Neurology Flashcards
What is cerebral palsy defined as?
What is its incidence?
= movement/postural abnormalities leading to activity limitation due to disturbances during fetal brain development
2 in 1000 live births
What are some other associated problems with cerebral palsy? (9)
Learning difficulties (60%) Epilepsy (40%) Squint (30%) Vision problems (20%) Hearing problems (20%)
Speech + lang disorders
Behavioural probs
Joint contractures/subluxations/scoliosis
Feeding probs
List the possible causes of cerebral palsy (12)
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
What are some of the early features of cerebral palsy? (7)
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
What are the different subtypes of cerebral palsy? (6)
Spastic cerebral palsy (90%): Hemiplegia, Quadriplegia, Diplegia
Dyskinetic cerebral palsy (6%)
Ataxic (hypotonic) cerebral palsy
Where is the neurological damage in spastic cerebral palsy?
What are the main features of spastic cerebral palsy? (3)
= due to damaged UMN pathway (pyramidal/corticospinal)
Hypertonia (persistent = spasticity)
Spasticity = velocity dependent
Hyperreflexia
How does each subtype of spastic CP present?
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
Where is the neurological damage in dyskinetic CP?
When will features begin to present?
Due to BG damage e.g. kernicterus, HIE
Features present by end of 1st year of life
What are the main features of dyskinetic CP? (5)
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
What patterns of involuntary movement may present in dyskinetic CP? (3)
Chorea (irregular, sudden, non-repetitive)
Athetosis (slow writhing, e.g. finger fanning)
Dystonia (agonist/antagonist contractions)
What is ataxic (hypotonic) cerebral palsy usually caused by?
How does it present? (6)
Usually genetic
Delayed motor milestones Early trunk/limb hypotonia Poor balance Incoordinate movements Intention tremor Ataxic gait (later)
What are the treatment options in cerebral palsy?
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)
Define a febrile convulsion
What is the incidence
What age group
Any RFs
= seizure + fever w/o intracranial infection (bact mening/ viral enceph)
Occurs in 3% of 6m-6yrs
Genetic predispo if 1st degree relative (10% risk)
How do febrile convulsions present?
What are the RFs for having more (after having 1)? (3)
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
What are some common misconceptions about complications / prognosis of febrile convulsions? (3)
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
What 3 things done in management of febrile convulsions?
What 4 things not to do
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
What is the incidence of epilepsy?
What are the poss causes? (3)
0.5%
Usually idiopathic
Brain tumours
Brain damage
What are the general features (3) of generalised seizures?
And specific features for each subtype (5 subtypes)
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
What are the features of focal seizures
From the diff sites (4)
May/may not lose consciousness
Frontal - motor, clonic movements
Temporal (commonest) - auditory/sensory
Occipital - visual phenomena (+ve/-ve)
Parietal - contralateral dysaesthesias (altered sensation)
What are some associated epileptic conditions / syndromes (5) seen in childhood + what would the EEG show?
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)
What types of EEG can be done in epilepsy?
What other Ix can be done? (2)
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
What drug(s) are 1st line / 2nd line anticonvulsants in: tonic-clonic absence myoclonic focal
T-C: 1st - valproate, carba + 2nd - lamotrigine
Absence: 1st - valproate, ethosuximide + 2nd - lamotrigine
Myo: 1st - valproate + 2nd - lamotrigine
Focal: 1st valproate, carba + 2nd - topiramate
What other drugs (not anticonvulsant) can be given in epilepsy?
What other non-pharm treatments are available? (3)
Rectal/buccal diazepam for prolonged seizures
Ketogenic diets
Vagal nerve stimulation
Surgery (if well localised)
What are the SEs of valproate (3)
Wt gain
Hair loss
Liver failure (rarely)
SEs of carbamazepine (5)
Rash Neutropenia Hyponatraemia Ataxia Liver enzyme induction
SEs of Lamotrigine
SEs of Ethosuximide
Rash
N+V
What are some RFs for SUDEP (sudden unexpected death) (3)
Generalised tonic-clonics
Poor seizure control
Seizures occurring in sleep
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
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
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
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)
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
What Ix could you do in ataxia? (3)
Genetics
LP
Brain scans
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%)
How does an astrocytoma present? (3)
Seizures
Headaches
Focal neuro signs
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
How does a brain stem glioma present? (4)
Early childhood CN defects Pyramidal tract signs Ataxia (No raised ICP)
How does a craniopharyngioma present? (4)
Visual field loss: bitemporal hemianopia (initially bitemporal inferior quadrantanopia)
Pituitary failure:
Growth failure
Wt gain
Diabetes insipidus
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)
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
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
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
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
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)
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
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
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
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)
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)
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
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
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)
List 2 causes of communicative (CSF resorption) hydrocephalus
Meningitis (pneumococcal, meningococcal)
Subarachnoid haemorrhage
What is the most common cause of subdural haematoma in children?
NAI (shaking/direct trauma in infants/toddlers)
or Fall from significant height
What are some features of subdural haematoma in children? (7)
Headache Seizures Apnoea / breathing difficulties Sudden cardiac arrest Retinal haemorrhages
Altered mental state
Lethargy
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?
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)
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)
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)
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)
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
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
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
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%)
What is spinal muscular atrophy?
Anterior horn neurodegeneration → progressive weakness / wasting of SkM
2nd commonest neuromuscular disease after DMD in UK
