Neurology Flashcards
What is cerebral palsy
a disorder of movement and posture due to a non-progressive lesion of the motor pathways in the developing brain
Give 3 antenatal factors that can cause cerebral palsy
- maternal illness - infections (CMV, rubella), thyroid disease etc
- prematurity
- cerebral malformation
Give a intrapartum factor that can cause cerebral palsy
- Birth asphyxia - hypoxic ischaemic injury
Give 4 postnatal factors that can cause cerebral palsy
- meningitis/ encephalitis
- hyperbilirubinemia
- intraventricular haemorrhage
- head injuries prior to age 2 (inc NAIs)
What are the 3 main types of cerebral palsy. Put in order of most to least common
- Spastic >80%
- Dyskinetic
- Ataxic
What is spastic cerebral palsy
increased tone resulting from damage to upper motor neurons
What are the 4 types of spastic cerebral palsy
- monoplegia - single limb involvement
- hemiplegia (unilateral) - ipsilateral involvement of arm and leg (arm>leg)
- diplegia - all 4 limbs but the legs affected much more than arms
- quadriplegia - all 4 limbs and the trunk (more severe)
What 3 signs are often associated with quadriplegia
- Seizures
- microcephaly
- moderate or severe intellectual impairment
What is dyskinetic cerebral palsy
- Involuntary, recurring and occasionally stereotyped movements with a varying muscle tone
- caused by damage to the basal ganglia and substantia nigra
Describe the 3 subgroups of dyskinetic cerebral palsy
- Dystonia - involuntary, sustained contractions resulting in twisting and abnormal postures
- chorea - rapid, involuntary, jerky and non-repetitive movements
- athetosis - slow writhing movements occurring more distally
What is ataxic cerebral palsy
- problems with coordinated movement and hypotonia
- most are genetically determined but can be due to injury to the cerebellum or its connections
Give 6 early features of cerebral palsy
- abnormal limb and/or trunk posture and tone
- delayed motor milestones
- feeding difficulties
- abnormal gait
- asymmetric hand function before 12 months
- retention of primitive reflexes - moro reflex
Give 3 clinical features of bilateral spastic cerebral palsy
- predominately affects legs
- young child - toe walking, scissoring
- older child - crouch gait pattern when the child gets heavier and can’t remain on their toes
How is cerebral palsy investigated
- MRI brain
- clinical exam - assess posture, tone and gait
How is cerebral palsy managed
- MDT: OT, physio, SALTs
- Hypertonia - botulinum toxin A injections
- oral/ Intrathecal baclofen (skeletal muscle relaxant)
- selective dorsal rhizotomy (proportion of the nerve roots are cut to reduce spasticity
- oral diazepam for spasticity
Give 5 complication of cerebral palsy
- learning disability
- hearing/ visual impairment
- behavioural problems
- drooling
- epilepsy
What are febrile convulsions
seizures provoked by fever in otherwise normal children
What is the typical age range for the occurrence of febrile convulsions?
typically occur between the ages of 6 months and 5 years
Describe simple febrile convulsions
- < 15 minutes
- generalised tonic clinic
- typically no recurrence within 24 hours
- Should be complete recovery within an hour`
Describe complex febrile convulsions
- last 15-30 minutes
- focal seizure
- may occur multiple times within 24 hours
When is a febrile convulsion considered febrile status epilepticus
when it lasts > 30 minutes
How are febrile convulsions managed
- first seizure/ complex seizure - admit to paeds
- parental advice: phone for ambulance if seizure lasts over 5 mins
- antipyretics do not prevent febrile convulsions
- recurrent febrile convulsions - specialist may consider prescribing rescue rectal diazepam or buccal midazolam
What are infantile spasms (west’s syndrome)
brief spasms beginning in the first few months of life
Describe the presentation of infantile spasms
- flexion of head, trunk, limbs → extension of arms
- last 1-2 secs, repeat up to 50 times
What causes infantile spasms
usually secondary to neurological abnormality
* encephalitis
* tuberous sclerosis
* birth asphyxia
How are infantile spasms managed
- combo therapy: vigabatrin and high dose prednisolone
- if <2y seek guidance from and refer urgently to tertiary paeds neurologist
- carries a poor prognosis
describe the presentation of typical absence seizures
- onset 4-8y
- child becomes blank and stares into space
- duration few-30 secs; no warning, quick recovery; often many per day
How are absence seizures managed
- first line: ethosuximide
- second line: male - sodium valproate, females - lamotrigine or levetiracetam
- most are seizure free by adolescence
Which antiepileptic may exacerbate absence seizures
carbamazepine
Describe a generalised tonic-clonic seizure
- loss of consciousness
- tonic (muscle tensing) and clonic (muscle jerking) movements
- may be associated tongue biting, incontinence, groaning and irregular breathing
- prolonged post-ictal period: confused, drowsy, irritable
How are tonic-clonic seizures managed
- boys: sodium valproate
- girls: lamotrigine or levetiracetam
- girls aged under 10 years and who are unlikely to need treatment when they are old enough to have children may be offered sodium valproate first-line
How may a focal seizure present
- Hallucinations
- Memory flashbacks
- Déjà vu
- Doing strange things on autopilot
- level of awareness varies
How are focal seizures managed
- First line: lamotrigine or levetiracetam
- Second line: carbamazepine, oxcarbazepine or zonisamide
Describe the presentation of Lennox-Gastaut syndrome
- atypical absences, falls, jerks
- 90% have cognitive dysfunction
What would indicate Lennox-Gastaut syndrome on electroencephalogram
slow spike waves
How is Lennox-Gastaut syndrome managed
- specialist paediatric neurologist involvement
- first line: sodium valproate
- second line: lamotrigine
- ketogenic diet if seizures continue
Describe the epidemiology of juvenile myoclonic epilepsy
- typical onset is in the teenage years
- more common in girls
Describe the features of juvenile myoclonic epilepsy
- infrequent generalized seizures, often in morning/ following sleep deprivation
- daytime absences
- sudden, shock-like myoclonic seizure (these may develop before seizures)
How is juvenile myoclonic epilepsy managed
- first line: sodium valproate
- first line (girls): levetiracetam
Which organisms cause neonatal to 3-month-old meningitis?
- Group B Streptococcus
- E. coli and other Gram-negative organisms
- Listeria monocytogenes
Which organisms are commonly associated with meningitis in children < 6 years?
- Neisseria meningitidis (meningococcus)
- Streptococcus pneumoniae (pneumococcus)
- Haemophilus influenzae
Which organisms commonly cause meningitis in children older than 6
Neisseria meningitidis (meningococcus)
Streptococcus pneumoniae (pneumococcus)
What are the contraindications for performing a lumbar puncture in suspected meningitis cases in children?
Focal neurological signs
Papilloedema
Significant bulging of the fontanelle
Disseminated intravascular coagulation
Signs of cerebral herniation
What should be done instead of a lumbar puncture in patients with meningococcal septicaemia?
- Blood cultures and PCR
- LP is contraindicated in meningococcal septicaemia.
What antibiotics are used for the management of meningitis in children under 3 months?
IV amoxicillin (or ampicillin) + IV cefotaxime
What antibiotics are used for the management of meningitis in children over 3 months?
IV cefotaxime (or ceftriaxone)
When are corticosteroids recommended in the management of meningitis in children?
- Not recommended in children younger than 3 months
Dexamethasone should be considered if the lumbar puncture reveals: - Frankly purulent CSF
- CSF white blood cell count >1000/microlitre
- protein concentration >1 g/litre
- Bacteria on Gram stain
How should shock be managed in children with meningitis?
Shock should be treated with fluids, such as colloids.
What public health measures should be taken in cases of meningococcal meningitis?
- Public health notification
- Antibiotic prophylaxis for contacts (ciprofloxacin preferred over rifampicin)
At what ages are the three doses of the Meningitis B vaccine given?
- 2 months
- 4 months
- 12-13 months
