neuro, eyes and development Flashcards
(134 cards)
1
Q
EEG of absence seizures
A
3 sec spike and wave
2
Q
management of absence seizures
A
ethosuximide, sodium valproate
3
Q
management of tonic clonic seizures
A
1st line - sodium valproate
1st line in girls >10 y/o - lamotrigine
4
Q
describe infantile spasm
A
sudden symmetrical synchronous spasm and then a tonic phase
developmental delya
within 1st year of life
5
Q
EEG of infantile spasm
A
hypsarrhythmia
6
Q
treatment fo infantile spasm
A
vigabatrin
7
Q
describe lennox gastraut syndrome
A
visual aura **
poor prognosis and only 10% seizure free on meds
due to structural brain abnormalities
8
Q
describe landua kleffner syndrome
A
subacute onset of aphasia
previous normal development
residual language impairment
self limiting seizures
9
Q
describe benign rolandic epilepsy
A
short lived night time seizures
focal motor aware seizures e.g. twitching of mouth, dribbling, gurgling
interfere with speech
10
Q
EEG of benign rolandic epilepsy
A
centrotemporal spikes
11
Q
describe panayiotopoulos syndrome
A
prominent autonomic features e.g. vomiting, retching,pallor
12
Q
EEG of panayiotopoulos
A
occipital spikes
13
Q
management of neonatal seizures
A
phenobarbitone
14
Q
describe juvenile myoclonic epilepsy
A
absence seizures + myoclonic jerks of upper limbs (usually in morning)
GTC first time 13-18 y/o
family history
can be provoked by sleep deprivation
15
Q
EEG of juvenile myoclonic epilepsy
A
generalised polyspike and wave
4-6 hz polysike and slow wave discharge with frontal predominance over a normal background activity
16
Q
describe reflex anoxic seizures
A
triggered by unexpected noise/ startle/ pain
brief cardiac asystolic followed by excessive activity of vagus nerve
looks pale + LOC -> apnoea, eye rolling, jerking of arms and legs
does not affect development
17
Q
describe dravet syndrome
A
severe myoclonic epilepsy
triggered by fevers
developmental delay
difficult to control
70% have SCN1A mutation
18
Q
describe subacute sclerosing panencephalitis
A
complication 7-10 years after measles infection
fatal and progressive CNS disorder
myoclonus seizure
mood change
19
Q
investigations for subacute sclerosing panencephalitis
A
EEG - burst suppression
elevated anti measles antibody Igg IN SERUM AND CSF
20
Q
describe temporal seizures
A
complex visual hallucinations
auras of taste
21
Q
describe occipital seizures
A
simple visual experiences
22
Q
symptoms of raised ICP
A
early morning headache
vomiting
visual disturbances
fluctuating consciousnessness
ataxia
seizures
abnormal pupils
23
Q
cushings triad
A
- hypertension
- irregular breathing
- bradycardia
24
Q
consequence of cushings triad
A
signs of impending brainstem herniation
25
management of raised ICP
1. CT head with contrast
2. urgent neuro surgical discussion
3. lie with head at 20-30 degrees
4. mannitol or hypertonic saline 3%
26
what is idiopathic intracranial hypertension
elevated ICH >25 CM WATER with unknwon cause
27
management of idiopathic intracranial hypertension
1. MRI with venography
2. acetazolamide (carbonic anhydrase inhibitor) or topiramate
28
causes of subarachnoid haemorrhage
1. arteriorvenous malformations ****
2. cerebral aneuryms (circle of willis, sylvian fissure)
3. space occupying lesion
4. head trauma
29
presentation of subarachnoid haemorrhage
1. thunderclap headache - severe, sudden
2. vomiting
3. focal neurological signs e.g. seizures, irritable, enck stiffness
30
management fo subarachnoid haemorrhage
1. cT head and cerebral angiography
2. discuss neuro surgical team (?surgical clipping)
3. bp control
31
SF2 type of hydrocephalus
1. non communicating - obstruction of CSF reabsorption ***
2. communicating - impaired secretion or reabsorption
32
causes of non communicating hydrocephalus
1. CONGENITAL
- arnold chiari malformation
- spina bifida
- dandy walker malformation
- congenital toxoplasmosis
2. ACQUIRED
- mass lesions (medulloblastoma - obstruction at 4th ventricle)
- IVH
- infection
33
causes of communicating hydrocephalus
1. impaired secretion
- choroid plexus papilloma (excessive production of CSF)
2. impaired reabsorption
- infections e.g. meningitis
- haemorrhage
34
direction of CSF travel
1. produced by ependymal cells (simple columnar epithelium) (rate 30ml/hr) in choroid plexus (in lateral ventricle)
2. CSF travels through lateral ventricle
3. into 3rd ventricle via foramen on munro
4. into 4th ventricle via aqeduct of sulvius
5. into posterior fossa and basal cisterns
6. absorbed by arachnoid granulations in superior sagital dural venous sinuses and around cerebral hemisphere and down spinal cord
35
signs of hydrocephalus
increasing head circumference
bulging fontanelle
dilated scalp veins
sun setting sign
irritable
vomiting
impaired consiousness level
headache
36
management of non communicating hydrocephalus
ventriculoperitoneal shunt - one way valve so CSF draians from cranial vault to low pressure peritioneal cavity
37
causes of neonatal meningitis
1. group b strep
2. e.coli
3. listeria
38
Causes of child meningitis
1. enterovirus ** -most common
2. h . influenza
3. strep pneumonia
4. meningococcal
39
presentation of meningitis
fever
headache
irritable
vomiting
seizures
neck stiffness
non blanching petechial rash - meningococcal
photophobia
40
bacterial CSF of meningitis
1. high neutrophil count **
2. low glucose
3.. high portein
41
viral CSF of meningitis
1. high lymphocyte count
2. normal glucose
3. normal protein
42
management of meningitis
notifiable disease
1. iv antibiotics - ceftriaxone + amoxicillin (cefotaxime + amoxicillin in neonates)
2. IV aciclovir - if herpes suspected
3. IV dex - helps hearing loss
43
management of household contacts of meningitis
ciprofloxacin
IM ceftriaxone to pregnant women
44
cause of guillain barre syndrome
post infectious neuropathy (generate IgG antibodies against myelin sheath) around 10 days after:
1. gastroenteritis - campylobacter
2. vaccines - rabies, influenza
3. resp infection - mycoplasma
45
presentation of guillain barre syndrome
1. ascending pattern of progressive symmetrical weakness (starts in lower extremities)
2. can lead to resp failure
3. facial weakness, dysphasia or dysarthria
4. sensory loss (start in lower extremities)
46
investigations for GBS
1. Nerve conduction test
2. LP - elevated CSF protein, normal CSF cell count, normal glucose
47
management of GBS
1. IV IG
2. corticosteroids
3. intubation and ventilation in ITU
4. pain relief
48
presentation of dermatomyositis
RASH
1. 'shawl' sign - sensitivity to UV light on sun exposed area
2. heliotrope rash
3. gottrons papulss - thickened plaques on DIP joints and knees and elbows
4. thickened hands
MUSCLE WEAKNESS
1. proximal muscle weakness e.g. neck, shoudlers
2. difficulty standing up
49
diagnosis of dermatomyositis
1. ALT *, CK, aldolase rise
2. ANA +ve in 80%
3. myositis specific antibodies
4. muscle biopsy
50
management of dermatomyositis
1. avoid sun
2. physiotehrapy
3. corticosteroids over 12 months
4. methotrextate and hydroxychloroquine
51
describe bells palsy
lower motor neurone palsy, unilateral, preceded by viral infection
weakness of whole face
facial nerve compression as passes through temporal bone
52
defect in hemi section syndrome brown sequard
ipsilateral weakness, loss of vibration and position sense
contralateral loss of temperature + pain sensation (anterior and lateral spinothalamic tracts)
53
describe dandy walker malformation
1. cerebellar vermis hypoplasia
2. dilation of 4th ventricle
3. enlarged posterior fossa
54
describe horner syndrome
1. ptosis
2. decreased sweating on affected side
3. contracted pupil
use cocaine eye drops to distinguish horners and physiological aniscoria -> small pupil that does not dilate to cocaine eye drops has no sympathetic supply (cocaine drops would normally make pupil dilate within 30 mins)
55
diagnosis of spinal muscular atrophy
chromosomal microarray
56
drugs used to improve aquous outflow in glaucoma
1. pilocarpine
2. aparclonidine (alpha 2 adrenergic agonists)
3. prostaglandin anologies (latanoprost)
56
where does abducens and facial nerve arise
pontine medulla junction
56
where does trigeminal nerve arise
pons
56
describe self gratification behaviour
intact consciosuness
cessation of episode after distraction
grunting/ abnromal posturing/ arching
57
describe blue breath holding attacks
provoked by pain or frustration
remain in end expiratory aponoea -> turn blue -> floppy and unconscious for few seconds -> rapid recovery
reassure parents, no investigations needed
57
treatment for JME
sodium valproate or keppra
AVOID carbamazepine or lamotrigine as makes episodes worse
usually life long treatment as high risk of relapse
57
describe marcus gunn pupil
relative afferent defect
retina has sluggish response to light shining
into it
'jaw winking syndrome'
as open mouth, R eye lid goes up
58
describe coloboma
failure of closure of optic fissure in the 6th week of gestation
can be part of CHARGE syndrome (coloboma, heart abnormalities, atresia choanae, retardation of growth, ear abnormalities)
59
vascularisation of retina in utero
starts at 14 weeks and continues until birth
stimulated by vascular endothelial growth factor A and insulin like growth factor
60
describe retinopathy of prematurity
neovascular disorder affecting babies less than 32 weeks gestation or low birth weight (<1.5kg)
61
pathophysiology of retinopathy of prematurity
1. HYPEROXIC PHASE - high oxygen environment causes down regulation of VEGF halting process of vascularisation of the anterior retina
2. HYPOXIC PHASE - anterior retina becomes ischameic as it matures - can create retinal detachment and blindness
62
management of ROP
laser ablation of the asvacsular ischaemic anterior retina for severe ROP
intra vitreal injection of anti VEGF (bevacizumab or ranibizumab)
screen from 31 weeks or 4 weeks post birth
63
causes of congenital cataracts
1. idiopathic
2. familial (20%) if bilateral or parental consanguinity
3. syndromes e.g. downs , galactosaemia, tORCH
64
management of congenital cataracts
surgery within first 2 months of life
topical steroid therapy post op
65
describe childhood glaucoma
1. raised intra ocular pressure - due to impaired aqueous outflow through the trabecular meshwork
2. optic disc cupping
66
causes of childhood glaucoma
1. primary - < 1 y/o, bilateral and due to abnormal development of the drainage angle
2. secondary aniridia, sturge weber sydnrome , after congenital cataract surgery, steroid therapy
67
pattern of eye pathology in NAI
multiple bilateral retinal haemorrhages
- dark round sub retinal haemorrhages with a white centre (resolve in months)
- dot and blot shaped intra retinal haemorrohages (resolve within weeks)
- flame shaped nerve fibre layer haemorrhages (resolve in days)
68
what does the oculomotor nerve control in the eye (CN III)
1. superior division
- levateor palpebrae superiosis -> upper lid elevation
- superior rectus -> elevation of eye
2. inferior devision
- medial rectus -> adduction of eye
- inferior rectus -> depression of the eye (downward movement)
- inferior oblique -> elevates the adducted eye outwards, outward rotation
68
oculomotor (CN III) palsy
'down and out' eye with ptosis
68
what does trochlear nerve (CN IV) control in the eye
superior oblique - depresses the adducted eye, inward rotation
69
trochlear nerve palsy
problems reading/ going downstairs
reduced depression in the adducted eye
head tilt towards unaffect side
vertical diplopia
both eyes still look forward
70
what does the abducen nerve control (CN VI)
Lateral rectus - controls abduction
palsy = horizontal gaze
71
describe horners syndrome
1. small pupil which dilates poorly in the dark
72
which anti muscarinic eye drops are used
1. cyclopentolate 0.5% - last 24 hours
2. atropine 1% - lasts for 7 days
3. tropicamide 1% - last 3-6 hours
block parasympathetic innervation of the ciliary muscle and pupil sphincter
73
which sympathomimemetic eye drops are used
used with anti muscarinics for intense mydriasis - needed in surgery, ROP screening etc
1. phenylephrine 2.5%. last 12 hours
74
describe adie pupil
b/l or unilateral inability to constrict pupil (viral infection disrupts ciliary ganglion causing parasympathetic dysinnervation)
test using pilocarpine - miosis if +ve
75
risk factors for squints
downs syndrome
cerebral palsy
refractive error
cataract
retinoblastoma
family history
acute RF : head trauma, neoplasm, meningitis, encephalitis, hydrocephalus
76
define myopia
short sighted
77
define hypermetropia
long sighted
78
types of squints/ strabismus
1. concomitant / non paralytic **
= extra ocular muscles and nerves normal but IMBALANCE in muscle tone
2. paralytic squint
= weakness of one or more extraocular muscles - need to find a cause, deviation varies with direction of gaze
79
define convergent and divergent
convergent/ esotropia = one eye turns inwards (more common)
divergent = one eye turns outwards
80
how to test for squint
1. cover testing - if eye moves outward when uncovered , shows esotropia
2. corneal light reflex test
3. visual acuity test - complication of squint is ambylopia (visual loss), use Kays cards for 2-4 y/o
4. eye movements
81
management of squint
1. squint over > 12 weeks old should be referred to eye specialist
2. patching of the normal eye
3. correct refractive error
4. corrective surgery
82
causes of orbital cellulitis
1. H influenza
2. staph aureus (trauma to eye)
3. strep pneumoniae
4. fungi
complication of sinusitis
83
presentation of orbital cellulitis
- orbital pain
- proptosis
- chemosis (swelling of eyelids)
- diplopia
- reduced visual acuity
- painful extra ocular motion / restricted eye movements
- fever
84
complications of orbital cellulitis
- visual loss
- increased intraocular pressure
- central retinal artery occlusion
- orbital abscess
- meningitis
- sub periosteal abscess
85
investigations for suspected orbital cellulitis
1. FBC, blood cultures
2. eye swabs
3. ct of sinuses and head and orbit
86
management of orbital cellulitis
1. co-amoxiclav PO (if no fever, pre septal) or IV cefuoxime
2. surgery if collection / abscess/ no response to abx
87
cause of uveitis
1. autoimmune - SLE, JIA (oligoarticular), crohns, sarcoidosis, rheumatoid arthritis
2. HLA B27 - ank spon
3. idiopathic
88
how does uveitis present
red eye
ocular pain
photophobia
reduced visual acuity
tenderness of upper eyelid
89
structures in the uvea
1. anterior uvea - iris and ciliary body
2. posterior uvea - choroid
90
investigations for uveitis
1. bloods - autoimmune markers
2. ocular imaging - optical coherance tomography + fluorescin angiography
3. slit lamp - congestion of iris and white preticipates at anterior cornea
91
management of uveitis
1. anterior uveitis -> topical corticosteroids and periocular injection
2. posterior uveitis -> systemic immunosuppressants + steroids
+ atropine and phenylephrine (relieves pain by paralysing eye and stops iris lens adhesions)
92
pathophysiology of retinoblastoma
intraocular malignancy arising from retinal nerual cells
mutations in RB1 gene on chromosome 13q/14
inherited via germline (higher risk of b/l disease) or sporadic mutation
93
presentation of retinoblastoma
1. leukocoria = white eye reflex on newborn screening
2. strabismus
3. reduced vision
4. eye redness and pain due to inflammmation
94
differentials for leukocoria
1. congenital cataracts **
2. retinoblastoma
95
investigations for retinoblastoma
1. CT and MRI brain
2. ophthalmoscopy with cyclopentolate eye drops
3. genetic testing for RB1 gene
96
management of retinoblastoma
systemic chemo and radio - vincristine, etoposide and carboplatin
surgery - laser therapy ?
97
how is bitemporal hemianopia caused
lesion in the optic chiasm e.g. pituitary adenoma (lies above sella turcica of sphenoid bone
98
how is monocular visual field loss caused
optic nerve lesion
99
how is homonymous hemianopia caused (defect same in both eye)
optic tract - contralateral optic tract to visual loss
optic radiation if quadrantopia
100
how is contralateral homonymous hemianopia caused (with macular sparing)
visual cortex lesions (broadman area 17) in occipital lobe
101
describe moebius syndrome
facial paralysis and inability to move eyes to the side (b/l convergent squint'
expressionless
drooling
feeding difficultues
cleft palate
missing digits
clubbed feet
102
describe duane syndrome
inability to turn eye to abduct
103
describe brown syndrome
defect in superior oblique tendon sheath so unable to look upwards
104
eye problems with MS
1. b/l intranuclear ophthalmoplegia - pontine lesion at medial longitudinal fascicularis (cant adduct either eye)
105
describe a stye
abscess with pus in lumen of infected gland
full of polymorphonuclear leukocytes
106
describe congenital glaucoma presentation
corneal cloduing from oedema
reduced red reflex
watering of the eye
photophobia
107
symptoms of retinal detachment
1. flashes of light in the eye
2. blurred vision
3. sudden loss of vision/ curtain shadow over visual fieldfs
108
describe the changes in diabetic retinopathy
1st sign = dot haemorrhages, cotton wool spots, venous bleeding
late = macular oedema
advanced = blot haemorrhages, hard exudates -> soft exudates
screen > 12 y/o
109
how is spina bifida caused
develop in week 3-4 gestation
sodium valproate increases risk by 10-20 fold
3% risk of 2nd baby
prevention y taking folic acid
110
describe spina bifida occulta
defect in development of vertebral arch, usually lumbar spine
111
describe spina bifida cystica
more severe neural tube defect where spinal cord +/- meninges protrude throygh skin
associated with chiari malformation (downward displacement of cerebellar tonsils through foramen magnum
112
describe flow of tears
1. produced in lacrimal glands and into lacrimal duct
2. collect in lacrimal lake
3. drain into lacrimal sac and then into nasolacrimal duct
113
how does NF2 present
bilateral acoustic neuromas
tumours in cranial nerves
meningiomas
spinal astrocytomas
ependyomas
114
where does HSV1 encephalitis normally affect in the brain
temporal lobe
shows b/l aysmmetric gyral oedema with areas of haemorrhagic necrosis
115
describe Battens disease
dementia
optic atrophy
myoclonic jerks
116
describe hereditary spastic paraplegia
family history - autosomal dominant
increased tone
absence of perinatal events - similar to CP
117
describe piaget 4 stages for normal development
1. sensory motor 0-2 y/o
2. preoperational 2-7 y/o - cannot contemplate other peoples perspective
3. concrete operational 7-11 y/o - logical and abstract thought
4. formal operational > 11y/o - logical reasoning and planning
118
describe anterior cord syndrome
paraplegia with loss of spinothalamic pathways (pain + temperature) but preservation of dorsal column (proprioception, vibration and pressure)
119
defect in parietal lobe (visual )
homonymous inferior hemianopic cleft (Land R quadrant affected)
120
features of brain death
absent gag or cough reflex to oropharyngeal suctioning
absent pupillary response to light or corneal reflex
rise in blood PCO2 >20mmHg from 40 to 60 mmHG with no resp response
121
common site for iVH
germinal matrix in subependymal region in preterm babies
choroid plexus in term babies
122
describe dissociative seizure
seizure with emotional upset
non epileptic
older child
123
control of afferent and efferent optic pathway
optic nerve and oculomotor
124
describe internuclear ophthalmoplegia
medial longtidunal fissures - lesion on side eye fails to adduct
nystagmus on horizontal gaze
weaken adduction, abduction spared
125
presentation of adrenoleukodystrophy
change in behaviour
alteration in gait
UMN lesion - brisk reflexes, increased tone
white matter disease
126
cause of autism
abnormality of neuronal migration or connectivity
cell adhesion proteins involved - neuroligin , neurexin and cadherin coding genes
127
Where does strawberry naevus grow from
dermis
