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Neurology Flashcards

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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
SEs of carbamazepine (5)
``` Rash Neutropenia Hyponatraemia Ataxia Liver enzyme induction ```
26
SEs of Lamotrigine | SEs of Ethosuximide
Rash N+V
27
What are some RFs for SUDEP (sudden unexpected death) (3)
Generalised tonic-clonics Poor seizure control Seizures occurring in sleep
28
What are some causes of Fits/Faints/Funny Turns (transient loss of consciousness) (7)
Reflex anoxic seizures Breath holding ``` Syncope Migraine Cardiac BPPV Other: pseudoseizures, atonic, NAI ```
29
What is a breath holding attack? How are they classified (2)/ present? What reassuring info about breath holding attacks can be passed onto parents
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
What are some of the symptoms of a cyanotic spell (e.g. in breath holding attacks) (5)
Rigorous crying → Hyperventilation → Pause breathing after exhalation → red/blue skin + lips → poss seizures
31
``` What are some RFs / triggers for reflex anoxic seizures? What happens (features)? ```
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
List some causes of ataxia (9)
``` 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
What Ix could you do in ataxia? (3)
Genetics LP Brain scans
34
What are the different types if brain tumours seen in children? (5)
``` Astrocytoma (40%) Medulloblastoma (20%) Ependymoma (8%) (post. fossa; acts like medulloblastoma) Brain stem glioma (6%) Craniopharyngioma (4%) ```
35
How does an astrocytoma present? (3)
Seizures Headaches Focal neuro signs
36
How does a medulloblastoma / ependyoma present? (4)
Truncal ataxia / coordination probs Abnormal eye movements Morning vomiting (raised ICP) 20% present with spinal metastases at Dx
37
How does a brain stem glioma present? (4)
``` Early childhood CN defects Pyramidal tract signs Ataxia (No raised ICP) ```
38
How does a craniopharyngioma present? (4)
Visual field loss: bitemporal hemianopia (initially bitemporal inferior quadrantanopia) Pituitary failure: Growth failure Wt gain Diabetes insipidus
39
List some causes of developmental regression (5)
→ neurodegenerative disorders: Battens disease Wilson's disease Rett's syndrome (pervasive developmental disorder) Leukodystrophies SSPE (subacute sclerosis panencephalitis)
40
What is Battens disease | How does it present? (7)
Rare fatal autosomal recessive disorder ``` Presents at 4-10y/o with gradual onset Seizures Visual problems Speech Behav change Devel regression → Dementia + death ```
41
What is Wilsons disease | How does it present in younger vs older children (3+4)
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
What are leukodystrophies? | How do they present?
Dysfunc of white matter - due to incorrect growth of myelin sheath ``` Gradual loss of: Movement Speech Vision Hearing Behaviour ```
43
What is SSPE due to? | Describe the natural history / presentation of it
Rare chronic progressive encephalitis due to immune resistant measles virus H/o infection at <2yrs → asymp for 6-15yrs before gradual psychoneuro deterioration
44
What is hydrocephalus due to | What are the 3 categories of causes + possible sites of pathology
= 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
How does benign external hydrocephalus present (4)
Raised ICP (signs seen in older children) Disproportionate large head circumference (failure of suture formation) Bulging anterior fontanelles Scalp vv's distend
46
What is macrocephaly defined as? What Ix can be done for it (3)
= head circumference >98th centile ``` Intracranial USS (if anterior fontanelle still open) or CT / MRI ```
47
List some possible causes of macrocephaly (8)
Tall Familial macrocephaly ``` NF-1 Cerebral gigantism (Sotos syndrome) CNS storage disorders e.g. mucopolysaccharidosis ``` Rapidly raised ICP: Hydrocephalus Subdural haematoma Brain tumours
48
What is microcephaly defined as? | List some causes (4)
= 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
What is craniosynostosis + the diff presentations of it (3)
= premature fusion of 1+ sutures → distortion of head shape Sagittal (commonest) → long narrow skull Coronal → asymmetrical Lambdoid → flattening (diff to plagcephaly - positional flattening)
50
What are some S&S of raised ICP in young infants? (5)
``` Vomiting Separation of sutures / tense fontanelle Raised head circumference Head tilt / posturing Developmental delay / regression ```
51
What are some S&S of raised ICP in older children? (5)
``` Headache - worse in mornings as supine Vomiting - on waking Change in personality/behaviour Visual disturbance Papilloedema ```
52
What are the main causes of raised intracranial pressure (4) + how are they managed
Tumour → surgical removal + chemo/radio Hydrocephalus → ventriculoperitoneal shunt Subdural haematoma → surgical drainage Idiopathic → osmotic diuretics + hypoventilation (vasocon)
53
List 2 causes of communicative (CSF resorption) hydrocephalus
Meningitis (pneumococcal, meningococcal) | Subarachnoid haemorrhage
54
What is the most common cause of subdural haematoma in children?
NAI (shaking/direct trauma in infants/toddlers) | or Fall from significant height
55
What are some features of subdural haematoma in children? (7)
``` Headache Seizures Apnoea / breathing difficulties Sudden cardiac arrest Retinal haemorrhages ``` Altered mental state Lethargy
56
What info should be gathered from the Hx in a child presenting with headaches (SATHEVAA)
``` 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
What are the different types of headache and their main features? (4)
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
What general signs (not red flag) can be looked for O/E in a child with headache? (4)
BP Visual acuity Sinus tenderness (sinusitis) Pain on chewing (temporomandibular joint malocclusion)
59
What are the red flag S&S for headaches? (5+10)
``` 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
What is myotonic dystrophy + the key features (4)
Myotonia = delayed relaxation after sustained mm contraction Poor feeding Failure meeting milestones Hypotonia Myotonia (e.g. releasing hand after shaking)
61
What are the main features of congenital muscular dystrophy? (4) How different to Duchenne's? What Ix can be done for it
Present in birth / early infancy (how diff to Duchenne's) Proximal weakness Hypotonia Contractures → Biopsy
62
``` What is Guillain-Barre Main features (5) ```
Autoimmune antibodies against myelin of PNS ``` Post-URTI/Gastroenteritis Ascending symmetrical weakness Areflexia Abnormal sensory symps in legs (pain, tingling) ? respiratory depression ```
63
What results seen in Ix for GBS? (2) | Management (2)
CSF: raised protein + normal WCC Reduced nerve conduction velocities Supportive: e.g. ventilation IVIG/Plasmaphoresis
64
What is the main features of Charcot-Marie-Tooth disease (5) What caused by
Autosomal dominant defect in neuronal proteins (myelin) ``` Onset by 10y/o Distal muscle wasting + sensory loss (→ ataxia) Areflexia Foot drop Spinal deformities (50%) ```
65
What is spinal muscular atrophy?
Anterior horn neurodegeneration → progressive weakness / wasting of SkM 2nd commonest neuromuscular disease after DMD in UK

Child Health (14 decks)

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