
Can range from incidental finding on x-ray to very obvious external abnormality:
- May have large lump of neural tissue on back
- Or may have skin changes such as unusual hairiness, skin dimpling or pigmentation over the spine
- ALWAYS CHECK DURING NEWBORN EXAM
- Bladder or lower limb dysfunction
- loss of sensation around bottom
What level of the spine in spina bifida most concerning at?
If lesion is above L3
How should we investigate spina bifida?
- MRI and USS
- Always assess power and tone of the legs as well as bladder and bowel function
What are the different types of spina bifida?
- Meningocele -just meninges (covering layer of skin)
- Myelomeningocele - this is when there is a considerable portion of neural tissue external also
How is spina bifida managed?
SURGERY SOON AFTER BIRTH
- Check for hydrocephalus
- Refer to physio for management of power and tone
- Indwelling catheter
- Check renal function, HTN and UTI regularly
- Monitor for scoliosis
What is West Syndrome?
A type of epilepsy that commonly presents between 4-8 months of age
Repeated extension and flexion attacks up to 50 times
- Most children with infantile spasms have intellectual disabilities later in life (5% will die)
- Tuberous sclerosis
Who/what age does muscular dystrophy present?
Symptoms of muscular dystrophy?
Diagnostic test?
-Muscular Dystrophy presents around 3-5 years BOYS (x linked recessive)
Symptoms
- frequent falls
- clumsy movement
- delay in walking
- tip toe walking
- large calves (fatty deposits)
- shoulder, arm or face weakness too
- heart problems and scoliosis
- GOWERS SIGN: hard to stand from lying or sitting
1st line Diagnostic test is Muscle biopsy
What medications are used for tics (after self help and behavioural therapy)
Antisychotics are used for tics (e.g. risperidone, aripiprazole.
How do you make a diagnosis of epilepsy?
• Epilepsy is diagnosed when a AT LEAST 2 UNPROVOKED SEIZURE OCCURING >24 HOURS APART
What type of epilepsy comes on at night?
How do they look?
Diagnosis?
Managment?
BECTS-Benign epilepsy with centro-temporal spikes/benign focal epilepsy
A benign type of focal seizure (15% of children epilepsy)
15% of children epilepsy
Appearance
- can’t talk
- saliva
- face twitching
Diagnosis
-EEG Temporal spikes (rolandic area)
Management
- can lead to tonic clonic
- no treatment-excellent prognosis
Memory help - focus on having a becks at night
How does Juvenile myoclonic epilepsy present
JME- Juvenile myoclonic epilepsy is the most common generalised siezure in kids
(kid that doesn’t want to go to school so dropping stuff)
- teenagers, clumsy in the morning (dropping stuff)
- triggered by TV
- early morning jerks
What is the treatment for focal/partial seizures?
Carbamazepine
• Focal/partial seizures
• Second line in generalized tonic clonic
• Avoid in myoclonic-may worsen
Whats the treatment for generalised seizures (including absent)
Sodium valproate
• Generalized seizures
•Absence seizures
