Paed:Neuro Flashcards
What is a seizure?
A sudden disturbance of neurological function caused by an abnormal or excessive. neuronal discharge. Can be epileptic or non-epileptic.
What can cause epilepsy?
idiopathic secondary to cerebral dysgeneis, cerebral vascular occlusion, cerebral damage cerebral tumour neurodegenerative disorder neurocutaneous syndromes
What can cause a non-epileptic seizure?
Febrile seizures Metabolic: hypoglycaemia, hypocalcaemia, hypomagnesia, hypo/ernatraemia Head trauma Meningitis/encephalitis Poisons/toxins
What is a febrile seizure?
Seizure + fever, without intracranial infection due to bacterial meningitis or viral encephalitis.
How does a febrile seizure present?
Brief, generalised tonic-clonic seizures
Occurs in early viral infection when temperature is rising rapidly
Does NOT cause brain damage
*treat underlying infection
Explain paroxysmal disorders/funny turns
Breath-holding episodes: toddler precipitated by anger, holds breath, goes blue then limp and rapid recovery
Reflex anoxic seizures: toddler precipitated by pain, tops breathing, goes pale, brief seizure then rapid recovery
What is epilepsy?
Chronic neurological disorder characterised by recurrent unprovoked seizures consisting of transient signs associated with abnormal, excessive or synchronous neuronal activity in the brain.
What is a generalised seizure?
Discharge from both hemispheres (absence, myoclonic, tonic, tonic-clonic or atonic)
Focal
Seizures arise from one (or part of one) hemisphere
Clinical presentation of frontal focal seizure?
Motor cortex
Clonic movements including Jacksonian march
Asymmetrical tonic seizures
Atonic seizures from messiah frontal discharge
Clinical presentation of temporal focal seizure?
Most common
Auditory or sensory phenomena
Lip smakcing
De ja vu
Clinical presentation of occipital focal seizure?
Positive/negative visual phenomena
Clinical presentation of parietal focal seizure?
Contralateral altered sensation sensation (dysaesthesia)
Describe a generalised seizure
always LOC
no warning
symmetrical seizure
bilaterally synchronous seizure discharge on leg or varying asymmetry
Describe an absence seizure
Transient LOC with abrupt onset and termination, unaccompanied by motor phenomena except for some flickering of the eyelids and minor alteration in muscle tone.
Absences may be typical (petit Mal) or atypical
Often precipitated by hyperventilation
Describe a myoclonic seizure
Brief, often repetitive, jerking movements of the limbs, neck or trunk
Non-epileptic myoclonic movements are also seen physiologically in hiccoughs (myoclonus of the diaphragm( or on passing through stage II sleep (sleep myoclonus)
Describe a tonic seizure
Generalised increase in tone
Describe a tonic-clonic seziure
Rhythmical contraction of muscle groups following the tonic phase
Rigid tonic phase: children may fall to ground, stop breathing and become cyanose.
Followed by the clonic phase, with jerking of the limbs.
Irregular breathing, cyanosis persists + saliva may accumulate in the mouth.
May be biting of tongue and incontience of urine
Lasts seconds to minutes followed by unconsciouness or deep sleep for several hours
Describe an atonic seizure
Often combined with a myoclonic jerk, followed by a transient loss of muscle tone causing a sudden fall to the floor or drop of the head.
How is epilepsy diagnosed?
- Detailed history including eyewitness account
- EEG: 3Hz spike
- MRI + CT structural brain scan (identify tumour, vascular lesion and sclerosis)
- Functional scans: PET, SPECT to find areas of hyper metabolism in epileptogenic lesions.
- Metabolic Ix: na+ and glucose
How is epilepsy managed?
Buccal benzos?
Generalised seizures: valproate + lamotrigene
Focal seizures: carbamazepine, lamotrigene
Last resort: phenytoin
What is West syndrome?
4-6months Violent flexor spasms of head, trunk and limbs followed by extension of arms Often occurs on walking many times a day EEG: hypsarrhtmia Tx: vigabatrin or corticosteroids Often develop LD or epilepsy
What is Lennox-gastaut syndrome?
1-3 years
multiple seizure types but mostly drop attacks (Astatic seizures), tonic seizures and atypical absences
- complex neurological problems
Explain childhood absence epilepsy?
4-12 years
Stare momentarily and stop moving only for a few seconds
Child has no recall, can interfere with schooling.
Induced by hyperventilation (child asked to blow on windmill for 2-3mins in OP clinic)
EEG: 3Hz spike, bilaterally synchronous
What is benign epilepsy with centrotemporal spike? (BECTS)
4-10 years
Tonic-clonic seizures in sleep, or simple focal seizures with awareness of abnormal feelings in tongue and distortion of face
EEG: focal sharp wave
Doesn’t always need treatment
What is early-onset benign childhood occipital epilepsy? (panayiotopoulos type)
1-14years
Younger children: periods of unresponsiveness, eye deviation, vomiting and atonic features
Older children: headache and visual disturbance including disortion of images and hallucinations
EEG: occipital discharges
Remit in childhood
What is juvenile myoclonic epilepsy?
Adolesecne - adulthood
Myoclonic seizures, but generalised tonic-clinic seizures and absences ma occur usually shortly after waking.
Typical Hx: throwing drinks or cornflakes about in morning as myoclonus occurs at this time. Learning is unimpaired.
Genetic link identified. Remission unlikely.
What is cerebral palsy?
Abnormality of movement and posture causing activity limitation attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.
Causes of CP?
80% antenatal: Vascular occlusion, cortical migration disorders, structural maldevelopment of the brain during gestation. Other antenatal: genetic syndromes, congenital infection
10% due to hypoxic-ischaemic injury during delivery
10% are postnatal orgin: meningitis, encephalitis, enchephalopathy, head trauma, symptomatic hypoglycaemia, hydrocephalus and hyperbilirubinaemia.
What can cause brain damage in pre term infants?
Periventricular leukomalacia secondary to ischaemia and/or severe intraventricular haemorrhage
Clinical presentation of CP?
Early features:
- Abnormal limb and or trunk posture and tone in infancy with delayed motor milestones, accompanied by slowing of head growth
- Difficulty feeding, oromotor incoordination, slow feeding, gagging, vomiting
- abnormal gait when walking
- asymmetric hand function before 12months
- persistent primitive reflexes
What are the clinical subtypes of CP?
1) Spastic CP 90%
1a) Hemiplegia
1b) Quadriplegia
1c) Diplegia
2) Dyskinetic CP 6%
3) Ataxic/hypotonic 4%
What is spastic cp?
Limb tone is persistently increased (spasticity) w/ associated brisk deep reflexes and extensor plantar reflexes. Clasp knife reflexes and initial hypotonia, especially of head and trunk.
What is hemiplegic spastic CP?
Unilateral invovlement of arm and leg, leg more than arm, face spared. happens 4-12months Fisting of affected harm flexed arm pronated form asymmetric reaching or hand function tiptoe walking (toe-heel gait) PMH normal
What is quadraplegic spastic CP?
All four limbs severely affected
Trunk has tendendcy to opisthonus (extensor posturing)
poor head control
low central control
ass w/ seizures, microcephaly, intellectual impairment
What is diplegic spastic CP?
All four limbs but legs much more than arms
Motor difficulties in arms apparent with functional use of hands
abnormal walking
ass w/ preterm birth due to periventricular brain damage
What is dyskinetic CP?
- Movements which are invvoluntary, uncontrolled and more evident with active movement and stress
- variable muscle tone
- dominate primitive reflex motor pattern
- Get chorea, athetosis and dystonia.
- Floppy, poor trunk control, delayed motor development in infancy due to damage in basal ganglia or extrapyramidal pathway
What are the causes of dyskinetic CP?
Kernicterus or hypoxic-ischaemic encephalopathy at term
What is chorea?
-Irregular sudden brief non repetitive movements
What is athetosis?
- Slow writhing movements occuring more distally eg. fanning fingers
W
What is dystonia?
Simultaneous contraction of agonist and antagnoist muscles of trunk and proximal muscles giving twisting appearance
What is ataxic cp?
- genetically determined
- signs on same side as lesion, usually symmetrical
- early trunk and limb hypotonia
- poor balance
- delayed motor development
- later: incoordinate movements, intention tremor, ataxic gait
3 core symptoms of ADHD?
1) Inattention
2) Hyperactivity
3) Impulsivity
What are the symptoms of inattention?
- Easily distracted
- not listening when spoken to directly
- difficulty sustaining attention during activities
- avoids/dislikes tasks requiring sustained mental effort
- forgetful in daily activities
- difficult to follow instructions and fails to complete tasls
- difficult to organise tasks and activities
- fails to give close attention to detail / makes careless mistakes
- loses important items
What are the symptoms of hyperactivity?
- Squirms and fidgets
- Cannot remains eated
- Runs/climbs excessively in inappropriate situations
- often on the go/ acts as if driven by motor
- talks excessively
- cannot perform leisure activities quietly
What are the symptoms of impulsivity?
- Blurts answers before questions completed
- Difficulty awaiting turn
- Intempts or intrudes on others
What is the assessment of ADHD?
Clinical interview: - Parents/caregivers - reason for referral - history inc. pregnancy and birth hx (alcohol, nicotine, prematurity) Behavioural observation: -varying contexts - ADHD worse when: Repetitive activiety, unstructured situ, boring situ, distractions, minimal superviion Info from 3rd parties - Interviews with nurses/teachers -behaviour reports -doctors notes
Proposed causes of adhd?
- Neurobiologic (neuroanatomical and neurcochemical)
- Genetic influences
- Environemntal/psychosical factors
- CNS insults (perinatal factors, cns infection)
- Acquired brain injury - prematurity, fetal alcohol syndrome, NF
- Maternal smoking
Treatment of ADHD?
- Education
- ADHD parenting program
- School support and liasion
- Medication
Non stimulant ADHD medications?
- Atomoxetine
- Lisdexamphetamine
What are some stimulant ADHD meds?
Short: Ritalin (methylphenidate)
Long: Concerta xl or equasym xl
What are the main symptoms of ASD?
1) Communication diffuclties
2) Social interaction
3) Difficulties with imagination/rigidity of thought
Describe communication difficulties in ASD?
- Lack of desire to communicate at all
- Communicating needs only
- Disordered/delayed language
- Repeats speech (echolalia)
- Poor non verbal commiunication, gesture and body language
- Good language but then no social awareness / cant start conversations
- Pedantic language (very literal), poor understanding of idioms and jokes
Describe social interaction in ASD?
- No desire to interact with others
- Being interested in others to have needs met
- Lack of motivation to please others
- Affectionate on own terms
- Friendly but with odd interactions
- No understanding of unspoken social rules
- Limited interaction with unfamiliar people in unfamiliar circumstances
Describe imagination/rigidity of thought in ASD?
- Using toys as object
- Inability to play or write imaginatively
- Resisting change
- Playing same game repeatedly
- Obsession/rituals
- Follows rules exactly
- Learning by rote but with no understanding
- Inability to see others points of view
- Asks the same question even when answered
Causes and risk factors of anorexia nervosa?
- Perfectionist character traits
- Depression can trigger binges
- Genetic predispostion
- Occupation and interest eg, ballet
- Family (attention seeking or attitude to food in family)
- Low self esteem
- Social pressure
- Anxiety disorder
- Reversing or halting effects of puberty
- Past or present events (life difficulties, sexual abuse, physical illness, upsetting events, important events)
How do you screen for eating problems?
S - Do you make yourself Sick because youre uncomfortably full?
C - Do you worry youve lost Control over how much you eat?
O - Have you recently lost about 1 stone (6kg) in 3 months
F - Do you believe you are Fat when others say youre thin?
F - Would you say that Food dominates your life?
Criteria for anorexia nervosa?
- Dietary restriction leading to significant and unhealthy self induced weight loss (less than 85% expected weight for height/age OR BMI <17.5)
- Intense fear of gaining weight
- Body image distortion with dread of fatness
- Amenorrhoea - primary or secondary
Clinical signs of Anorexia Nervosa?
- Weak proximal muscles (squat test)
- Dry skin
- Lanugo hair
- Orange skin/palms
- Cold hands/feet
- Bradycardia
- Drop in BP on standing
- Oedema
When would anorexia be treated as inpatient?
- If BMI <13.5
- Severe suicidal risk and/or severe depression
- Rapid weight loss
- Failed outpatient care (non-compliance)
- Refusal to eat and drink
- Electrolyte imbalance and ECG changes (low potassium below 3.5mmol/l)
- Crisis situation
- Dehydration
Whatt are some psychological treatments of AN?
- Cognitive analytic therapy
- CBT
- Interpersonal psychotherapy
- Focal psychodynamic therapy
- Family therapy
What medications can be given for AN?
- Multivitamins, thaimine and phopshate
- Antidepressants
- Olanzapine
- Quetiapine
What are the weight restoration goals for AN?
Outpatients: 0.5kg/week
Inpatients: 0.5-1kg/week
What factors give lower AN mortality?
- Early age of onset
- Short duration between sx and tx start
What factors give higher AN mortality?
- Lower weight at presentation
- PResenting at 20-29 years
- alcohol misuse
- repeated admissions for anorexia
- admissions for other psych disorders
Poorer AN outcome if?
- Late onset
- Excessive weight loss
- Vomiting/purging
- Poor social adjustment
- Poor parental relationships
- Being male
- Chronic course of illness
What is the moro reflex?
Sudden extension of the head causes symettrical extension then flexion of arms
What is the grasp reflex?
Flexion of the fingers when an object is placed in the palm
What is the rooting reflex?
Head turns to stimulus when touched near the mouth
What is the stepping reflex?
Stepping response/movements when held vertically and dorsum of feet touch a surface
What is the aysymmetric tonic neck reflex?
Lying supine the infant adopts a outsretched arm to the side when the head is turned
When would you expect the primitive reflexes to go?
Diminish over 4-6 months from term (Moro, grasp, galant)
When would you expect the postural reflexes to emerge>
3-8 months. Positve support reflex, landau, lateral propping and parachute
What is the issue if primitive reflexes persist?
Persistence of primitive reflexes and lack of development of postural reflexes are a hallmark of upper motor neurone abnormality in the infant
Why does CP seem like it gets worse?
It is a static lesion, although as the child ages the deficit becomes more apparent as it fails to reach milestones with peers.
Causes of childhood developmental delay?
Genetics: Chromosomal disorder eg trisomy 21, microdeletion, microduplication, single gene disroders eg. duchennes and retts, polygene eg. autism, adhd
Factors in pregnancy: congenital infection eg. CMV/HIV, exposure to drugs and alcohol, MCA infarct - CP
Factors around birth: Prematurity and birth asphyxia,
Factors in childhood: infections eg meningits, encephalitis, chronic ill health, metabolic condtions, acquired brain injury, hearing impairment, vision impairment
Environmental factors: abuse and neglect, low stimulation
At 6 weeks what would you expect?
Gross motor: Good head control
Fine motor/vision: Tracks objects/face
Speech and language: Stills, startles at loud sounds
Social: Social smile
At 6 months what would you expect?
Gross motor: Sits without support, rolls tummy to back
Fine motor: Palmar grasp, transfer hand to hand
Speech and language: Turns head to loud sounds, understands bye bye and no. babbles.
Social: Puts object to mouth, shakes rattle, reaches for bottle/breast
At 9 months what would you expect?
Gross motor: Stands holding on, straight back sitting
Fine motor: Inferior pincer grip, object permanence
Speech and language: Responds to own name, imitates adult sounds
Social: Stranger fear, holds and bites food
At 12 months what would you expect?
Gross motor: Walks alone
Fine motor: Neat pincer grip, casting bricks, can build two bricks
Speech and language: Shows understanding of nouns eg. Wheres mummy?. Can say three words. points to own body parts or on doll.
Social: Waves bye bye, hand clapping, plays alone if familar person nearby, drinks from beaker with lid
At 18months what would you expect?
Gross motor: Runs and jumps
Fine motor: Draws to and fro. build four bricks
Speech and language: Shows understanding of nouns eg. show me the door. 1-6 diff words
Social: Imitates everyday activities
At 2 years what would you expect?
Gross motor: Run on tiptoes, walk upstairs (both feet on each stair), throws ball at shoudler level
Fine motor: can draw a vertical line, build 8 bricks, turn several book pages at once, match shape puzzle
Speech and language: Shows understanding of verbs eg. what do you eat with/draw with, join two words together, 50 worss
Social: Eats skilfully with a spoon
At 2.5years what would you expect?
Gross motor: Kicks ball
Fine motor: Draw horizontal line
Speech and language: Understanding of prepositions eg. put cat on the bowl. Join 3-4 words together
Social: Eats skilfully with a spoon
at 3 years what expect?
Gross motor: Hop on one foot for 3 steps on each foot. Walk upstairs one foot per step. Downstairs two feet at a time.
Fine motor: Draw a circle. make a bridge with bricks, single cut with scissors. turn one page at a time.
Speech and language: Understand negatives, eg. which is not an animal? Understand adjectives eg. which is red?
Social: begins to share toys with friends, plays alone without parents, eats with fork and spoon, bowel control
3.5 years what expect?
Gross motor:
Fine motor: Cut pieces
Speech and language: Understand comparatives eg. which boy is bigger than middle?
Social:
What at 4?
Gross motor: FINALLY can walk on stairs like a grown up
Fine motor: Draw a cross(4), square (4.5), triangle/person. build 12 blocks and steps. cut paper in half.
Speech and language: understand complex instructions. eg. before putting x in y , give z to mummy. use complex narrative to describe events
Social: concern/sympathy for others if hurt. has a best friend. bladder control at 4.5. engages in imaginative plat. observing rules. eat skilfully with little help. handles knife. dressing and undressing.
When does not meeting a milestone become concerning?
Gross motor:
- Not sitting by one year
- Not walking by 18 months (?DMD/hip problems/CP?)
Fine motor:
- hand preference before 18months
Speech and language:
- Not smiling by 3 months
- no clear words by 18months
Social:
- no social smile by 6 weeks ?visual problem
- No resposne to carers interations by 8 weeks
- not intersted in playing w/ peers by 3 years
What are some red flags in development?
- Regression
- Poor health/growth
- Significant FH
- Findings on exam: Microcephaly and dismoprhic features
- Safeguarding
What are the different types of child abuse?
Physical injury, sexual abuse, emotional abuse and neglect
What does neglect mean?
Standard of care that does not meet the needs for the child.
What are some parental predisposing factors for child abuse?
- lack of support
- generational effect (their experiences of parenting)
- deprivation
- mental health problems
- LD
- alcohol and substance misuse
- domestic violence
What are some child predisposing factors for child abuse?
- ‘difficult child’
- preterm
- disabled
How can child abuse present?
- disclosure (someone declares it)
- injury observed (e.g. at school or GP)
- found incidentally when attending for another reason (parents allegding that injuries have just appeared)
- injury presented with or without explanation
What makes you concerned from a history investigating child abuse?
Hx doesn’t fit with observed injuries
- too many injuries
- wrong site (bony prominence + outer external surfaces)
- unusual shape or pattern
- wrong type or pattern
- wrong type of incident
- not consistent with child’s development
- delay in presentation (why didn’t they bring them for healthcare originally - covering up?)
No Hx
A Hx that changes
How might physical abuse present?
Superficial: bruises + scratches
Marks from implements
Burns + scalds
Fractures (multiple, long bones, ribs, metaphyseal)
Internal organ damage: abdo, intracraniall
How might a shaken baby present?
Shaking + impact Hypoxia Subdural haematoma Rib fractures or other fractures (torn frenulum) Retinal haemorrhages
What can be in the DD to abuse?
Birthmarks, Mongolian blue spots, paint, bleeding tendency, brittle bones (osteogenic imperfecta) + normal accidents
What are Mongolian blue spots?
Looks like a bruise, often on buttocks, sacral + spinal area
How can a child be neglected?
Food + drink Warmth, shelter, clothing Grooming Health + dental care Education, appropriate social opportunities Emotional support Protection from harm Discipline
Explain emotional abuse
Common, often unrecognised
Coexists with other abuse types
Damaging
Relationship high in criticism, low in warmth
Explain sexual abuse
Can affect boys + girls, any age
Can be family or stranger abuse
Wide spectrum of activities
Linked to other abuse types
How can sexual abuse present?
Disclosure
Behaviour change: sexualised, indicative of distress
Physical Sx: bleeding, discharge, soreness, wetting/soiling, STI, pregnancy
How is sexual abuse investigated?
Medical assessment
Lab tests: FBC, clotting for bruising/bleeding
Swabs (e.g. ?impetigo)
Bone profile: vit D and PTH if fractures
Radiology: skeletal survey, CT, MRI
Developmental assessment (inappropriately stimulated to learn)
What agencies are involved with abuse?
Social services assessment
+/- police investigation
Case conference: multiagency, parents invited, decide placement on formal register, child protection plan led by key worker
Review process
