Neuro Flashcards
(94 cards)
1
Q
How can you classify headaches
A
Primary
Secondary
Trigeminal /other cranial neuralgia
2
Q
WHat are primary headaches
A
Migraine
Tension
Cluster
3
Q
What are the secondary headaches
A
intracranial haemorrhage
raised ICP
substance / its withdrawal
infection
4
Q
Describe tension headacher
A
symmetrical
gradual onset
tightness
5
Q
Describe migraine without aura
A
Bilat /unilat pulsatile (frontal / temporal area) GI disturbance photophobia, phonophobia worse with activity, loud noises, light better with sleep, dark
6
Q
Describe migraine WITH aura
A
aura is usually visual disturbance
- negative phenomenon: hemianopia, scotoma
- positive phenomenon: zigzag
7
Q
How do you manage migraine
A
Heacache diary
Nasal sumatriptan + NSAID/paracetamol
8
Q
What are features of raised ICP
A
worse lying down, straining morning vomiting changes in mood/personality visual field defect abnormal gait
9
Q
What is a seizure
A
paroxysmal abnoprmality of motor, sensory, autonomic, cognitive unction
10
Q
What is an EPILEPTIC seizure
A
seizure caused by ABNORMAL underlying electrical activity
11
Q
What are causes of seizures categorised as
A
epileptic
non-epilleptic
12
Q
causes of epileptic seizure
A
- idiopatic - cause UNKNOWN
- cerebral vascular occlusion
- cerebral dysgenesis/malformation
- cerebral damage
- cerebral tumour
- neurodegenerativ disorder
13
Q
What is causes of non epileptic seizures
A
febrile seizure metabolic (hypoglycaemia, hypernatraemia, hyponat) head trauma meningitis encephalitis toxins, poisons
14
Q
What is a febrile seizure
A
Seizure accompanied by FEVER without intracranial infection
without prior non febrile seizure
without infection of brain tissue
15
Q
What are febrile seizures like
A
brief generalised tonic clonic
16
Q
How do you manage a seizure in a child
A
protect from injury (cushion head)
remove harmful objects
Do NOT restrain / put anything in mouth
Once seizure stops, place in recovery position
17
Q
What do you do if seizure is longer than 5 mins
A
STATUS EPILETTICUS
if IV access: Lorazepam
otherwise:
- rectal diazepam (repeated once after 5 mins)
- buccal midazolam
CALL FOR SENIOR HELP
18
Q
What do you do if they are still seizing after the first round of medication > what about after that
A
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
19
Q
What are the types of epileptic seizures
A
Tonic clonic atonic tonic myotonic absecnce
20
Q
what is a myotonic seizure like
A
brief repetitive jerky movement of limbs
21
Q
what is a tonic seizure like
A
increase in tone
22
Q
whart is an atonic seizure like
A
loss of muscle tone (causes fall to floor / drop of head)
23
Q
what are investigations for atonic seizure
A
ECG
EEG
MRI
24
Q
Why do yu need to do an ECG in child with seizure
A
exclude arrhytmia causing convulsive syncope (e.g. long QT)
25
What is appropriate choice of antiepileptic
GENERALISED (tonic clonic, absence, myoclonic): VALPROATE
FOCAL: carbamazepine, lamotrigine
26
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
27
What is Gower's sign
Need to turn prone to rise
Normal until 3yo
Then indicates weakness
28
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)
29
What is another name for Charcot Marie Tooth Disease
hereditary sensory myopathy
30
Explain Charcot Marie Tooth
mutation in myelin genes
| causes symmetrical slow progressive distal muscle wasting
31
How does Charcot Marie Tooth present
during preschool
| tripping from bilat foot drop
32
What does exam for Charcot Marie Tooth reveal
loss of ankle reflex
pes cavus
LL>UL
33
What are ix for Charcot Marie Tooth
nerve conduction studies
| Nerve biopsy- onion bulb formation
34
What is GBS caused by
post infectious polyneuropathy
occurs 2-3 weeks post URTI/campylobacter infection
35
How does GBS present
ascending, progressive, symmetrical weakness over days / weeks
loss of tendon rteflexes
36
what is the risk of GBS
risk of aspiration (due to difficulty swallowing(
37
How do you Ix GBS
MRI
LP (high CSF protein, normal WCC)
Nerve conduction (reduced velocities)
38
What is bell's palsy
LMN paresis of facial nerve (CN7)
39
What can trigger bells palsy
HSV
| Lyme disease
40
What causes MG
antibody to nAch receptor in NMJ
41
What is presentation of MG
```
Opthalmoplegia
Ptosis
Facial droop
Difficulty chewing
Proximal weakness (also generalised)
```
42
How do you investigate MG
Tensilon test
43
How do you manage MG
Cholinesterase inhibitors e.g. pyridostigmine, neostigmine
44
What do muscular dystrophies cause
progressive muscular degeneration
45
What is duchenne muscular dystrophy (genetically)
x linked recessibe
| deletion in dystrophin gene > myofibre necrosis
46
How does duchenne muscular dystrophy present
waddling gait
language delay
Gower sign positive
47
What is prognosis for duchenne muscular dystrophy (
not walking at 10yo
| death at 20-30 yo from resp failure
48
How do you manage duchenne muscular dystrophy (
physio
exercise
Surgery for scoliosis, tendoachilles lengthening
Weakness of intercostal muscles > nocturnal hypoxia > overnight CPAP
49
Explain becker muscular dystrophy
milder, progresses slowly
50
How does dermatomyositis present
```
fever
mysery
symmetrical muscle weakness
purple rash on eyelids
periorbital oedema
HIGH CK
```
51
What is Fredrich ataxi
AR > lack of frataxin protein
52
How does Fredrich ataxia present
DAWN P
Dysarthria
Ataxia
Wasting of LL (distal)
No reflexes
Pes cavus
53
What is anencephaly
failure to develop cranium and brain
| stillborn /die after birth
54
What is encephalocoele
extrusion of brain and meninges through midline skull defect
55
What is spina bifida occulta
failuyre of fusion of vertebral arch
| incidental finding on X reay
56
How does spina bifida occulta present
tuft of hair, lipoma, birth mark
57
what is meningocoele
sac protrudes from the body but only contains CSF
58
What is myelomeningocoele
severe spina bifida
| spinal corrd and nerves develop outside in CSF filled sac
59
How do you manage neural tube defects
surgical closure of lesion
physio
walking aids, wheelchair
neuropathic bladder...
60
what are the two types of hydrocephalus
communicating: failure to absorb CSF at arachnoid villi
| non-communicating: obstruction in venricular system / aqueduct
61
Give causes of communicating hydroceph
SAH
| meningitis
62
Give causes of non comm hydroceph
congenital malformation (aqueduct stenosis, fourth ventricle atrasia, Chiari)
63
How do you investigate hydrocephalus
cranial USS
CT/MRI
head circumference monitored, plotted on centile chart
64
What is management for hydrocphalus
insert ventriculoperioteal shunt
65
What is NF1 pattern of inheritance
Autosomal DOMINANT mutation in NF1 gene
66
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
67
What are features of NF2
- Schwannoma
- Meningioma
- Ependymoma
68
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
69
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
70
what is the triad occurring in shaken baby syndrome
retinal haemorrhages
subdural haematoma
encephalopathy
71
What is West syndrome also known as
infantile spasm w
72
what is west syndrome
form of childhood epilepsy
73
when does west syndrome present
4-8months of life
74
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
75
how do you investigate west syndrome
EEG (hypsarrhyrhmia)
| CT (brain disease e.g. tuberous sclerosis)
76
what is prognosis for west syndrome
POOR
77
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
78
what are features of cerebellar disortder
DANISH D
```
Dysdiadocokinesia
Ataxia
Nystagmus
Intention tremor
Slurred staccato speech
Hypotonia
```
Dysmetria
79
What is dysdiadocokinesia
inability to perform rapid alternating movements (palm to palm-dorsum)
80
what is dysmetria
past pointing
81
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
82
What mutations occur in tuberous sclerosis
AUTOSIOMAL DOMINANT
| TSC1 and TSC2
83
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
84
what can you give in Duchenne to prevent wheelchair dependence
glucocorticoids
85
How do you manage West syndrome
corticosteroids e.g prednisolone, vigabatrin
86
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
```
87
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
88
What is an extradural hemorrhage due to
Extradural haemorrhage: due to head trauma & skull fracture (middle meningeal artery rupture )
89
What are sx of extradural haemorrhage
lucid interval
| then consciousness deteriorates
90
What is a subdural haematoma due to
tearing of bridging veins across subdural space (slow bleed)
due to shaking injury in children
91
What is a subarachnoid haematoma due to
Rupture of aneurysm / AV malformation
92
What are symptoms of SAH
```
Severe headache, rapid onset
vomiting
confusion
reduced consciousness
cioma
```
93
What are the 3 features of shaken baby syndrome
Shake Every Registrar
Subdural haemorrhage
Encepalopathy
Retinal haemorrhage
94
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
