Paeds- Neuro and Psych Flashcards
what is cerebral palsy?
chronic disorder of movement and posture causing activity limitation attributed to non-progressive disturbances that occurred in the developing foetal brain
antenatal causes of cerebral palsy
- vascular occlusion
- cerebral malformation
- congenital infection (rubella, toxoplasmosis)
postnatal causes of cerebral palsy
meningitis, encephalitis, encephalopathy
head trauma
intraventricular haemorrhage
clinical presentation of cerebral palsy
- abnormal limb/ trunk tone and posture
- delayed motor milestones
- abnormal gait when walking
- feeding difficulties
- learning difficulties
- speech/ language difficulties
describe the Gross Motor Function Classification (GMFCS)
1- walks without limitations
2- walks with limitations
3- walks using handheld mobility device
4- self mobility with limitations- may use powered mobility
5- manual wheelchair transportation
how is cerebral palsy diagnosed ?
clinical examination
what are the 4 clinical subtypes of cerebral palsy?
- mixed pattern
- spastic (90%)
- dyskinetic (6%)
- ataxic (4%)
describe spastic cerebral palsy
- damage to UMN (corticospinal/ pyramidal)
- hypertonia
- brisk deep tendon reflexes
- clasp knife reflexes
- spastic hemiplegia
- rare- quadriplegia
describe dyskinetic cerebral palsy
- chorea, athetosis and dystonia
- basal ganglia damage
- primitive motor reflex patterns
- intellect unimpaired
what is athetosis and when is it commonly seen?
slow writhing movements distally (e.g fanning fingers)
seen in dyskinetic cerebral palsy
describe ataxic cerebral palsy
- cerebellum damage- result in difficulty with coordinated movement
- poor balance, delayed motor development, trunk and limb hypertonia
- intention tremor
what can be given to manage spasticity in a patient with cerebral palsy?
- oral diazepam
- baclofen
- botulinum toxin
what is autism?
A complex developmental condition that includes a range of possible
developmental impairments in reciprocal social interaction and communication as well as a stereotyped, repetitive or limited behavioural repertoire
what is autism defined by?
- presence of abnormal/ impaired development presenting before the age of 3
- abnormal functioning in all 3 areas of psychopathology- social interaction, impairment of language, restricted and repetitive behaviour
how does autism present clinically?
- communication difficulties- lack of desire to communicate, disordered, repetition, poor non-verbal communication
- no social awareness
- poor social interactions
- lack of empathy
- rigidity of thought
- obsessions
what medical problems are associated with autism?
- epilepsy
- visual and hearing impairment
- mental health- depression, anxiety, OCD, ADHD
- sleep disorders
how is autism managed? (non- pharmalogical)
- behavioural intervention
- speech therapy
- school liaison
- aids- timetables, written instructions etc
how is autism managed pharmacologically?
risipiridone- agression
melatonin- sleep
SSRI’s= repetitive behaviour
what features must be present to diagnose ADHD?
- 6/9 inattentive symptoms
- 6/9 hyperactive symptoms
also must:
- present before 12
- developmentally inappropriate
- several symptoms in 2 or more settings
- clear evidence that symptoms interfere with functioning (e.g. academic ability)
aetiology of ADHD
- CNS insults
- genetic
- acquired brain injury- prematurity, fetal alcohol syndrome
what are the 3 core symptoms of ADHD?
- inattention
- hyperactivity
- impulsivity
describe the inattentive symptoms of ADHD
- easily distracted
- does not listen
- forgetful
- difficult to follow instructions
- difficulty organising tasks
describe the hyperactive symptoms of ADHD
- squirms and fidgets
- cannot remain seated
- runs/ climbs all the time
- always ‘on the go’
- talks excessively
describe the impulsive symptoms of ADHD
- blurts out answers before question has been completed
- difficulty awaiting turn
- interrupts or intrudes others
describe the clinical picture of ADHD in primary school children (6-12)
- distractibility
- motor restlessness
- impulsive and disruptive
- learning disorders
- agressive
- low self esteem
- rejection by peers
describe the clinical picture of ADHD in adolescents (13-17)
- difficulty in planning and organisation
- persistent inattention
- associated- aggressive, antisocial, alcohol and drug problems, emotional problems, sexually inappropriate
describe the clinical picture of ADHD in adults
- mental disorders- autism, antisocial
- lack of achievement
- crime
- drugs/ alcohol
- impulsive spending
how is ADHD managed non-pharmaceutically?
education
school support
CBT
how is ADHD managed pharmaceutically?
methlyphenidate (Ritalin)- first line
Atomoxetine
Lisdexamfetamine- only used when unresponsive to max dose of the other 2
what is a seizure?
transient occurence of signs and symptoms due to abnormal excessive neuronal activity in the brain
what is syncope?
sudden reduction in cerebral perfusion with oxygenated blood either secondary to reduced cerebral blood flow or reduced O2 content
what can cause paroxysmal non-epileptic events (go through each system)
cardiac- arrhythmia, syncope
GI- GORD
Neuro- migraine, Tic, sleep related
Psych- pseudo-seizure
Behavioural- day dreaming
features of a simple febrile seizure
less than 15 mins
generalised seizure
no recurrence within 24h
complete recovery in 1h
features of a complex febrile seizure
15-30 mins
focal seizure
repeat seizures within 24h
how does does febrile status epilepticus last?
over 30 mins
define febrile seizures
Seizures occurring in children aged 6 months to 6 years, associated with fever and raised temperature (>37.8 axilla temp), without other underlying causes such as CNS infection or electrolyte imbalance
what is a simple febrile seizure?
generalised, tonic-clonic seizures lasting less than 15 minutes that do not recur within 24 hours or within the same febrile illness
patient is drowsy for > 1 hour after seizure
what is a complex febrile seizure?
one or more of:
- focal features at onset/ during
- duration of more than 15 mins
- recurrence within same illness
what is febrile status epilepticus?
febrile seizure lasting for longer than 30 mins
if a patient presented with focal CNS signs and a history of epilepsy with a seizure lasting longer than 15 minutes what would you be concerned about?
meningo-encephalitis
CNS lesion
epilepsy
trauma
metabolic (low glucose, calcium or magnesium)
when should a child presenting with a febrile seizure be urgently referred?
- first febrile seizure
- lasting over 5 mins
- drowsy for 1 hour after
- previous history of febrile seizure
- age under 18 months
- complex seizure
- recent antibiotic use
how is a patient presenting with a febrile seizure investigated?
- developmental screen
- cause of fever? infective
- LP- if under 18 months
what are some contra-indications for performing a LP in a paeds patient?
- reducing consciousness (GCS<13)
- septic shock
- suspected meningococcal disease
- signs of raised ICP
- focal neurology
- bleeding tendency
how would you treat a febrile seizure lasting over 5 minutes?
diazepam, midazoloam, lorazepam
what is reflex anoxic seizure?
paroxysmal, self limited brief (15 seconds) asytole
occurs in infants and toddlers (6 months- 2 years)
what can trigger a reflex anoxic seizure?
- pain/ discomfort
- cold foods
- fright
- fever
how does a reflex anoxic seizure present clinically?
- stops breathing following trigger
- deathly pale
- falls to floor
- clinic movements
- urinary incontinence
- brief- recovers quickly
explain the pathophysiology of a reflex anoxic seizure
- cardiac asytole from vagal inhibition
- slow wave discharge seen on ECG
how is a reflex anoxic seizure diagnosed?
vagal excitation tests w/ continuous EEG + ECG
how is a reflex anoxic seizure differentiated from epilepsy?
NO TONGUE BITING
how is a patient presenting with a reflex anoxic seizure managed?
- check ferritin
- no drugs needed
- pacemaker
usually self limiting ! no medical treatment needed
describe breath holding episodes
- occur when toddler is upset
- child cries, holds breath and goes blue
- can lose consciousness but recovers rapidly
- resolves spontaneously
define childhood epilepsy
chronic, neurological disorder characterised by recurrent unprovoked seizures, consisting of transient signs and/or symptoms associated with excessive neuronal activity in the brain
which types of childhood epilepsy are classified as generalised?
- absent
- myoclonic
- tonic
- tonic-clonic
- atonic
describe absent childhood epilepsy
- transient LoC (less than 30s)
- abrupt onset + termination
- no motor phenomena
- eyelid flickering
- stare momentarily and stop moving
- will only recall that they missed something- ‘puzzled’
describe the aetiology of absent childhood epilepsy
2/3rd female
4-12 years old
describe myoclonic childhood epliepsy
brief, often repetitive, jerking movements
what is a tonic-clonic seizure?
- tonic- rigid, falls to ground, cyanotic
- clonic- contractions, jerking, irregular breathing, tongue biting, incontinence
episode followed by unconsciousness/ deep sleep for several hours
describe an atonic seizure
transient loss of muscle tone causing drop to the floor/ drop of head
what types of seizure are described as focal/ partial?
frontal
temporal
occipital
parietal
describe a frontal seizure
motor phenomena- clonic movements
asymmetrical
describe a temporal seizure
- auditory/ smell/ taste
- lip smacking, plucking at clothes
- Deja vu and Jamais vu
- longer
describe an occipital seizure
distortion of vision
describe a parietal seizure
contralateral altered sensation
how are generalised seizures managed?
1st- valproate/ carbamazepine
2nd- lamotrigine
how are focal seizures managed?
1st- carbamazepine/ valporate/ lamotrigine
2nd- topiramate, gabapentin, tigabine, bigatrin
what can be given to a patient with prolonged seizures?
rectal diazepam
when is therapy for paediatric patients with epilepsy discontinued?
after 2 years without a seizure
how is status epilepticus managed?
- supportive ! ABC etc
drugs:
- ABC
- IV lorazepam after 5 mins
- repeat lorazepam after 15 mins
- IV Phenytoin- 20 mins
if not stable after this refer to PICU
what are the 3 components of West syndrome?
infantile spasms
hypsarrythmia- EEG
general learning disability
aetiology of West Syndrome
- prenatal conditions
- hypoxia, ischaemia, meningitis, encephalitis, trauma, intracranial haemorhage
- idioathpic
describe the epidemiology of West syndrome
4-7 months
confined to infants
describe the spasms seen in West Syndrome
- clusters of head nodding and arm jerks
- sudden, rapid, tonic contraction of trunk and limb muscles
- contraction relaxation up to 2s
- occur just before sleep or on waking up
other than spasms, describe the other features of West Syndrome
- learning disabilities
- hypo-pigmented skin lesions
- mild/moderate growth restriction
how is West syndrome diagnosed?
EEG- hypsarrythmia
describe the treatment pathway of West Syndrome
- vigabatrin
- ACTH- adrenocrticotropic hormone
- prednisolone
however it has a poor prognosis