Neuro-phthalmology Flashcards
Tell me about arteritic AION
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- most often caused by GCA
- chalky white optic disc
- gradual headache and scalp tenderness, sudden vision loss
- do bloods (CRP/ ESR), temporal artery biopsy
- give steroids
Tell me about non-arteritic AION
- HTN and T2DM
- sudden headache and vision loss
- segmental hyperaemia of disc and splinter haemorrhages
- exclude GCA
- treat underlying cause
Leber hereditary optic neuropathy
mitochondiral
boys 10-30
Triad
1. Disc pseudo-oedema
2. peripapillary telangiectasia
3. Tortuosity of the medium sized retinal arterioles
- optic disc atrophy later
- centrocaecal scotoma
Ix - genetic testing and OCT (optic disc oedema or atrophy in late)
Nutritional optic neuropathy aka tobacco-alcohol amblyopia
causes:
- vitamin B, copper, folate deficiency
- medications e.g. amiodarone, ethambutol, sildenagil
- alcohol and smoking
Sx - gradual ↓ VA, cetrocaecal scotoma, dyschromatopsia
Papilloedema
- Hyperaemia and blurred margins
- Swelling and elevation of the whole optic disc with peripapillary haemorrhages
Features of raised ICP
- headache, worse in the morning ± vomiting
- pulsatile tinnitus
- unilateral or bilateral transient vision loss
- enlarged blind spot
- CN VI palsy
- Cushing reflex (bradycardia, bradypnoea, hypertension)
Optic disc coloboma
Defect in embryonic fissure closing
- Associated with Goldenhar syndrome
- Glistening white bowl excavation of optic disc (often in inferior part, hence superior visual field defect)
Morning glory anomaly
Congenital optic disc malformation
- funnel shaped excavation of optic disc
- vessels originate from periphery of optic disc
- retinal detachment
Optic nerve hypoplasia
- Double ring sign
RF: genetic disease, maternal smoking and alcohol and recreational drugs
Horner syndrome
- partial ptosis
- miosis
- anhydrosis (not present in 3rd order neurone lesion)
Causes of Horner syndrome
- primary neurone - lateral medullary lesion or syringomyelia
- secondary neurone - Pancoast tumour, neck trauma
- Tertriary neurone - internal carotid artery dissection (painful)
Horner syndrome investigations
- Topical apraclonidine - alpha 1 and 2 agonist -> dialation due to denervation hypersensitivity
- Topical cocaine - inhibits neuroadrenaline reuptake. MYDRIASIS IN NORMAL PEOPLE BUT NOT IN HORNER!!!
- Hydroxymethamphetamine - stimulates NA release from post-ganglionic neurone. Does not dialate in 3rd order neurone lesion
- TOPICAL ADRENALINE - MYDRIASIS 3»_space;»2 , 1
Holmes Adie pupil anisocria
Unilateral, dialated pupil
constricts in response to light and accommodation
Constriction in accommodation to near object»_space; light
cause: loss of postganglionic parasympathetic nerves to iris and ciliary muscle
Holmes Adie investigations
Slit lamp
Topical pilocarpine - constriction due to denervation hypersensitivity
Argyll Robertson anisocria
Bilateral irregular small pupils
No response to light
But constrict in accommodation (near object)
Cause: DM, neurosyphilis
Anterior chiasmatic lesion
Willebrand’s knee (inferonasal fibres)
Junctional scotoma: ipsilateral anopia + contralateral supratempral VF defect
cause: tuberculum sellae meningioma
Bitemporal superior quadranopia
pituitary adenoma
Bitemporal inferior quadranopia
Craniopharyngioma
Bitemporal hemianiopia
Pituitary adenoma
Craniopharyngioma
Large anterior communicating artery aneurysm
Binasal hemianopia
Bilateral internal carotid aneurysm
CN III palsy, pupil sparing
HTN and DM
Pupillomotor fibres are located superficially, supplied by pial blood vessels
CN III palsy, pupils involved
Pupillomotor fibres involved
Surgical, trauma, uncal hernication, posterior communicating artery aneurysm
Vascular syndromes of CNIII palsy
- Weber’s syndrome
- Benedikt syndrome
- Nothangel synrome
- Claude syndrome (Benedikt + Nothangel syndromes)
Weber’s syndrome
ipsilateral CNiii palsy
contralateral hemiparesis
Benedikt syndrome
Ipsilateral CNIII palsy
Contralateral hemiataxia and hemitremor (damage to red nucleus)
Nothangel syndrome
ipsilateral CNIII palsy
Ipsilateral cerebellar ataxia (damage to superior cerebellar peduncles)
when is CNIV palsy worse
ipsilateral head tilt
CNIV palsy compensatory head position
contralateral head tilt
Internuclear ophthalmoplegia
- damage to MLF
impaired ipsilateral adduction + contralateral horizontal nystagmus
Parinaude syndrome
- often due to pinealoma
- Supranuclear gaze palsy (vertical gaze palsy) - sunsetting downwards gaze
- Collier sign (lid retraction)
- convergence retraction nystamus
Progressive supranuclear palsy
Vertical gaze palsy
- Slowing of vertical saccades
- Postural instability
- Parkinsonism (Parkinson’s plus)
2 types of physiological nystagmus
- End-point nystagmus (nystagmus at extreme gaze)
- Optokinetic nystagmus due to fast moving repetitive objects
Cogan lid twitch
In MG
Brief upshoot of lid after making patient look downwards then upwards
Myotonic dystrophy
- AD, trinucleotide repeats
Features:
1. unable to relax
2. polymorphic lens opacities - christmas tree cataract
3. ophthalmoplegia
4. Ptosis
*frontal baldness, testicular atrophy, cardiomyopathy
Kearns Sayre syndrome
mitochondrial. upto 20
Histopathology - ragged red fibres due to high mitochodnria
triad:
1. bilateral ptosis and ophthalmoplegia
2. pigmented retinopathy ‘salt and pepper’
3. cardiac conduction defect
Miller Fisher syndrome
Rare variant of Guillain Barre syndrome
tetrad:
ataxia, areflexia, ophthalmoplegia and facial diplegia
NF1
AD chromosome 17
features
- cafe au lait
- axillary freckling
- optic nerve glioma
- Lisch nodules
- ‘bag of worm sensation’ neurofibroma
- choroidal naevi
NF2
AD chromosome 22
- meningiomas
- Bilateral acoustic schwannoma
- posterior subcapsular cataracts
Tuberous Sclerosis
AD
- Ash leaf
- angiofibroma
- seziures and cognitive impairment
- intracranial or retinal astrocytic hamartomas
Benign essential blepharospasm
females, 60s
mx: articial tears for dry eyes, botox injection in orbicularis occuli (ptosis, dry eyes), surgical myectomy
Posterior chiasm lesion
Paracentral scotoma (macula fibres cross posteriorly)
Gradenigo syndrome
otitis media + CN6 palsy - spread of infection to apex of petrous bone
Dorello canal in petrous bone
Test for stereopsis
Titmus test
Synoptophore
TNO and Lang
Test for sensory fusion
worth 4 dot
Bagolini glasses
Test for motor fusion
Prism test (Risley)
