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Child Health > Neurology > Flashcards

Neurology Flashcards

1
Q

What is cerebral palsy defined as?

What is its incidence?

A

= movement/postural abnormalities leading to activity limitation due to disturbances during fetal brain development
2 in 1000 live births

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2
Q

What are some other associated problems with cerebral palsy? (9)

A 
Learning difficulties (60%)
Epilepsy (40%)
Squint (30%)
Vision problems (20%)
Hearing problems (20%)

Speech + lang disorders
Behavioural probs
Joint contractures/subluxations/scoliosis
Feeding probs

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3
Q

List the possible causes of cerebral palsy (12)

A 
Antenatal causes (80%):
Vascular occlusion
Congenital infection
Genetic syndromes
Cortical migration disorders
Structural maldevelopment

Hypoxic Ischaemic injury during delivery (10%)

Post-natal causes (10%):
Head trauma
Meningitis/encephalitis/encephalopathy
Periventricular leukomalacia (after ischaem/h'age - preterm)
Hydrocephalus
Hyperbilirubinaemia
Symptomatic hypoglycaemia
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4
Q

What are some of the early features of cerebral palsy? (7)

A 
Delayed motor milestones
Abnormal gait (once walking)
Asymmetrical hand func <12m
Feeding difficulties
Primitive reflexes may persist
Muscle stiffness (but floppy at birth)
Neonatal seizures
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5
Q

What are the different subtypes of cerebral palsy? (6)

A

Spastic cerebral palsy (90%): Hemiplegia, Quadriplegia, Diplegia
Dyskinetic cerebral palsy (6%)
Ataxic (hypotonic) cerebral palsy

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6
Q

Where is the neurological damage in spastic cerebral palsy?

What are the main features of spastic cerebral palsy? (3)

A

= due to damaged UMN pathway (pyramidal/corticospinal)

Hypertonia (persistent = spasticity)
Spasticity = velocity dependent
Hyperreflexia

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7
Q

How does each subtype of spastic CP present?

A

Hemiplegia:
Unilateral, arm>leg (face spared)
Hypo then hypertonic
Presents at 4-12m with fisted hand, arm flexed/pronated, asymm hand func/reaching

Quadriplegic:
All 4 limbs + often severe
Opisthotonus
Poor head control
Poor central tone

Diplegic:
Can affect all 4 but legs>arms (walking abnorm, hand func norm)
Normal intellectual function

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8
Q

Where is the neurological damage in dyskinetic CP?

When will features begin to present?

A

Due to BG damage e.g. kernicterus, HIE

Features present by end of 1st year of life

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9
Q

What are the main features of dyskinetic CP? (5)

A

Involuntary movements (more evident with active movement/ stress)
Muscle tone variable but generally poor trunk control + floppy
Delayed motor milestones
Primitive reflexes predominate
Intellect unimpaired

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10
Q

What patterns of involuntary movement may present in dyskinetic CP? (3)

A

Chorea (irregular, sudden, non-repetitive)
Athetosis (slow writhing, e.g. finger fanning)
Dystonia (agonist/antagonist contractions)

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11
Q

What is ataxic (hypotonic) cerebral palsy usually caused by?

How does it present? (6)

A

Usually genetic

Delayed motor milestones
Early trunk/limb hypotonia
Poor balance
Incoordinate movements
Intention tremor
Ataxic gait (later)
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12
Q

What are the treatment options in cerebral palsy?

A

Physio (start soon as Dx - to prevent unused mm weakness / mms getting stuck in rigid position)

Drugs: diazepam (musc relaxant), botox (relieve stiffness), baclofen intrathecal (blocks some nerve signals)

Orthopaedic surgery (lengthens muscles - req multiple as child grows)

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13
Q

Define a febrile convulsion
What is the incidence
What age group
Any RFs

A

= seizure + fever w/o intracranial infection (bact mening/ viral enceph)
Occurs in 3% of 6m-6yrs
Genetic predispo if 1st degree relative (10% risk)

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14
Q

How do febrile convulsions present?

What are the RFs for having more (after having 1)? (3)

A

Usually during viral infection when temp rapidly rises
Brief generalised tonic-clonic

RF for more:
Younger child
+ve FH
Shorter illness duration/ lower temp before 1st seizure

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15
Q

What are some common misconceptions about complications / prognosis of febrile convulsions? (3)

A

Do not cause brain damage
No effect on subsequent intellectual ability
V slightly increased risk of developing epilepsy (esp if complex febriles - 4-12%) but still only 1-2% same as all children

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16
Q

What 3 things done in management of febrile convulsions?

What 4 things not to do

A

Rule out meningitis
Inform/reassure parents
Teach first aid basics: recovery position on soft surf + 999 if >5mins

Antipyretics, cold sponges, anti epileptics, EEG not needed / don’t work

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17
Q

What is the incidence of epilepsy?

What are the poss causes? (3)

A

0.5%

Usually idiopathic
Brain tumours
Brain damage

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18
Q

What are the general features (3) of generalised seizures?

And specific features for each subtype (5 subtypes)

A

Loss of consciousness (always)
No warning
Symmetrical seizure

Absence - no motor signs except eye flickering
Precipitated by hyperventilation

Myoclonic - brief repetitive jerky movements
Atonic - myoclonic jerk then transient loss tone → sudden fall/head drop

Tonic - generalised increased tone

Tonic-clonic
Fall to ground, stop/irreg breathing, cyanose
Saliva, tongue bite + incontinence
Rhythmical contraction of mm groups after tonic phase
V tired after

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19
Q

What are the features of focal seizures

From the diff sites (4)

A

May/may not lose consciousness

Frontal - motor, clonic movements
Temporal (commonest) - auditory/sensory
Occipital - visual phenomena (+ve/-ve)
Parietal - contralateral dysaesthesias (altered sensation)

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20
Q

What are some associated epileptic conditions / syndromes (5) seen in childhood + what would the EEG show?

A

Juvenile myoclonic - generalised 4-6Hz polyspike + slow wave discharge

Benign epilepsy (tonic-clonic in sleep OR simple focal with awareness) - sharp focal waves

Childhood absence epilepsy - 3 per second spike + wave (bilaterally synchronous)

West syndrome (infantile spasms) - hypsarrthymia (chaotic slow-wave with sharp multifocals)

Lennox-Gastaut syndrome (tonic/atypical absence)

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21
Q

What types of EEG can be done in epilepsy?

What other Ix can be done? (2)

A

EEG unreliable unless seizure captured
If normal → sleep/sleep-deprived study
24hr ambulatory EEG or video-telemetry can be done

MRI/CT (id tumours/vascular/sclerotic area)
PET/SPECT

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22
Q 
What drug(s) are 1st line / 2nd line anticonvulsants in:
tonic-clonic
absence
myoclonic
focal
A

T-C: 1st - valproate, carba + 2nd - lamotrigine
Absence: 1st - valproate, ethosuximide + 2nd - lamotrigine
Myo: 1st - valproate + 2nd - lamotrigine

Focal: 1st valproate, carba + 2nd - topiramate

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23
Q

What other drugs (not anticonvulsant) can be given in epilepsy?
What other non-pharm treatments are available? (3)

A

Rectal/buccal diazepam for prolonged seizures

Ketogenic diets
Vagal nerve stimulation
Surgery (if well localised)

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24
Q

What are the SEs of valproate (3)

A

Wt gain
Hair loss
Liver failure (rarely)

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25
Q

SEs of carbamazepine (5)

A 
Rash
Neutropenia
Hyponatraemia
Ataxia
Liver enzyme induction
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26
Q

SEs of Lamotrigine

SEs of Ethosuximide

A

Rash

N+V

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27
Q

What are some RFs for SUDEP (sudden unexpected death) (3)

A

Generalised tonic-clonics
Poor seizure control
Seizures occurring in sleep

28
Q

What are some causes of Fits/Faints/Funny Turns (transient loss of consciousness) (7)

A

Reflex anoxic seizures
Breath holding

Syncope
Migraine
Cardiac
BPPV
Other: pseudoseizures, atonic, NAI
29
Q

What is a breath holding attack?
How are they classified (2)/ present?
What reassuring info about breath holding attacks can be passed onto parents

A

When young child (6m-6yrs) angry/pain etc → stops breathing up to 1min (reflex not deliberate)

Cyanotic (commonest): angry/frustrated → skin red/blue-purple

Pallid: fear/pain (esp head trauma)

Not serious/damaging + should eventually resolve

30
Q

What are some of the symptoms of a cyanotic spell (e.g. in breath holding attacks) (5)

A

Rigorous crying → Hyperventilation → Pause breathing after exhalation → red/blue skin + lips → poss seizures

31
Q 
What are some RFs / triggers for reflex anoxic seizures?
What happens (features)?
A

Toddlers/infants
1st degree FH faints
PMH febrile convulsions
Triggers: pain/discomfort (e.g. head trauma, cold food)

Vagal inhibiton → cardiac asystole + hypoxia
→ Child goes pale + falls (+ generalised tonic-clonic)

32
Q

List some causes of ataxia (9)

A 
Acute causes:
Drugs/meds
Alcohol
Solvents
Trauma

Posterior fossa lesions/tumours (CPA syndrome)

Post viral (varicella)

Genetic/degenerative disorders:
Ataxic CP
Freiderichs ataxia
Ataxia telangiectasia

33
Q

What Ix could you do in ataxia? (3)

A

Genetics
LP
Brain scans

34
Q

What are the different types if brain tumours seen in children? (5)

A 
Astrocytoma (40%)
Medulloblastoma (20%)
Ependymoma (8%) (post. fossa; acts like medulloblastoma)
Brain stem glioma (6%)
Craniopharyngioma (4%)
35
Q

How does an astrocytoma present? (3)

A

Seizures
Headaches
Focal neuro signs

36
Q

How does a medulloblastoma / ependyoma present? (4)

A

Truncal ataxia / coordination probs
Abnormal eye movements
Morning vomiting (raised ICP)
20% present with spinal metastases at Dx

37
Q

How does a brain stem glioma present? (4)

A 
Early childhood
CN defects
Pyramidal tract signs
Ataxia
(No raised ICP)
38
Q

How does a craniopharyngioma present? (4)

A

Visual field loss: bitemporal hemianopia (initially bitemporal inferior quadrantanopia)

Pituitary failure:
Growth failure
Wt gain
Diabetes insipidus

39
Q

List some causes of developmental regression (5)

A

→ neurodegenerative disorders:
Battens disease
Wilson’s disease
Rett’s syndrome (pervasive developmental disorder)
Leukodystrophies
SSPE (subacute sclerosis panencephalitis)

40
Q

What is Battens disease

How does it present? (7)

A

Rare fatal autosomal recessive disorder

Presents at 4-10y/o with gradual onset
Seizures
Visual problems
Speech 
Behav change 
Devel regression
→ Dementia + death
41
Q

What is Wilsons disease

How does it present in younger vs older children (3+4)

A

Autosomal recessive - reduced copper binding prot → defective excretion in bile → accum of Copper

Presents in >3y/o with liver disease:
Hepatitis
Cirrhosis
Portal HT

Present in older with neuropsych features:
Reduced school performance (cognitive deterioration)
Parkinsonism
Seizures/migraines
Behavioural change

42
Q

What are leukodystrophies?

How do they present?

A

Dysfunc of white matter - due to incorrect growth of myelin sheath

Gradual loss of:
Movement
Speech
Vision
Hearing
Behaviour
43
Q

What is SSPE due to?

Describe the natural history / presentation of it

A

Rare chronic progressive encephalitis due to immune resistant measles virus
H/o infection at <2yrs → asymp for 6-15yrs before gradual psychoneuro deterioration

44
Q

What is hydrocephalus due to

What are the 3 categories of causes + possible sites of pathology

A

= obstruction of CSF flow → dilated ventricular system

Obstructive: within ventricular system / aqueduct

Communicating: arachnoid villi / CSF absorption site / CSF overproduction / venous drainage insufficiency

External: arachnoid villi immaturity → absorption defc (benign, self-limiting in infancy/early)

45
Q

How does benign external hydrocephalus present (4)

A

Raised ICP (signs seen in older children)
Disproportionate large head circumference (failure of suture formation)
Bulging anterior fontanelles
Scalp vv’s distend

46
Q

What is macrocephaly defined as?

What Ix can be done for it (3)

A

= head circumference >98th centile

Intracranial USS (if anterior fontanelle still open)
or CT / MRI
47
Q

List some possible causes of macrocephaly (8)

A

Tall
Familial macrocephaly

NF-1
Cerebral gigantism (Sotos syndrome)
CNS storage disorders e.g. mucopolysaccharidosis

Rapidly raised ICP:
Hydrocephalus
Subdural haematoma
Brain tumours

48
Q

What is microcephaly defined as?

List some causes (4)

A

= head circumference <2nd centile

Familial
Autosomal recessive condition (assoc w. devel delay)
Congenital infection (e.g. Zika)
Acquired post-insult to developing brain (e.g. perinatal hypoxia / hypogly / meningitis) (assoc w. CP / seizures)

49
Q

What is craniosynostosis + the diff presentations of it (3)

A

= premature fusion of 1+ sutures → distortion of head shape
Sagittal (commonest) → long narrow skull
Coronal → asymmetrical
Lambdoid → flattening (diff to plagcephaly - positional flattening)

50
Q

What are some S&S of raised ICP in young infants? (5)

A 
Vomiting
Separation of sutures / tense fontanelle
Raised head circumference
Head tilt / posturing 
Developmental delay / regression
51
Q

What are some S&S of raised ICP in older children? (5)

A 
Headache - worse in mornings as supine
Vomiting - on waking
Change in personality/behaviour
Visual disturbance
Papilloedema
52
Q

What are the main causes of raised intracranial pressure (4) + how are they managed

A

Tumour → surgical removal + chemo/radio
Hydrocephalus → ventriculoperitoneal shunt
Subdural haematoma → surgical drainage
Idiopathic → osmotic diuretics + hypoventilation (vasocon)

53
Q

List 2 causes of communicative (CSF resorption) hydrocephalus

A

Meningitis (pneumococcal, meningococcal)

Subarachnoid haemorrhage

54
Q

What is the most common cause of subdural haematoma in children?

A

NAI (shaking/direct trauma in infants/toddlers)

or Fall from significant height

55
Q

What are some features of subdural haematoma in children? (7)

A 
Headache
Seizures
Apnoea / breathing difficulties
Sudden cardiac arrest
Retinal haemorrhages

Altered mental state
Lethargy

56
Q

What info should be gathered from the Hx in a child presenting with headaches (SATHEVAA)

A 
SOCRATES
Aura / premonitory symps (tired, autonomic features)
Triggers: food / relaxation / stress / menstruation
Head trauma? 
Emotional/behav probs at school?
Vision checked?
Analgesia overuse?
Alc/drug/solvent abuse?
57
Q

What are the different types of headache and their main features? (4)

A

Tension-type - symmetrical constriction band

Migraine w/o aura - bi/unilateral, pulsatile, GI disturbance (N+V), photophobia, relieved by sleep

Migraine w. aura - visual (zigzags) / sensory, premon symps

Mixed-type (common)

58
Q

What general signs (not red flag) can be looked for O/E in a child with headache? (4)

A

BP
Visual acuity
Sinus tenderness (sinusitis)
Pain on chewing (temporomandibular joint malocclusion)

59
Q

What are the red flag S&S for headaches? (5+10)

A 
Headache worse supine / cough/straining
Wakes child up (e.g. migraine)
N+V
Confusion
Personality/behaviour change

Visual field defects (craniopharyngioma)
Fundus (papilloedema)
Squint
Torticollis

CN abnormality
Coordination abnormality (cerebellar)
Gait (UMN/cerebellar)

Growth failure
Bradycardia
Carotid bruits (AVM)

60
Q

What is myotonic dystrophy + the key features (4)

A

Myotonia = delayed relaxation after sustained mm contraction

Poor feeding
Failure meeting milestones
Hypotonia
Myotonia (e.g. releasing hand after shaking)

61
Q

What are the main features of congenital muscular dystrophy? (4) How different to Duchenne’s?
What Ix can be done for it

A

Present in birth / early infancy (how diff to Duchenne’s)
Proximal weakness
Hypotonia
Contractures

→ Biopsy

62
Q 
What is Guillain-Barre
Main features (5)
A

Autoimmune antibodies against myelin of PNS

Post-URTI/Gastroenteritis
Ascending symmetrical weakness
Areflexia 
Abnormal sensory symps in legs (pain, tingling)
? respiratory depression
63
Q

What results seen in Ix for GBS? (2)

Management (2)

A

CSF: raised protein + normal WCC
Reduced nerve conduction velocities

Supportive: e.g. ventilation
IVIG/Plasmaphoresis

64
Q

What is the main features of Charcot-Marie-Tooth disease (5)

What caused by

A

Autosomal dominant defect in neuronal proteins (myelin)

Onset by 10y/o
Distal muscle wasting + sensory loss (→ ataxia)
Areflexia
Foot drop
Spinal deformities (50%)
65
Q

What is spinal muscular atrophy?

A

Anterior horn neurodegeneration → progressive weakness / wasting of SkM
2nd commonest neuromuscular disease after DMD in UK

Child Health (14 decks)

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