Neuro Flashcards
How can you classify headaches
Primary
Secondary
Trigeminal /other cranial neuralgia
WHat are primary headaches
Migraine
Tension
Cluster
What are the secondary headaches
intracranial haemorrhage
raised ICP
substance / its withdrawal
infection
Describe tension headacher
symmetrical
gradual onset
tightness
Describe migraine without aura
Bilat /unilat pulsatile (frontal / temporal area) GI disturbance photophobia, phonophobia worse with activity, loud noises, light better with sleep, dark
Describe migraine WITH aura
aura is usually visual disturbance
- negative phenomenon: hemianopia, scotoma
- positive phenomenon: zigzag
How do you manage migraine
Heacache diary
Nasal sumatriptan + NSAID/paracetamol
What are features of raised ICP
worse lying down, straining morning vomiting changes in mood/personality visual field defect abnormal gait
What is a seizure
paroxysmal abnoprmality of motor, sensory, autonomic, cognitive unction
What is an EPILEPTIC seizure
seizure caused by ABNORMAL underlying electrical activity
What are causes of seizures categorised as
epileptic
non-epilleptic
causes of epileptic seizure
- idiopatic - cause UNKNOWN
- cerebral vascular occlusion
- cerebral dysgenesis/malformation
- cerebral damage
- cerebral tumour
- neurodegenerativ disorder
What is causes of non epileptic seizures
febrile seizure metabolic (hypoglycaemia, hypernatraemia, hyponat) head trauma meningitis encephalitis toxins, poisons
What is a febrile seizure
Seizure accompanied by FEVER without intracranial infection
without prior non febrile seizure
without infection of brain tissue
What are febrile seizures like
brief generalised tonic clonic
How do you manage a seizure in a child
protect from injury (cushion head)
remove harmful objects
Do NOT restrain / put anything in mouth
Once seizure stops, place in recovery position
What do you do if seizure is longer than 5 mins
STATUS EPILETTICUS
if IV access: Lorazepam
otherwise:
- rectal diazepam (repeated once after 5 mins)
- buccal midazolam
CALL FOR SENIOR HELP
What do you do if they are still seizing after the first round of medication > what about after that
if still seizing after 10 minutes: IV/IO Lorazepam (0.1mg/kg)
If still seizing after 10 mins: Senior Help (anaethetic/ICU)
- phenytoin 20mg/kg IO/IV over 20 mins
- if on phenytoin already: phenobarbitone 20mg/kg IV/IO over 20 mins
What are the types of epileptic seizures
Tonic clonic atonic tonic myotonic absecnce
what is a myotonic seizure like
brief repetitive jerky movement of limbs
what is a tonic seizure like
increase in tone
whart is an atonic seizure like
loss of muscle tone (causes fall to floor / drop of head)
what are investigations for atonic seizure
ECG
EEG
MRI
Why do yu need to do an ECG in child with seizure
exclude arrhytmia causing convulsive syncope (e.g. long QT)
What is appropriate choice of antiepileptic
GENERALISED (tonic clonic, absence, myoclonic): VALPROATE
FOCAL: carbamazepine, lamotrigine
What are locations for neuromuscular disorders, and give examples of the disorders that arise
Anterior horn:
- spinal muscular atrophy
- polio
Peripheral nerve:
- hereditary sensory myopathy = Charcot Marie Tooth Disease
- GBS
- Bell’s
NMJ
- MG
Muscle disorders
- Duchenne/becker
- Myositis, polymiositis, dermatomyositis
What is Gower’s sign
Need to turn prone to rise
Normal until 3yo
Then indicates weakness
What is spinal muscle atrophy
AR - mutation in survival motor neurone 1
causes progressive weakness and wasting of skeletal muscle
T1: Werndig-Hoffman Disease (reduced foetal movements, arthrogyposis, death from resp failure)
What is another name for Charcot Marie Tooth Disease
hereditary sensory myopathy
Explain Charcot Marie Tooth
mutation in myelin genes
causes symmetrical slow progressive distal muscle wasting
How does Charcot Marie Tooth present
during preschool
tripping from bilat foot drop
What does exam for Charcot Marie Tooth reveal
loss of ankle reflex
pes cavus
LL>UL
What are ix for Charcot Marie Tooth
nerve conduction studies
Nerve biopsy- onion bulb formation
What is GBS caused by
post infectious polyneuropathy
occurs 2-3 weeks post URTI/campylobacter infection
How does GBS present
ascending, progressive, symmetrical weakness over days / weeks
loss of tendon rteflexes
what is the risk of GBS
risk of aspiration (due to difficulty swallowing(
How do you Ix GBS
MRI
LP (high CSF protein, normal WCC)
Nerve conduction (reduced velocities)
What is bell’s palsy
LMN paresis of facial nerve (CN7)
What can trigger bells palsy
HSV
Lyme disease
What causes MG
antibody to nAch receptor in NMJ
What is presentation of MG
Opthalmoplegia Ptosis Facial droop Difficulty chewing Proximal weakness (also generalised)
How do you investigate MG
Tensilon test
How do you manage MG
Cholinesterase inhibitors e.g. pyridostigmine, neostigmine
What do muscular dystrophies cause
progressive muscular degeneration
What is duchenne muscular dystrophy (genetically)
x linked recessibe
deletion in dystrophin gene > myofibre necrosis
How does duchenne muscular dystrophy present
waddling gait
language delay
Gower sign positive
What is prognosis for duchenne muscular dystrophy (
not walking at 10yo
death at 20-30 yo from resp failure
How do you manage duchenne muscular dystrophy (
physio
exercise
Surgery for scoliosis, tendoachilles lengthening
Weakness of intercostal muscles > nocturnal hypoxia > overnight CPAP
Explain becker muscular dystrophy
milder, progresses slowly
How does dermatomyositis present
fever mysery symmetrical muscle weakness purple rash on eyelids periorbital oedema HIGH CK
What is Fredrich ataxi
AR > lack of frataxin protein
How does Fredrich ataxia present
DAWN P
Dysarthria
Ataxia
Wasting of LL (distal)
No reflexes
Pes cavus
What is anencephaly
failure to develop cranium and brain
stillborn /die after birth
What is encephalocoele
extrusion of brain and meninges through midline skull defect
What is spina bifida occulta
failuyre of fusion of vertebral arch
incidental finding on X reay
How does spina bifida occulta present
tuft of hair, lipoma, birth mark
what is meningocoele
sac protrudes from the body but only contains CSF
What is myelomeningocoele
severe spina bifida
spinal corrd and nerves develop outside in CSF filled sac
How do you manage neural tube defects
surgical closure of lesion
physio
walking aids, wheelchair
neuropathic bladder…
what are the two types of hydrocephalus
communicating: failure to absorb CSF at arachnoid villi
non-communicating: obstruction in venricular system / aqueduct
Give causes of communicating hydroceph
SAH
meningitis
Give causes of non comm hydroceph
congenital malformation (aqueduct stenosis, fourth ventricle atrasia, Chiari)
How do you investigate hydrocephalus
cranial USS
CT/MRI
head circumference monitored, plotted on centile chart
What is management for hydrocphalus
insert ventriculoperioteal shunt
What is NF1 pattern of inheritance
Autosomal DOMINANT mutation in NF1 gene
What is criteria for NF1 dx
Two or more of following:
- 6+ cafe au lait spots
- 1+ neurofibroma
- axillary freckling
- optic glioma
- 1 lisch nodule
- first degree relative with NF
What are features of NF2
- Schwannoma
- Meningioma
- Ependymoma
What is tuberous sclerosis presentation
CUTANEOUS FTS:
- ash leaf shaped patches / amelanotic lesions, fluoresce under UV light
- Shagreen patches (roughened patches of skin over lumbar region)
- Adenoma sebaceum (angiofibromata over cheeks)
NEURO FTS:
- Infantile spasms
- Developmental delay
- Epilepsy
- intellectual disability
What does Sturge-Weber syndrome present with
Port wine stain in trigeminal N distribution
With similar lesion intracranially
ALWAYS involves opthalmic division of trigeminal nerve
what is the triad occurring in shaken baby syndrome
retinal haemorrhages
subdural haematoma
encephalopathy
What is West syndrome also known as
infantile spasm w
what is west syndrome
form of childhood epilepsy
when does west syndrome present
4-8months of life
what are features of west syndrome
- Salaam attacks - flexion of head, trunk and arms, followed by extension of arms
- lasts 1-2 seconds, repeated x50,
- progressive mental handicap
how do you investigate west syndrome
EEG (hypsarrhyrhmia)
CT (brain disease e.g. tuberous sclerosis)
what is prognosis for west syndrome
POOR
what do you need to explain to parents of children with febrile convulsions
NOT same as epilepsy
Risk of epilepsy in the future is only slightly higher than normal population
Short lasting seizures are NOT harmful to child
1/3 of children will have another febrile convulsion
Reducing the fever does not prevent recurrence
what are features of cerebellar disortder
DANISH D
Dysdiadocokinesia Ataxia Nystagmus Intention tremor Slurred staccato speech Hypotonia
Dysmetria
What is dysdiadocokinesia
inability to perform rapid alternating movements (palm to palm-dorsum)
what is dysmetria
past pointing
what are differentials for a FLOPPY BABY
CoMe GeNe
CENTRAL
- HIE
- cortical malformation
METABOLIC:
- hypothyroidism,
- hypocalcaemia
GENETIC: Down’s, Prader WIlli
PERIPHERAL - Neuromuscular
- spinal muscular atrophy
- myopathy
- myotonia
- congenital myasthenia
What mutations occur in tuberous sclerosis
AUTOSIOMAL DOMINANT
TSC1 and TSC2
How do you manage Duchenne
FOR MOVEMENT:
- Physio
- Exercise, psychological support
- Surgery: Tendochilles lengthening and scoliosis surgery if required
FOR RESP:
- Consider overnight CPAP (if weak intercostal muscles)
- glucocorticoids e.g. prednisolone
TO PREVENT WHEELCHAIR DEPENDENCE:
- Glucocorticoids
what can you give in Duchenne to prevent wheelchair dependence
glucocorticoids
How do you manage West syndrome
corticosteroids e.g prednisolone, vigabatrin
What investigations can you get in seizure ?
Obs (temperature for fever etc) Glucose, urine dip, MSU Bloods if indicated (e.g. U&E if D&V) Consider LP Consider CT if suspecting raised ICP/neuro origin
When do you admit a child with a suspected febrile seizure
first febrile seizure / priorly not assessed
diagnostic uncertainty
seizure lasted >15mins
focal features during the seizure
seizure recurred in same febrile illness
incomplete recovery
What is an extradural hemorrhage due to
Extradural haemorrhage: due to head trauma & skull fracture (middle meningeal artery rupture )
What are sx of extradural haemorrhage
lucid interval
then consciousness deteriorates
What is a subdural haematoma due to
tearing of bridging veins across subdural space (slow bleed)
due to shaking injury in children
What is a subarachnoid haematoma due to
Rupture of aneurysm / AV malformation
What are symptoms of SAH
Severe headache, rapid onset vomiting confusion reduced consciousness cioma
What are the 3 features of shaken baby syndrome
Shake Every Registrar
Subdural haemorrhage
Encepalopathy
Retinal haemorrhage
How do you manage traumatic head injury in children
Criteria:
- LOC >5mins
- Drowsy
- Vomiting 3+ times
- Dangerous mechanism of injury
- Amnesia >5mins
- On warfarin
If 0-1; observe for 4h
If 2+: Urgent CT
Criteria severe:
- GCS <15
- suspected open/depressed skull fraacture
- seizuree
- focal neurological deficit
Immediate CT + trauma call
