Paediatric Neurology Flashcards
Prodrome for syncope
Hot, clammy or sweaty
Dizzy
Vision going blurry
Headache
Primary syncope causes
Dehydration
Missed meals
Extended standing
Stimulus - surprise, pain or sight of blood
Secondary causes of syncope
Hypoglycaemia Dehydration Anaemia Infection Anaphylaxis Arrhythmias Valvular heart disease Hypertrophic obstructive cardiomyopathy
Investigations for syncope
ECG or 24hr ECG if paroxysmal arrythmia
Echocardiogram
Bloods - FBC, electrolytes and blood glucose
Epilepsy pathophysiology
Tendency to have seizures in which there is abnormal electrical activity
Generalised tonic clonic seizures
Loss of consiousness
Tonic - muscle tensing
Clonic - muscle jerking
Associated symptoms: - tongue biting - incontinence - irregular breathing -
Post ictal period
Period after the seizure where the patient is confused, drowsy and irritable which lasts a while
Management of a generalised tonic clonic seizure
First line - sodium valproate
Second line - lamotrigine or carbamazepine
Focal seizure features
- start in temporal lobes
- affect hearing, speech, memory and emotions
Associated features:
- hallucinations
- memory flashbacks
- Deja vu
- doing strange things on auto pilot
Management of focal seizures
First line: lamotrigine ot carbamazepine
2nd line: sodium valproate or levetiracetam
Absence seizure features
Patient becomes blank, stares into space and wont respond then abruptly returns to normal
Lasts 10 to 20 seconds
Managment of absence seizures
First line: sodium valproate or ethosuximide
Atonic seizure pathophysiology
Drop attacks - brief lapses in muscle tone
Lasts for less than 3 minutes
May indicate lennox gastaut syndrome
Atonic seizure management
1st line - sodium valproate
2nd line - lamotrigine
Myoclonic seizure pathophysiology
Sudden brief muscle contractions like a sudden jump when awake
Management of myoclonic seizures
1st line: sodium valproate
2nd line: lamotrigine, levetiracetam or topiramate
West syndrome pathophysiology
Infantile spasms starting at 6 months
Clusters of full body spasms
Treatment of infantile spasms
Prednisolone and vigabatrin
Features of febrile convulsions
- Occur in children aged 6 months to 6 years
- Seizures due to fever
- Paracetamol will not decrease the number of seizures
- < 15 mins - simple
- > 15 mins, multiple or focal - complex
Investigations for epilepsy
EEG - after second simple tonic clonic seizure
MRI - rule our tumours, encephalopathy
Bloods - electrolytes
Blood glucose - hypoglycaemia
Blood, urine cultures and LP - if meningitis, encephalitis or sepsis is suspected
General advice for seizures
Take showers rather than baths Cautious about swimming Caution with heights Caution with traffic Caution with heavy, hot or electrical equipment
Side effects of sodium valproate
Teratogenic - contraception advice
Liver damage and hepatitis
Hair loss
Tremors
Side effects of carbamazepine
Agranulocytosis
Aplastic anaemia
P450 inducer
Side effects of phenytoin
Folate and vitamin D deficiency - megaloblastic anaemia and osteomalacia
Side effects of ethosuximide
Night tremors
Rashes
Side effects of lamotrigine
Steven - Johnson syndrome
Leukopenia
Management of seizures in general
Recovery position or safe position
Put something soft under their head
Remove potential hazards
Make a note of the time and end of seizure
Call an ambulance if it lasts > 5 minutes
Status epilepticus
Seizure lasting for more than 5 mins
2+ seizures without regaining consciousness in the interim
Management of status epilepticus in the hospital
A-E - high flow oxygen
IV lorazepam - repeated after 10 mins if seizures continue
If seizure persists - IV phenobarbital or phenytoin
Medical management for seizures in the community
Buccal midazolam
Rectal diazepam
Reflex anoxic seizures
Occurs when child is startled as vagus nerve sends signals to heart to transiently stop beating
Child goes pale, loses consciousness and may have muscle twitching
Regains consciousness in 30 seconds
Causes of headaches in children
Primary:
- tension headache
- migraine
- cluster headache
Secondary:
- sinusitis
- SOL
- meningitis or encephalitis
- carbon monoxide poisoning
- ENT infection
- problems with vision
Features of tension headaches
Site - band like pattern around the head, symmetrical
Onset - gradual
Character - ache
Relieving factors - analgesia, hydration
Aggravating factors - dehydration, stress, skipping meals and infection
Timing - approx 30 mins
Associated features - pale, tired
Features of migraines
Site - unilateral, often forehead or eyes
Onset - can be gradual or sudden
Character - sharp, throbbing
Relieving factors - dark room, rest, analgesia (triptans)
Aggrevating factors - stress, solvents, dehydration
Timing - takes longer to resolve
Associated features - photophobia, aura, nausea, abdo pain
Prophylaxis for migraines and when to use it
Propranolol
Pizotifen
Topiramate
Significantly impacting life
Frequent - > 1 per week
Cerebral palsy
Permanent neurological problems resulting from damage to the brain around the time of birth
Causes of cerebral palsy
Antenatal:
- maternal infections
- trauma during pregnancy
Perinatal:
- birth asphyxia
- preterm
Post-natal:
- meningitis
- severe neonatal jaundice
- head injury
Types of cerebral palsy
Spastic - hypertonia and reduced function
Dyskinetic - hypertonia and hypotonia causing athetoid movement and oro-motor problems
Ataxic - problems with coordinated movements
Mixed
What causes spastic, dyskinetic and ataxic cerbral palsy
Spastic - damage to the UMNs
Dyskinetic - damage to the basal ganglia
Ataxic - damage to the cerebellum
Monoplegia
One limb affected
Hemiplegia
One side of the body affected
Diplegia
4 limbs are affected but mostly the legs
Quadriplegia
4 limbs affected more severely
Often have seizures, speech disturbance and other impairments
Signs of cerebral palsy
- failure to meet developmental milestones
- prefence of 1 hand before the age of 18 months
- hypertonia or hypotonia
- problems with speech, coordination and walking
- feeding or swallowing problems
- learning difficulties
Cerebral palsy neurological exam
Hemiplagia or diplegia - UMN damage
Broad based gait/ataxic - cerebellar lesion
High stepping gait - LMN damage causing foot drop
Complications associated with cerebral palsy
Learning disability Epilepsy Kyphoscoliosis GORD Muscle contractures Hearing and visual impairments
Management of cerebral palsy
MDT:
- physiotherapy
- dieticians
- occupational health
- speech and language therapy - may need NGT/PEG
- social worker
Medications for cerebral palsy
Glycopyrronium bromide - excessive drooling
Anti - epileptic drugs
Muscle relaxants e.g. baclofen
Strabismus
Misalignment of the eyes causing double vision
Hydrocephalus pathophysiology
Accumulation of CSF within the brain and spinal cord due to eith over production or impaired clearance
Where is CSF created?
Choroid plexus
Where is CSF absorbed?
Arachnoid granulations
Causes of hydrocephalus
Increased production
Decreased absorption:
- aqueductal stenosis
- arachnoid cysts
- coning (arnold - chiari malformation) - cerebellum herniates through the foramen magnum
Presentation of hydrocephalus
Bulging of the anterior fontanelle
Poor feeding and vomiting
Poor tone
Sleep disturbance
Treatment of hydrocephalus
VP shunt
Complications of craniosynostosis
Raised intracranial pressure:
- developmental delay
- cognitive impairment
- vomiting
- visual defects
- seizures
- neurological symptoms
Muscular dystrophy pathophysiology
Gradual weakening and wasting of muscles due to genetical conditions
Gower’s sign
Children with proximal muscle weakness stand up by first getting on their hands and knees, pushing their hips up and backwards then shifting their weight back and putting hands on knees
Indicative of Duchennes muscular dystrophy
Inheritamce pattern on Duchennes muscular dystrophy
X linked recessive
Management of muscular dystrophy
MDT:
- physiotherapy
- occupational therapy
- medical appliance use
- surgical and medical input - spinal scoliosis and HF
Duchennes muscular dystrophy
Defective gene for dystrophin
Present at 3 - 5 yo with proximal muscle weakness of their pelvis - Gower’s sign
Wheelchair bound by teenage years
How to slow down the development of Duchennes dystrophy
Oral steroids
Creatine supplements
When does Beckers muscular dystrophy present
8 - 12 yo
Features of myotonic muscular dystrophy
Normally presents in adulthood with:
- progressive muscle weakness
- prolonged muscle contractions
- cataracts
- cardiac arrhythmia
Facioscapulohumeral muscular dystrophy
Affects facial muscles and then progresses to the shoulders and arms
- sleep with eyes open and weakness in pursing lips and blowing cheeks out
Oculopharyngeal muscular dystrophy presentation
Bilateral ptosis, restricted eye movements and swallowing difficulties
Emery dreifuss muscular dystrophy
Contractures affecting elbows and ankles commonly during childhood
Pathophysiology of spinal muscular atrophy
Autosomal recessive condition causing progressive loss of motor neurones causing progressive muscular weakness
Affects LMNs in the spinal cord
Signs of spinal muscular atrophy
Fasciculations Muscle atrophy Hypotonia Reduced power Reduced or absent reflexes
Types of spinal muscular atrophy
SMA 1 - onset within few months of birth, normally die within 2 years
SMA 2 - onset within first 18 months, cant walk but survive to adulthood
SMA 3 - onset after 12 months old, walk with support but eventually lose ability
SMA 4 - onset in 20 yos - can walk short distances
Management of spinal muscular atrophy
MDT
- physiotherapy
- occupational therapy
- respiratory support
- PEG may be required
