6.1.14 Assesses signs and symptoms of neurological significance.

Question 1

Optic nerve dysfunction, expect:

Answer 1

• Reduced VA – D&N
• Impaired CV – R/G or B/Y
• RAPD
• VF defect
• Impaired CS

Question 2

Differential diagnosis of raised optic disc:

Answer 2

Optic disc oedema
* Papilloedema
* Anterior ischemic optic neuropathy
* Optic neuritis

Compression of optic nerve
* Infiltration of the optic nerve
* Congenital optic neuropathies
* Toxic optic neuropathies
* Traumatic optic neuropathies

(Raised but no swelling)
* Optic disc drusen
* Tilted optic disc
* Myelinated nerve fibres
* Hypermetropic crowded disc

Intraocular disease which can cause raised disc appearance
* CRVO, posterior uveitis, posterior scleritis, hypotony

Excavated disc anomalies
* Optic disc pit
* Coloboma
* Optic nerve hypoplasia
* Morning glory anomaly
*

Question 3

Optic disc oedema

Characteristic appearance of the optic nerve head:

Answer 3

• Elevated appearance of nerve head
• Filling of cup
• Retinal vessels drape over disc margin
• Blurring of margin
• RNFL oedema – greyish appearance that obscures vessels
• Hyperaemia – BV swollen, redness
• Retinal venous dilatation & tortuosity
• Peripapillary haemorrhages, exudates, cotton wool spots
  *

Question 4

Optic disc oedema

Papilloedema

Answer 4

Swelling of the optic nerve head secondary to raised ICP
Causes
* Intracranial mass
* Hydrocephalus (dilation of ventricles)
* Central nervous system infection e.g. meningitis
* Trauma
* Infiltration e.g. leukaemia, sarcoidosis
* Benign intracranial hypertension (only benign when all other causes are absent) – most common in overweight, middle aged women
*

Question 5

Papilloedema

Symptoms

Answer 5

• Occasionally asymptomatic (could be picked up in routine eye exam)
• Nausea and vomiting – projecticle vomiting
• Deterioration of consciousness – from slight (drowsy) to dramatic
• Pulsatile tinnitus
• Can be ‘muzzy headed’ at start and then develop into extreme pain, usually presenting at hospital within 6 weeks
• Characteristically in morning, waking up px
• Generalized or localised
• Headaches worse when pressure increases - moving head, bending over, coughing

Visual symptoms (only after some time i.e. in established cases)

• Visual symptoms often absent but reduced VA in later stages
• Transient visual loss (especially when bendning over)
• Horizontal diplopia (6th nerve palsy)
• Constriction of visual field
• Altered colour perception

Question 6

Papilloedema

Signs

Answer 6

EARLY – first few weeks
- VA normal
- Optic disc shows Hyperaemia, mild elevation and indistinct margins
- Dilation of capillary net
- Absent SVP (absent in 20% normals)

ESTBALISHED ~ 6 months
VA normal or reduced
- Optic disc shows venous engorgement, moderate elevation, flame-shaped haemorrhages and cotton wool spots
- Enlarged blind spot
- Hard exudates in macular fan

CHRONIC > 6 months
- VA is variable
- Optic discs are pale and markedly elevated
- No haemorrhages or cotton wool spots
- Collateral vessels form
- Variable field loss

ATROPHIC – years after initial presentation
- VA severely impaired
- Fields impaired
- Optic discs are pale grey and slightly elevated with indistinct margins

Question 7

OCT and papilloedema?

Answer 7

 RNFL white showing raised
 Can use measurement tools on OCT to measure disc swelling (>400 microns indicate a swollen disc)

Question 8

Management (CMGS) of papillaedema

Answer 8

• Communicate severity of situation to px
• Phonecall to ARC
• Emergency referal to ophthalmology/neurology with letter in hand
• Younger px = less likely to be bengin
• Treatment of cause at HES
• Carbonic anhydrase inhibitor to reduce ICP in IIH

Question 9

Arteritic

Answer 9

• Occurs in >70s
• Caused by giant cell arteritis
• Associated systemic features e.g. hard temporal artery, scalp tenderness, jaw claudification, muscle weakness, reduced appetite
• Severe vision loss (poorer than 6/60)
• Pale swollen disc, cotton wool spots, RAPD
• Stroke risk high: high chance of 2nd eye being affected
  *

Question 10

Non-arteritic

Answer 10

• Occurs in >60s
• Caused by occlusion of short posterior arteries (RF’s: DM, HBP, smoking)
• No systemic features
• Mod-severe loss of vision (better than 6/60)
• Hyperaemic swollen disc, peripapillary flame shaped haems
• Lower risk
  *

Question 11

AAION (GCA);risk factors, symptoms, signs and management

Answer 11

**Inflammation of ON due to ischaemia **

Risk factors

• Hypertension
• Smoking
• Diabetes
• Stroke
• Sleep apnoea
• Anaemia

Symptoms

• Sudden, profound unilateral visual loss
• May be proceeded by transient visual obscuration (amaurosis fugax), flashing lights
• Periocular pain
• Systemic – headache, pain in the neck & temples, weight loss, fatigue, scalp tenderness, jaw claudication

Signs

• VA HM to no PL
• Pale swollen disc
• Peripapillary haemorrhages
• Cotton wool spots (signifying retinal ischaemia)
• RAPD

Management

• Emergency referral to ophthalmology
• Sight-threatening nature, and to administer prompt treatment with the aim of preventing blindness to the 2nd eye
• Temporal artery biopsy
• Vision lost usually permanent
• Prompt administration of steroids (with gastric protection) may allow for partial visual recovery
• Iv methylprednisolone 500m to 1g/day for 3 days, followed by oral prednisolone for 3 days
• Treatment continued until symptom resolution and ESR normalization, or 4 weeks
• In 25% cases 2nd eye is affected despite treatment/steroid administration– within a few days

Question 12

NAION;risk factors, symptoms, signs and management

Answer 12

Inflammation of optic nerve due to ischaemia, not related to inflammation of arteries
Causes
* Drop in BP
* Increase in blood viscosity
* Reduced blood flow around nerve
* Most AION’s are non-arteritic - 90% of AION cases

Risk factors

• Structural crowding of disc - cup is small/absent
• Common modifiable risk factors include DM, HBP, high cholesterol, smoking

Symptoms

• Sudden, painless unilateral vision loss
• Over hours - days
• Often described as a blurring or cloudiness of vision
• Px may also report H/A (8-12% px’s)

Signs

• VA 6/18 to CF
• May only be slightly reduced/normal in some individuals
• Diminished colour vision
• Hyperaemic, swollen disc with a few splinter shaped haemorrhages
• Altitudinal hemianopia – most commonly inferior
• Fellow eye has smaller or absent cup

Management:
* Emergency referral to ophthalmology
* Sudden loss in vision warrants emergency referral according to CMGs
* No definitive treatment, some advocate short-term systemic steroid treatment
* Treatment of any underlying systemic predispositions

Question 13

Posterior Ischaemic Optic Neuropathy (PION)

Answer 13

• Much rarer than AION
• Caused by ischaemia of the retrolaminar portion of the optic nerve supplied by the surrounding pial capillary plexus, which in turn is supplied by the plial branches of the ophthalmic artery.
• Diagnosis made only after other causes of retrobulbar optic neuropathy have been excluded (e.g. compression, inflammation

Question 14

Optic neuritis

Ophthalmology classifications

Answer 14

Acute or subacute inflammatory or demyelinating process affecting the optic nerve
Retrobulbar neuritis – normal appearance of the OD, at least initially
Most common type in adults
Frequently associated with MS
Other causes: tuberculosis, syphilis, viral infections

Papillitis – disc hyperaemia/oedema, occasionally PP flame-shaped haemorrhages
* Most associated with diabetes

Neuroretinitis – papillitis and a macular scar
* 66% caused by cat scratch

Question 15

Optic neuritis

Aetiology classification

Answer 15

 Demyelinating (MS)
 Para-infectious
 Infectious (Cat-scratch)
 Non-infectious/autoimmune (DM)

Question 16

Retrobulbar neuritis

Retrobulbar neuritis

Answer 16

 Demyelination may be caused by MS or be idiopathic, usually, age range 20-50 years,
 75% patient’s female

Question 17

Focal Visual Field Defects types

Answer 17

1. Central scotoma
   * Demyelination
   * Toxic and nutritional
   * Leber hereditary optic neuropathy
   * Compression
2. Enlarged blind spot
   * Papilloedema
   * Congenital anomalies
3. Respecting horizontal meridian
   * Anterior ischaemic optic neuropathy
   * Glaucoma
   * Disc drusen
4. Upper temporal defects not respecting vertical
   meridian
   * Tilted discs

Question 18

Differential Diagnosis of Different Neurological Conditions

Classification of Optic Neuropathy: (in bold is what you NEED to know!)

Answer 18

• INFLAMMATORY - Optic Neuritis; this includes demyelinating, parainfectious, infectious & non-infectious & neuroretinitis
• PAPILLOEDEMA - secondary to increased intracranial pressure
• ISCHAEMIC - Anterior non-arteritic, anterior arteritic, posterior ischaemic and diabetic papillopathy
• COMPRESSIVE - Secondary to Orbital lesion
• INFILTRATIVE - Inflammatory conditions (e.g. sarcoidosis), tumours and infective agents
• TRAUMATIC
• HEREDITARY - Leber & other ones
• NUTRITIONAL & TOXIC

Question 19

Differential Diagnosis of Different Neurological Conditions

Classification of Optic Neuritis

Answer 19

• Based on Ophthalmoscope Appearance
  
  • Retrobulbar neuritis - ONH not involved initially so looks normal; associated with MS!
  • Papillitis - common in children. Hyperaemia & oedema of disc, with flame haems & cells in posterior vitreous
  • Neuroretinitis - papillitis + inflammation of RNFL + macular star. Rare!

Question 20

Differential Diagnosis of Different Neurological Conditions

Based on Aetiology

Answer 20

• Demyelinating - by far most common cause!
• Parainfectious - viral infections (measles, mumps, chickenpox, whooping cough & grandular fever)
• Infectious - inflammation of nerve (cat scratch fever, tuberculosis, lyme disease)
• Non-infectious

Question 21

Demyelinating Optic Neuritis

• What is demyelinating?
• What is the association of Optic neuritis Vs MS?

Answer 21

What is demyelinating?

• A pathological process in which normally myelinated nerve fibres lose their insulating myelin layer. Disrupts nervous conduction within the white matter tracts of the brain, brainstem and spinal cord
• MS more in women then men, 3rd-4th decade

What is the association of Optic neuritis Vs MS?

• Optic neuritis is the presenting feature of MS in up to 30%.
• Optic neuritis occurs at some point in 50% of patients with established MS

Question 22

Demyelinating Optic Neuritis

• Symptoms
• Signs
• Management

Answer 22

• Symptoms
  
  • Subacute monocular VA drop
  • Phosphenes - tiny white or coloured flashes
  • Pain on motility, in 90% of patients. Lasts few days
  • Frontal headaches & tenderness
• Signs
  
  • VA 6/18-6/60 - generally worsens for few weeks then slowly improves over next 6-12 months
  • RAPD, dyschromatopsia
  • Normally normal optic disc (retrobulbar) then rest are papillitis
  • Visual field - Diffuse central 30 degrees depression, altitudinal, focal central/centrcaecal
• Management
  Urgent referral within 1 week. Steroid treatment

Question 23

Papilloedema

• What is this condition?
• Symptoms
• Signs
• Is Idiopathic Intracranial Hypertension similar to Papilloedema?
• Management

Answer 23

• What is this condition?Swelling of the optic nerve head secondary to raised intracranial pressure
• Symptoms
  
  • Headaches - worse in morning, may wake up px at night, intensify with head movement/bending/coughing. Get worse with time
  • Nausea - vomiting possible
  • Consciousness deterioration - when papilloedema is far spread
  • Visually - transient visual obscurations lasting for up to 30 seconds
  • Horizontal diplopia - possible
• Signs
  
  • Disc hyperaemia
  • Indistinct margins & difficulty seeing optic cup. As it worsens, cup looks larger
  • Disc elevation
  • Optociliary shunts - sometimes
  • Venous engorgement, peripapillary flame haems, CWSs
  • Paton lines
  • Enlarged blind spot
  • Macular fan - NOT macular star. In younger people, this is small vesicles in superifical retina. NOT exudates
  • Secondary optic atrophy - VA impaired, grey-white disc, few crossing vessels, indistinct margins, elevated
• Is Idiopathic Intracranial Hypertension similar to Papilloedema?Quite similar!Sometimes other symptoms & cranial nerve palsiesNo identifiable cause. Occurs most young obese women. Contraceptive pill
• ManagementEmergency ReferralWeight lossLumbar puncture/spinal tap - drains extra fluid from brain to reduce swellingCorticosteroids to reduce pressureAcetazolamide to keep nervous system pressure normalIf HBP the issue, other medications can be injected or taken orally (B blockers, diuretics, ACE inhibitors)

Question 24

NAION

• How is it caused?
• Signs
• Differential Diagnosis between AAION
• Management

Answer 24

• How is it caused?Occlusion of short posterior ciliary arterys causing partial or total infarction of ONH
• Signs
  
  • Other typical neuro symptoms like RAPD, inferior altitudinal defect most common, reduced colour vision etc
  • Painless, acute monocular drop in VA (6/24 or worse), described as cloudy or blurry vision
  • NO ACCOMPANYING PAIN - no HAs, pain on motility, periocular pain
  • How is dyschromatopsia different compared to optic neuritis?In optic neuritis - VA can be good & CV may be more impaired than it should beIn NAION - VA proportional to CV loss
  • Disc hyperaemia - diffuse or sectoral, swelling, few splinter peripapillary haems
    
    • Generally resolves, atrophy 3-6 weeks after
• Differential Diagnosis between AAION
  
  • NAAION - associated with small-crowded optic nerves, hypertension, diabetes, and hyperlipidemia whereas AAION do not normally have same vascular risk factors. MORE SYSTEMIC
  • Px with GCA are typically older than those with NAION (i.e. 8th-9th decade vs. 6th-7th decade)
  • In NAION, patients will usually not note any associated systemic symptoms such as headache, jaw claudication, scalp tenderness, weight loss, anorexia, fever, or myalgias/arthralgias
  • VA worse in GCA, 1/3 patients having 6/60 or worse
  • Unlike NAION, AAION patients demonstrate a diffuse “chalky” white edema, eventual cupping of the disc, and possible coexistent retinal ischemia. NAION often demonstrates segmental optic nerve edema with eventual sectoral or total flattening and pallor
• ManagementUrgent referral within 1 weekNo definitive treatment; steroids may work alongside treatment of anything systemic underlying the condition

Question 25

# AAION - What is this condition? - Symptoms - Signs - Management

Answer 25

- What is this condition? - Giant cell arteritis is an inflammation of the lining of your arteries. Most often, it affects the arteries in your head - Symptoms - Age ≥ 55 years - Scalp tenderness (first noticed when combing hair) - Recent HA (frontal, temporal, occipital or more generalized) - Jaw claudication (ischaemia of the masseter muscles) - Weight loss, fever, night sweats, malaise, depression - Signs - Thick, tender & inflamed temporal artery & pulsating - Sudden painless monocular VA loss (on awakening normally) - Preceded by amarousis fugax attacks - Potentially diplopia present - Pale oedematous disc, flame haemorrhages - Visual field defect - inferior altitudinal, can be central, paracentral, quadrantic & arcuate - Ocular motor nerve palsies manifest - 3rd nerve potentially - Management - TREATMENT - Emergency referral, Steroids

Question 26

# Tumours & Compressive Lesions Pituitary Adenoma - Why should a low threshold be implored in regard to px symptoms? - What are the main Signs & Symptoms? - Management

Answer 26

- Why should a low threshold be implored in regard to px symptoms? Early tumours are likely to give vague symptoms and so you may miss it! - What are the main Signs & Symptoms? - What visual field defect is classic? Bitemporal hemianopia - Frontal HA - gradual onset of symptoms - Desaturated red - across vertical midline from nasal to temporal comparison for each eye suggests compression - Optic atrophy possible - Management Depends on extent, generally urgent if a lot of vision loss. Soon within a month if very subtle signs MRI done Radiation modulated radiation therapy

Question 27

Anisocoria

Answer 27

* Difference of >0.4mm * 25% of normal have this in dim light, 10% in bright light * If anisocoria present – size difference must be measured in bright & dim light * If difference remains constant = likely to be normal * 20% of population have physiological anisocoria * Difference greatest in dim light * Possibly normal * Possible problem with dilation of smaller pupil * Suggests lesion affecting sympathetic innervation of dilator * Difference largest in bright light * Possible problem with constriction of larger pupil = REFER * Suggests a lesion affecting parasympathetic innervation of sphincter * Abnormal eye movements suggest preganglionic lesions; normal eye movements suggest post ganglionic lesion * Pathological Anisocoria = defect in the efferent (motor) pathway

Question 28

Sympathetic pupil pathway (efferent pathway = brain > eyes)

Answer 28

* Sympathetic innervation = dilation = dilator pupillae * Sends signals from Edinger Westphal nuclei to both pupils via the ciliary ganglion & then CN3 (oculomotor nerve) * Efferent defects are due to damage to the efferent innervation of the pupil and will result in unequal pupil size (anisocoria) *** If affected; anisocoria seen; no RAPD** * Congenital (causes heterochromia also) – reassure & record * Horner’s syndrome * Holmes Aidie Pupil * Intraocular surgery * Trauma * Uveitis * Acute angle-closure glaucoma * Tumours of iris or ciliary body * Anterior segment neovasc

Question 29

Horner’s Syndrome

Answer 29

* Anisocoria greater is dark conditions (affected pupil cannot dilate) * Affects sympathetic nerve supply * Signs: miotic pupil, ptosis, sweating down one side of face (hemifacial anhidrosis), reduced IOP on effected side * Cause: commonly lung tumour * Management: urgent referral / record if longstanding * Diagnosis is usually confirmed pharmacologically using apraclonidine drop

Question 30

Holmes-Adie pupil

Answer 30

* Common in young woman & usually unilateral * Anisocoria greater in light conditions (affected pupil cannot constrict) * Affects parasympathetic nerve supply * Symptoms: reading problems & glare/photophobia * Signs: dilated pupil with poor/tonic reaction to light – restricts better when accommodating * Cause: damage to the post-ganglionic supply to the ciliary ganglion & sphincter pupillae or viral infection * Management: Urgent referral to check for underlying cause Pilocarpine check test: disease/dilated eye constricts more than the other after pilocarpine instillation as the sphincter becomes very sensitive in the defect eye & absorbs more

Question 31

Argyll Robertson Pupil

Answer 31

* Very rare * Signs: Bilateral miosed & irregular pupils; dilate poorly in darkness * Accommodative but not reactive to light (light/near dissociation) * Cause: neurosyphilis / lesions in the rostral midbrain *- In bright light neither pupil constricts, but on accommodation (near target) both constrict - In dark conditions, lack of dilation