neuro, eyes and development Flashcards

1
Q

EEG of absence seizures

A

3 sec spike and wave

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2
Q

management of absence seizures

A

ethosuximide, sodium valproate

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3
Q

management of tonic clonic seizures

A

1st line - sodium valproate
1st line in girls >10 y/o - lamotrigine

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4
Q

describe infantile spasm

A

sudden symmetrical synchronous spasm and then a tonic phase
developmental delya
within 1st year of life

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5
Q

EEG of infantile spasm

A

hypsarrhythmia

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6
Q

treatment fo infantile spasm

A

vigabatrin

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7
Q

describe lennox gastraut syndrome

A

visual aura **
poor prognosis and only 10% seizure free on meds
due to structural brain abnormalities

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8
Q

describe landua kleffner syndrome

A

subacute onset of aphasia
previous normal development
residual language impairment
self limiting seizures

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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

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10
Q

EEG of benign rolandic epilepsy

A

centrotemporal spikes

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11
Q

describe panayiotopoulos syndrome

A

prominent autonomic features e.g. vomiting, retching,pallor

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12
Q

EEG of panayiotopoulos

A

occipital spikes

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13
Q

management of neonatal seizures

A

phenobarbitone

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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

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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

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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

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17
Q

describe dravet syndrome

A

severe myoclonic epilepsy
triggered by fevers
developmental delay
difficult to control
70% have SCN1A mutation

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18
Q

describe subacute sclerosing panencephalitis

A

complication 7-10 years after measles infection
fatal and progressive CNS disorder
myoclonus seizure
mood change

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19
Q

investigations for subacute sclerosing panencephalitis

A

EEG - burst suppression
elevated anti measles antibody Igg IN SERUM AND CSF

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20
Q

describe temporal seizures

A

complex visual hallucinations
auras of taste

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21
Q

describe occipital seizures

A

simple visual experiences

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22
Q

symptoms of raised ICP

A

early morning headache
vomiting
visual disturbances
fluctuating consciousnessness
ataxia
seizures
abnormal pupils

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23
Q

cushings triad

A
  1. hypertension
  2. irregular breathing
  3. bradycardia
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24
Q

consequence of cushings triad

A

signs of impending brainstem herniation

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25
Q

management of raised ICP

A
  1. CT head with contrast
  2. urgent neuro surgical discussion
  3. lie with head at 20-30 degrees
  4. mannitol or hypertonic saline 3%
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26
Q

what is idiopathic intracranial hypertension

A

elevated ICH >25 CM WATER with unknwon cause

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27
Q

management of idiopathic intracranial hypertension

A
  1. MRI with venography
  2. acetazolamide (carbonic anhydrase inhibitor) or topiramate
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28
Q

causes of subarachnoid haemorrhage

A
  1. arteriorvenous malformations **
  2. cerebral aneuryms (circle of willis, sylvian fissure)
  3. space occupying lesion
  4. head trauma
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29
Q

presentation of subarachnoid haemorrhage

A
  1. thunderclap headache - severe, sudden
  2. vomiting
  3. focal neurological signs e.g. seizures, irritable, enck stiffness
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30
Q

management fo subarachnoid haemorrhage

A
  1. cT head and cerebral angiography
  2. discuss neuro surgical team (?surgical clipping)
  3. bp control
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31
Q

SF2 type of hydrocephalus

A
  1. non communicating - obstruction of CSF reabsorption ***
  2. communicating - impaired secretion or reabsorption
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32
Q

causes of non communicating hydrocephalus

A
  1. CONGENITAL
    - arnold chiari malformation
    - spina bifida
    - dandy walker malformation
    - congenital toxoplasmosis
  2. ACQUIRED
    - mass lesions (medulloblastoma - obstruction at 4th ventricle)
    - IVH
    - infection
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33
Q

causes of communicating hydrocephalus

A
  1. impaired secretion
    - choroid plexus papilloma (excessive production of CSF)
  2. impaired reabsorption
    - infections e.g. meningitis
    - haemorrhage
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34
Q

direction of CSF travel

A
  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
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35
Q

signs of hydrocephalus

A

increasing head circumference
bulging fontanelle
dilated scalp veins
sun setting sign
irritable
vomiting
impaired consiousness level
headache

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36
Q

management of non communicating hydrocephalus

A

ventriculoperitoneal shunt - one way valve so CSF draians from cranial vault to low pressure peritioneal cavity

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37
Q

causes of neonatal meningitis

A
  1. group b strep
  2. e.coli
  3. listeria
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38
Q

Causes of child meningitis

A
  1. enterovirus ** -most common
  2. h . influenza
  3. strep pneumonia
  4. meningococcal
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39
Q

presentation of meningitis

A

fever
headache
irritable
vomiting
seizures
neck stiffness
non blanching petechial rash - meningococcal
photophobia

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40
Q

bacterial CSF of meningitis

A
  1. high neutrophil count **
  2. low glucose
    3.. high portein
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41
Q

viral CSF of meningitis

A
  1. high lymphocyte count
  2. normal glucose
  3. normal protein
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42
Q

management of meningitis

A

notifiable disease

  1. iv antibiotics - ceftriaxone + amoxicillin (cefotaxime + amoxicillin in neonates)
  2. IV aciclovir - if herpes suspected
  3. IV dex - helps hearing loss
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43
Q

management of household contacts of meningitis

A

ciprofloxacin
IM ceftriaxone to pregnant women

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44
Q

cause of guillain barre syndrome

A

post infectious neuropathy (generate IgG antibodies against myelin sheath) around 10 days after:
1. gastroenteritis - campylobacter
2. vaccines - rabies, influenza
3. resp infection - mycoplasma

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45
Q

presentation of guillain barre syndrome

A
  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)
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46
Q

investigations for GBS

A
  1. Nerve conduction test
  2. LP - elevated CSF protein, normal CSF cell count, normal glucose
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47
Q

management of GBS

A
  1. IV IG
  2. corticosteroids
  3. intubation and ventilation in ITU
  4. pain relief
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48
Q

presentation of dermatomyositis

A

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

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49
Q

diagnosis of dermatomyositis

A
  1. ALT *, CK, aldolase rise
  2. ANA +ve in 80%
  3. myositis specific antibodies
  4. muscle biopsy
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50
Q

management of dermatomyositis

A
  1. avoid sun
  2. physiotehrapy
  3. corticosteroids over 12 months
  4. methotrextate and hydroxychloroquine
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51
Q

describe bells palsy

A

lower motor neurone palsy, unilateral, preceded by viral infection
weakness of whole face
facial nerve compression as passes through temporal bone

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52
Q

defect in hemi section syndrome brown sequard

A

ipsilateral weakness, loss of vibration and position sense
contralateral loss of temperature + pain sensation (anterior and lateral spinothalamic tracts)

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53
Q

describe dandy walker malformation

A
  1. cerebellar vermis hypoplasia
  2. dilation of 4th ventricle
  3. enlarged posterior fossa
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54
Q

describe horner syndrome

A
  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
Q

diagnosis of spinal muscular atrophy

A

chromosomal microarray

56
Q

drugs used to improve aquous outflow in glaucoma

A
  1. pilocarpine
  2. aparclonidine (alpha 2 adrenergic agonists)
  3. prostaglandin anologies (latanoprost)
56
Q

where does abducens and facial nerve arise

A

pontine medulla junction

56
Q

where does trigeminal nerve arise

A

pons

56
Q

describe self gratification behaviour

A

intact consciosuness
cessation of episode after distraction
grunting/ abnromal posturing/ arching

57
Q

describe blue breath holding attacks

A

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
Q

treatment for JME

A

sodium valproate or keppra

AVOID carbamazepine or lamotrigine as makes episodes worse
usually life long treatment as high risk of relapse

57
Q

describe marcus gunn pupil

A

relative afferent defect
retina has sluggish response to light shining
into it
‘jaw winking syndrome’
as open mouth, R eye lid goes up

58
Q

describe coloboma

A

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
Q

vascularisation of retina in utero

A

starts at 14 weeks and continues until birth
stimulated by vascular endothelial growth factor A and insulin like growth factor

60
Q

describe retinopathy of prematurity

A

neovascular disorder affecting babies less than 32 weeks gestation or low birth weight (<1.5kg)

61
Q

pathophysiology of retinopathy of prematurity

A
  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
Q

management of ROP

A

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
Q

causes of congenital cataracts

A
  1. idiopathic
  2. familial (20%) if bilateral or parental consanguinity
  3. syndromes e.g. downs , galactosaemia, tORCH
64
Q

management of congenital cataracts

A

surgery within first 2 months of life
topical steroid therapy post op

65
Q

describe childhood glaucoma

A
  1. raised intra ocular pressure - due to impaired aqueous outflow through the trabecular meshwork
  2. optic disc cupping
66
Q

causes of childhood glaucoma

A
  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
Q

pattern of eye pathology in NAI

A

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
Q

what does the oculomotor nerve control in the eye (CN III)

A
  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
Q

oculomotor (CN III) palsy

A

‘down and out’ eye with ptosis

68
Q

what does trochlear nerve (CN IV) control in the eye

A

superior oblique - depresses the adducted eye, inward rotation

69
Q

trochlear nerve palsy

A

problems reading/ going downstairs
reduced depression in the adducted eye
head tilt towards unaffect side
vertical diplopia
both eyes still look forward

70
Q

what does the abducen nerve control (CN VI)

A

Lateral rectus - controls abduction

palsy = horizontal gaze

71
Q

describe horners syndrome

A
  1. small pupil which dilates poorly in the dark
72
Q

which anti muscarinic eye drops are used

A
  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
Q

which sympathomimemetic eye drops are used

A

used with anti muscarinics for intense mydriasis - needed in surgery, ROP screening etc

  1. phenylephrine 2.5%. last 12 hours
74
Q

describe adie pupil

A

b/l or unilateral inability to constrict pupil (viral infection disrupts ciliary ganglion causing parasympathetic dysinnervation)

test using pilocarpine - miosis if +ve

75
Q

risk factors for squints

A

downs syndrome
cerebral palsy
refractive error
cataract
retinoblastoma
family history

acute RF : head trauma, neoplasm, meningitis, encephalitis, hydrocephalus

76
Q

define myopia

A

short sighted

77
Q

define hypermetropia

A

long sighted

78
Q

types of squints/ strabismus

A
  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
Q

define convergent and divergent

A

convergent/ esotropia = one eye turns inwards (more common)
divergent = one eye turns outwards

80
Q

how to test for squint

A
  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
Q

management of squint

A
  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
Q

causes of orbital cellulitis

A
  1. H influenza
  2. staph aureus (trauma to eye)
  3. strep pneumoniae
  4. fungi

complication of sinusitis

83
Q

presentation of orbital cellulitis

A
  • orbital pain
  • proptosis
  • chemosis (swelling of eyelids)
  • diplopia
  • reduced visual acuity
  • painful extra ocular motion / restricted eye movements
  • fever
84
Q

complications of orbital cellulitis

A
  • visual loss
  • increased intraocular pressure
  • central retinal artery occlusion
  • orbital abscess
  • meningitis
  • sub periosteal abscess
85
Q

investigations for suspected orbital cellulitis

A
  1. FBC, blood cultures
  2. eye swabs
  3. ct of sinuses and head and orbit
86
Q

management of orbital cellulitis

A
  1. co-amoxiclav PO (if no fever, pre septal) or IV cefuoxime
  2. surgery if collection / abscess/ no response to abx
87
Q

cause of uveitis

A
  1. autoimmune - SLE, JIA (oligoarticular), crohns, sarcoidosis, rheumatoid arthritis
  2. HLA B27 - ank spon
  3. idiopathic
88
Q

how does uveitis present

A

red eye
ocular pain
photophobia
reduced visual acuity
tenderness of upper eyelid

89
Q

structures in the uvea

A
  1. anterior uvea - iris and ciliary body
  2. posterior uvea - choroid
90
Q

investigations for uveitis

A
  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
Q

management of uveitis

A
  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
Q

pathophysiology of retinoblastoma

A

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
Q

presentation of retinoblastoma

A
  1. leukocoria = white eye reflex on newborn screening
  2. strabismus
  3. reduced vision
  4. eye redness and pain due to inflammmation
94
Q

differentials for leukocoria

A
  1. congenital cataracts **
  2. retinoblastoma
95
Q

investigations for retinoblastoma

A
  1. CT and MRI brain
  2. ophthalmoscopy with cyclopentolate eye drops
  3. genetic testing for RB1 gene
96
Q

management of retinoblastoma

A

systemic chemo and radio - vincristine, etoposide and carboplatin
surgery - laser therapy ?

97
Q

how is bitemporal hemianopia caused

A

lesion in the optic chiasm e.g. pituitary adenoma (lies above sella turcica of sphenoid bone

98
Q

how is monocular visual field loss caused

A

optic nerve lesion

99
Q

how is homonymous hemianopia caused (defect same in both eye)

A

optic tract - contralateral optic tract to visual loss

optic radiation if quadrantopia

100
Q

how is contralateral homonymous hemianopia caused (with macular sparing)

A

visual cortex lesions (broadman area 17) in occipital lobe

101
Q

describe moebius syndrome

A

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
Q

describe duane syndrome

A

inability to turn eye to abduct

103
Q

describe brown syndrome

A

defect in superior oblique tendon sheath so unable to look upwards

104
Q

eye problems with MS

A
  1. b/l intranuclear ophthalmoplegia - pontine lesion at medial longitudinal fascicularis (cant adduct either eye)
105
Q

describe a stye

A

abscess with pus in lumen of infected gland
full of polymorphonuclear leukocytes

106
Q

describe congenital glaucoma presentation

A

corneal cloduing from oedema
reduced red reflex
watering of the eye
photophobia

107
Q

symptoms of retinal detachment

A
  1. flashes of light in the eye
  2. blurred vision
  3. sudden loss of vision/ curtain shadow over visual fieldfs
108
Q

describe the changes in diabetic retinopathy

A

1st sign = dot haemorrhages, cotton wool spots, venous bleeding

late = macular oedema

advanced = blot haemorrhages, hard exudates -> soft exudates

screen > 12 y/o

109
Q

how is spina bifida caused

A

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
Q

describe spina bifida occulta

A

defect in development of vertebral arch, usually lumbar spine

111
Q

describe spina bifida cystica

A

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
Q

describe flow of tears

A
  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
Q

how does NF2 present

A

bilateral acoustic neuromas
tumours in cranial nerves
meningiomas
spinal astrocytomas
ependyomas

114
Q

where does HSV1 encephalitis normally affect in the brain

A

temporal lobe

shows b/l aysmmetric gyral oedema with areas of haemorrhagic necrosis

115
Q

describe Battens disease

A

dementia
optic atrophy
myoclonic jerks

116
Q

describe hereditary spastic paraplegia

A

family history - autosomal dominant
increased tone
absence of perinatal events - similar to CP

117
Q

describe piaget 4 stages for normal development

A
  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
Q

describe anterior cord syndrome

A

paraplegia with loss of spinothalamic pathways (pain + temperature) but preservation of dorsal column (proprioception, vibration and pressure)

119
Q

defect in parietal lobe (visual )

A

homonymous inferior hemianopic cleft (Land R quadrant affected)

120
Q

features of brain death

A

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
Q

common site for iVH

A

germinal matrix in subependymal region in preterm babies
choroid plexus in term babies

122
Q

describe dissociative seizure

A

seizure with emotional upset
non epileptic
older child

123
Q

control of afferent and efferent optic pathway

A

optic nerve and oculomotor

124
Q

describe internuclear ophthalmoplegia

A

medial longtidunal fissures - lesion on side eye fails to adduct

nystagmus on horizontal gaze
weaken adduction, abduction spared

125
Q

presentation of adrenoleukodystrophy

A

change in behaviour
alteration in gait
UMN lesion - brisk reflexes, increased tone

white matter disease

126
Q

cause of autism

A

abnormality of neuronal migration or connectivity
cell adhesion proteins involved - neuroligin , neurexin and cadherin coding genes

127
Q

Where does strawberry naevus grow from

A

dermis